Pulmonary embolism with cardiogenic shock

A woman in her mid-60s with end-stage lung disease secondary to systemic sclerosis and pulmonary hypertension underwent bilateral sequential lung transplantation without cardiopulmonary bypass through a clamshell thoracotomy

The immediate postoperative course was uneventful, and she was weaned off oxygen support

On postoperative day 10, she developed seizures when walking and was immediately intubated

Brain imaging revealed a new intracerebral hemorrhage in the right frontal and left occipital lobes, with narrowing of the internal carotid, basilar, posterior cerebral, and right posterior communicating arteries, suggestive of vasospasm or vasculopathy

Subsequently, she developed hypoxemia and hypotension associated with acute pulmonary edema and new cardiomegaly (Figure 1A)

An electrocardiogram showed nonspecific ST changes with T-wave inversion (Figure 1B), and serum levels of troponin and creatine phosphokinase were negative

Transesophageal echocardiography revealed new dilation of the left ventricle (LV), with an ejection fraction of 10%, functional mitral regurgitation, anterior and apical akinesis, and basal hypokinesis (Video 1, Video 2, and Video 3)

The right ventricle (RV) was distended, with moderate tricuspid regurgitation

Recent pretransplantation coronary angiography did not reveal atherosclerosis

Her cardiopulmonary status rapidly worsened, with cardiogenic shock and refractory hypoxemia (arterial blood pH, 7.12

arterial partial pressure of oxygen, 43 mm Hg

and partial pressure of carbon dioxide, 56 mm Hg), despite mechanical ventilation (rate, 24/min

and positive end-expiratory pressure, 15 cm H2O), neuromuscular blockade, and administration of inotropes

A, Chest radiographs were obtained on the morning of postoperative day 10 before (left) and after (right) the onset of pulmonary edema

B, An electrocardiogram shows nonspecific ST changes and T-wave inversion


A man in his 30s presented with a steady 5-year progression of facial asymmetry and new-onset left-sided facial weakness, pain, and otalgia

Physical examination revealed no palpable parotid mass and no overlying skin changes

Neurologic examination showed incomplete left eye closure with a grade 5 of 6 on the House-Brackmann scale

Treatment with steroids and antiviral agents marginally improved facial strength and allowed eye closure

Magnetic resonance imaging showed subcutaneous nodules focally infiltrating the superficial parotid gland with evidence of perineural spread along the labyrinthine segment of CN VII

Fine-needle aspiration (FNA) with ultrasonographic guidance was nondiagnostic

An open excisional biopsy demonstrated dense subcutaneous tissue overlying and invading the parotid gland

Intraoperative frozen section revealed a spindle cell tumor

A superficial parotidectomy with facial nerve dissection and preservation was performed

Histopathologic examination revealed a subcutaneous mass with stellate extension into and around the parotid gland

The tumor consisted of bland fusiform tumor cells in a storiform pattern near inconspicuous hyalinized vessels (Figure, A and B)

Immunohistochemical staining demonstrated diffuse positivity for CD34 (Figure, C), positivity for bcl-2, and negativity for CD99 and S-100. Fluorescent in situ hybridization (FISH) further characterized the tumor with a translocation t(17

q13) and corresponding COL1A1/PDGFB fusion protein (Figure, D)

A, Tumor infiltrating around and within the parotid gland

B, Fusiform tumor cells arranged in a storiform pattern

C, Tumor cells strongly positive for CD34. D, Image from FISH studies


A young boy presented to the pediatric otolaryngology clinic with bilateral conductive hearing loss (CHL) and delayed speech development

Examination revealed normal otoscopic findings, and an audiogram demonstrated moderate low-frequency CHL of 45 dB in the right ear and mild 30-dB CHL in the left ear with a normal tympanogram (Figure, A)

Two sets of tympanostomy tubes were placed the year before his initial visit for presumed otitis media–related CHL, but his hearing did not improve

An axial computed tomographic (CT) scan performed at that time was interpreted as normal

Owing to concerns of visual changes and possible associated genetic cause for hearing loss (HL), magnetic resonance imaging (MRI) was ordered 1 year after initial presentation and showed no central or peripheral otologic abnormalities

A genetic evaluation revealed no abnormalities

Follow-up audiograms at 3 and 6 months showed similar persistent CHL despite normal otoscopy results

The patient and family decided to treat the CHL with bilateral hearing aid amplification and a frequency modulation (FM) system at school

After 30 months of persistent CHL with an unknown etiology, a repeated CT scan of the temporal bones was performed (Figure, B and C)

Operative intervention vs amplification was discussed, and the patient and family decided to continue with hearing aids because he had improved performance in school

Surgical treatment will be considered in the future if there is a decline in hearing

A, Right-sided (45-db) and left-sided (30-db) conductive hearing loss in low frequencies (arrows)

O and X indicate right and left air conduction, respectively

opening and closing brackets, right and left bone conduction, respectively

B and C, Axial computed tomographic (CT) scans of the temporal bones


A man in his 50s presented with intermittent dysphagia to solid foods, dry cough, foreign-body sensation in the throat, and dyspnea on effort for 4 months

His medical history included hypertension and chronic rhinosinusitis

On oral endoscopic examination, mild bilateral hyperemia of the tonsillar area and posterior pharyngeal wall were noted

Fiber-optic laryngoscopy revealed a reddish, 2-cm supraglottic lesion involving medially the free margin of the suprahyoid epiglottis (Figure, A)

The neoplasm appeared highly vascularized on narrowband imaging (Figure, B)

For this reason, before attempting an incisional biopsy, contrast magnetic resonance imaging (MRI) was performed

The MRI scan revealed an oval, 2.2-cm hypervascular mass arising from the free border of the suprahyoid epiglottis, which extended along the right aryepiglottic fold

The mass appeared to be capsulated, with regular margins, and did not show laryngeal deep tissues invasion

We proposed to the patient a surgical excision that could have been transoral or open according to the quality of exposure (which would be evaluated in general anesthesia)

After evaluating the optimal exposure using a Crowe-Davis retractor, a transoral excision using the da Vinci robotic system was performed

The postoperative course was uneventful, and patient did not develop complications

On the second postoperative day, pain was fully controlled with oral medications, and the patient tolerated oral intake

He was discharged on the second postoperative day

A, Endoscopic examination of the supraglottic larynx revealing reddish supraglottic neoplasm arising from the free epiglottic border

B, Narrowband imaging of highly vascularized supraglottic neoplasm


A man in his 20s presented for further evaluation of an elevated serum ionized calcium level (6.28 mg/dL

reference range, 4.56-5.32 mg/dL) found incidentally during a routine examination 5 months prior

He had a several-year history of joint, back, and abdominal pain

Laboratory workup revealed a normal intact parathyroid hormone (PTH) level (50 pg/mL

reference range, 13-88 pg/mL) and a slightly elevated 24-hour urinary calcium level (314 mg/d

A technetium Tc-99m sestamibi scan showed no definitive lesion but demonstrated a small focus of possible increased tracer uptake near the mid-pole of the left thyroid lobe (Figure, A, arrowheads)

No abnormal masses were readily identifiable by ultrasonography or contrast-enhanced computed tomography (CT) of the neck

He underwent a parathyroid exploration and excision of a soft, ovoid tissue, believed to be a slightly enlarged left inferior parathyroid gland (120 mg

Intracytoplasmic fat vacuoles were evident on intraoperative touch preparation

Histological examination of permanent sections showed a thinly encapsulated parathyroid tissue (Figure, B) with approximately 30% displaying myxoid stromal change and focal presence of interspersed adipocytes (Figure, C, yellow arrowhead) bordered by a rim of compressed gland containing abundant stromal fat (Figure, C, blue arrowhead)

Examination at higher power showed chief cells in lobules, nests, and focal trabeculae with occasional clusters of clear and oxyphil cells (Figure, C and D)

A, Technetium Tc-99m sestamibi scan with sodium iodide I-123 subtraction

Columns 2 to 4 show delayed images at 30 minutes, 1 hour, and 2 hours

B-D, Histopathologic images (hematoxylin-eosin) of thinly encapsulated parathyroid tissue


Primary cutaneous anaplastic large-cell lymphoma

Transformed mycosis fungoides and Sézary syndrome

A man in his 30s with no medical history reported a 2-year history of waxing and waning pruritic papules involving his left popliteus, contralateral popliteus, groin, and penis with each lesion occurring at different time points

He was initially evaluated by an infectious disease physician and a local dermatologist, who had intermittently prescribed empirical antibiotics (clindamycin hydrochloride and doxycycline calcium) for a presumptive clinical diagnosis of methicillin-resistant Staphylococcus aureus (MRSA) infection for more than 1 year with minimal response

Because no objective data including wound culture or biopsy were obtained during his prior evaluations, he sought an alternate clinical consultation given the appearance of new violaceous ulcerated lesions over his right lateral proximal calf (Figure, A) despite his actively taking doxycycline

He endorsed spontaneous healing of previously involved sites despite the absence of antibiotic exposure but denied constitutional complaints, recent travel, or high-risk behavior

His physical examination was unremarkable for hepatosplenomegaly or systemic adenopathy

Laboratory evaluation including complete blood cell count, peripheral blood cultures, MRSA swab, human immunodeficiency virus/human T-cell lymphotropic virus testing, flow cytometry, and Lyme disease titers had unremarkable results

Pathologic sampling of the lesions revealed V-shaped perifascicular infiltrative large lymphocytes with abundant amphophilic cytoplasm and prominent nucleoli (Figure, B and C)

Lesional cells exclusively involving the dermis stained positive for CD30, leukocyte common antigen, CD2, TIA-1, and CD4 and negative for CD7, CD8, CD43, CD56, and programmed cell death protein 1. Scattered B lymphocytes were PAX5 positive and CD20 positive

Scattered dermal dendritic and Langerhans cells were S100 positive

T-cell receptor (TCR) gene rearrangement testing had negative results

Systemic imaging did not reveal extracutaneous involvement, and screening endoscopy for upper and lower gastrointestinal involvement had unremarkable results

A, Photograph of right lateral proximal calf shows violaceous ulcerated nodule

B and C, Biopsy of the lesion revealed V-shaped perifascicular infiltrative large lymphocytes with abundant amphophilic cytoplasm and prominent nucleoli hematoxlyn-eosin, original magnification ×2 and ×20, respectively)


A man in his 40s presented with a 2-year history of multiple asymptomatic lesions on the face and neck

The lesions were round, hypopigmented macules and were symmetrically distributed in the affected area

The patient reported that the macules had appeared on his cheeks 2 years earlier and had gradually coalesced (Figure)

The size of the lesions ranged from approximately 2 to 3 mm to 25 mm in diameter

There was no hair growth at the site of the lesions, and the patient reported no symptoms

He was treated for tinea versicolor, without success

A skin biopsy was performed to rule out some kind of morphea

A, Hypopigmented macules on the face and neck

B, Absence of hair growth in the affected area

C, Hematoxylin-eosin staining (original magnification ×10)

D, Hematoxylin-eosin staining (original magnification ×20)


Antimelanoma differentiation associated gene 5 clinically amyopathic dermatomyositis

A 42-year-old African American male presented with a 7-month history of fatigue, exertional dyspnea, arthralgias, hand swelling, tender ulcerations, hair loss, and a 50-pound weight loss

He had been hospitalized 6 months prior for hypoxia

Chest computed tomography (CT) showed hilar lymphadenopathy, interstitial thickening, and perilymphatic ground glass opacities

Bronchoscopy revealed plaquelike lesions, and transbronchial nodal aspiration of mediastinal nodes was negative for carcinomas and/or granulomas

Prednisone was initiated for presumed sarcoidosis

Antineutrophil cytoplasmic antibodies, antinuclear antibodies, angiotensin-converting enzyme, rheumatoid factor, and Aspergillosis antigen were negative and/or normal

The patient was referred for a second opinion

On presentation he had symmetric swelling of metacarpophalangeal joints (MCPs) and interphalangeal joints (IPs), preventing fist formation

Dorsal MCPs had ulcers distal fingers were dyspigmented and atrophic, capillaroscopy was normal, and palmar IP creases had ulcerated lichenoid papules (Figure, A and B)

Oxford Scale muscle strength was 5 of 5 throughout

He had normal aldolase levels (4.5 U/L [to convert to μkat/L, multiply by 0.0167]), depressed creatine kinase (32 U/L), hyperferritinemia (1543 ng/mL), and T lymphocytopenia (absolute CD3, CD4, and CD8 counts, 328 μL, 233 μL, and 85 μL, respectively)

the patient also had anti-SSA (Ro) antibodies, and immunoglobulin G anticardiolipin antibodies (19.5 units)

The patient tested negative for HIV, anti–Scl-70 antibodies, anti–β2-glycoprotein 1 antibodies, lupus anticoagulant, cryoglobulins, and anti–Mi-2 antibodies

prothrombin time, partial thromboplastin time, and tissue thromboplastin were normal

A, Edematous hands with crusted ulcers and surrounding hyperpigmentation overlying dorsal metacarpophalangeal joints and interphalangeal joints

B, Ulcerating papules with crust located in the palmar creases of the priximal interphalangeal joints and distal interphalangeal joints


A man in his 60s presented with a 2-month history of painful, occasionally pruritic, fixed erythema on the abdomen

In addition, the patient suffered from a splenic marginal zone lymphoma with bone marrow infiltration, had a history of splenectomy, and an IgM-κ paraproteinemia

His hematologic condition was considered stable by his physician, who had also been treating the patient for hepatitis C with ledipasvir-sofosbuvir for 3 months

On examination, there was a warm erythema on most of the abdomen, which appeared distended owing to hepatitis-induced ascites (Figure, A)

Laboratory results showed an elevated but stable white blood cell count of 19.04 × 103µL (reference range, 3.6-10.5 × 103µL), a recent rise of C-reactive protein levels to 179 mg/L (reference range, 0-5 mg/L), as well as a rise of IgM levels from 1060 mg/dL to 2220 mg/dL (reference range, 34-248 mg/dL) within the past year

A biopsy procedure was performed on the abdomen (Figure, B-D)

A, Light red erythema on most of the abdomen

B, Interstitial infiltrate of deeply eosinophilic cells in the entire dermis and adjacent subcutis

Hematoxylin-eosin staining (original magnification ×2)

C, Hematoxylin-eosin staining (original magnification ×40)


A man in his 30s presented with a 5-day history of vomiting, followed by lethargy and confusion

Two years prior, he was hospitalized with coma with no identifiable precipitant and an extensive workup done at that time only revealed an elevated ammonia level of 233 µg/dL (to convert to µmol/L, multiply by 0.714)

etiology remained unclear but he improved with lactulose

Prior to this admission, he was working in a restaurant, did not ingest any drugs or mushrooms, and had no operations and no family history of similar problems

He was found to have hyperammonemia to 239.5 µg/dL and respiratory alkalosis (pH level, 7.482

partial pressure of carbon dioxide, 27.3 mm Hg, and partial pressure of oxygen, 109 mm Hg [to convert to kPa, multiply by 0.133]

and bicarbonate, 20.2 mEq/L [to convert to mmol/L, multiply by 1])

Liver and kidney function were normal (protein, 6.4 g/dL, and albumin, 3.4 g/dL [to convert to g/L, multiply by 10]

direct bilirubin, 0.1 mg/dL, and indirect bilirubin, 0.8 mg/dL [to convert to µmol/L, multiply by 17.104]

alkaline phosphatase, 38 U/L, aspartate aminotransferase, 22 U/L, and alanine aminotransferase, 42 U/L [to convert to µkat/L, mutiply by 0.0167])

His mental status rapidly declined despite treatment with lactulose, necessitating intubation

Head computed tomography and cerebrospinal fluid analysis were unrevealing

Ammonia levels progressively increased to 1120.5 µg/dL, requiring hemodialysis and sodium phenylbutyrate, which did not lead to significant clinical improvement

On hospital day 6, he developed continuous myoclonus due to status epilepticus (Figure 1A), which was refractory to treatment with lorazepam and levetiracetam as well as continuous midazolam, lorazepam, and propofol infusions

Plasma amino acids and urine organic acids were requested

Continuous venovenous filtration hemodialysis and carnitine treatment were started because of concern for inborn errors of metabolism

Within a few hours of transfer, his ammonia level peaked to 1960 µg/dL and he lost all brainstem activity due to diffuse cerebral edema (Figure 1B)

A, Electroencephalography (EEG) showing diffuse polyspike complexes consistent with myoclonic seizures

B, Head computed tomography showing diffuse cerebral edema, obliteration of basal cisterns, and transtentorial and tonsillar herniation


A previously healthy 7-year-old girl presented with fever, sore throat, bilateral conjunctival hemorrhage, periorbital edema, and eyelid ecchymoses (Figure)

The illness began 1 week prior with low-grade fever and back pain

The following day, she developed periorbital swelling and subjective high fever

The back pain resolved, but she complained of fatigue, stiffness in her limbs, and sore throat

A rapid antigen detection test from a throat swab was positive for group A Streptococcus, so she was given an “antibiotic shot,” presumably penicillin

She developed a mild cough and had several episodes of vomiting and diarrhea

She presented for reevaluation when she developed eyelid ecchymoses and conjunctival hemorrhage

Four days prior to the onset of illness, she had completed a 10-day course of amoxicillin for acute otitis media

A 7-year-old girl with periorbital edema, ecchymosis, and bilateral conjunctival hemorrhage

A physical examination revealed a febrile patient with bilateral conjunctival hemorrhages, periorbital edema, and eyelid ecchymoses

She had tonsillar hypertrophy with erythema and exudates

Her liver and spleen were mildly enlarged

Laboratory tests showed leukocytosis with a white blood cell count of 16.7 000/μL (to convert to ×109 per liter, multiply by 0.001) with a predominance of lymphocytes, mild thrombocytopenia (platelet count, 108 × 103/μL [to convert to ×109 per liter, multiply by 1.0]), elevated aminotransferases (alanine aminotransferase, 232 U/L [to convert to microkatals per liter, multiply by 0.0167]

aspartate aminotransferase, 261 U/L [to convert to microkatals per liter, multiply by 0.0167]), γ-glutamyltransferase (265 U/L), conjugated bilirubin (2.3 mg/dL [to convert to microkatals per liter, multiply by 0.0167]), and lactate dehydrogenase (2116 U/L [to convert to microkatals per liter, multiply by 0.0167])

A throat culture grew group A Streptococcus


Blunt traumatic rupture of the diaphragm

A man in his early 40s was referred for surgical evaluation following abnormal imaging obtained during workup of recent left-sided chest discomfort and longstanding dyspnea on exertion

A large air-fluid level was seen on a recent chest roentgenogram, and subsequent computed tomographic scans of his chest were obtained (Figure 1)

The only medical history reported was a previous umbilical hernia repair

On further discussion, it was discovered that he was involved in a motorcycle crash many years ago

Other than a brief loss of consciousness, no significant injuries were sustained per the patient’s report

He did undergo a formal trauma evaluation at that time

however, the radiographic images and medical records were no longer available

In addition to complaints of dyspnea and chest discomfort, the patient reported 5 to 6 episodes of small-volume emesis over the last 6 months

He denied any other gastrointestinal symptoms, such as heartburn, dyspnea, or constipation

The results of his examination were unremarkable, with the exception of bowel sounds being heard on auscultation of the left chest


A 61-year-old man presented with a progressive shortness of breath for 1 week

He was diagnosed with myocardial infarction 1 month earlier at another hospital and a coronary intervention was performed with 1 drug-eluted stent put in the left anterior descending artery

Progressive dyspnea at rest was noted in recent days

Review of systems and family history were unremarkable

Physical examination revealed tachycardia and tachypnea

His heart rate was 138 beats/min and respiratory rate, 25 breaths/min

Chest auscultation revealed bilateral rales

Heartbeat was regular, but with faint S1 and S2. The extremities were cold, while no pitting edema was found

Laboratory findings were within normal limits

Twelve-lead electrocardiography demonstrated persistent ST-T segment elevation in V2 to V6 leads

Transthoracic echocardiography showed a hypoechoic mass around the apical region (Figure 1A)

Computed tomography of the chest revealed a large crescentic hypodense lesion surrounding the anterolateral wall of the left ventricle and apex (Figure 1B)

A, Transthoracic echocardiography, apical 4-chamber view, showing a hypoechoic mass (arrowhead)

B, Computed tomography of the chest, axial view, showing a hypodense lesion surrounding the anterolateral wall of the left ventricle and apex (arrowhead)


A woman in her 30s presented with a 3-month history of painful swelling of the left hard palate and cheek

The growth also caused malocclusion and ipsilateral upper lip numbness

no difficulty speaking, breathing, or swallowing

no epistaxis, nasal congestion or nasal drainage

She did have a history of recent pericoronitis, but several courses of antibiotics were unhelpful

Physical examination revealed a submucosal growth centered on the mesial aspect of the left maxillary alveolus

Contrasted computed tomographic and magnetic resonance imaging showed a heterogeneously ossified lesion filling the left maxillary sinus, with local destruction of hard palate and alveolar bone (Figure, A and B)

Positron emission tomographic scans highlighted the fluorodeoxyglucose-avid nature of both the tumor and associated cervical lymphadenopathy

A biopsy showed a tumor with lobular architecture, an incomplete peripheral ossified rim (Figure, C), and focal areas of osteoid matrix

Higher power showed atypical cells with increased mitotic activity in a myxoid stroma (Figure, D)

Immunostains were negative for S100, pankeratin AE1/AE3, SMSA, and GFAP

A, Computed tomographic (CT) image of the head, coronal view

B, Magnetic resonance image (MRI) of the face, coronal view with contrast


A man in his 60s presented with positional shortness of breath and sensation of airway obstruction of 3 to 4 months’ duration, with acute increase in symptoms over 4 days

He described the need to cough to clear the airway, but coughing provided no relief

He noted some relief when bending at the waist or lying prone

Flexible fiber-optic laryngoscopy revealed a large, irregular pedunculated mass on the left anterior false vocal fold (Figure, A and B)

The mass was seen to ball-valve in and out of the glottis with respiration

The position of the mass was discussed with the anesthesiology team, who felt comfortable performing prone intubation

Preparations for emergency tracheotomy were made

In the operating room, with the patient, the anesthesia unit performed flexible fiber-optic laryngoscopy and advanced the endotracheal tube into the trachea over the flexible bronchoscope

A video laryngoscope was used to ensure that advancing the tube would not shear the mass

The patient was turned supine, and microdirect laryngoscopy was performed

The mass originated from a stalk in the left anterior ventricle

Pathologic examination (Figure, C and D) revealed a 1.7 × 1.3 × 1.0-cm mass with a papillary-like architecture, expansion of the submucosa by benign mature adipose tissue, and variable amount of fibrous tissue

There was mild chronic submucosal inflammation without squamous epithelial proliferation

B, Direct laryngoscopy with retraction of mass to view stalk

C, Hematoxylin-eosin, original magnification ×40. D, Hematoxylin-eosin, original magnification ×200.


Intrasphenoid pseudoaneurysm of the internal carotid artery

Hypervascular metastasis from thyroid cancer

A woman in her 40s had experienced dizziness and headache to the vertex with left retro-orbital irradiation for 1 year

She referred only to minor head trauma 15 years earlier

Findings from a physical examination were negative, while nasal endoscopy showed diffuse mucous rhinorrhea

A CT scan of the paranasal sinuses showed complete opacification of the left sphenoid sinus, a double-lined appearance of the sinus walls (an inner bony sclerotic layer and a thin external one) (Figure, A), and 2 bony defects of the sphenoid sinus walls next to the cavernous sinus along the floor (Figure, B)

An MRI scan showed a concentric multistratified appearance of the sinus content

On T2-weighted images (Figure, C), a hyperintense line peripheral layer consistent with fluid retention, an irregularly thick hypointense line, consistent with the sclerotic bone visible on CT, a crescent-shaped heterogeneous material in the lower part, and a black oval area in the upper part of the sinus were identified

After gadolinium administration, volumetric interpolated breath-hold examination (VIBE) sequences revealed very intense enhancement of the oval black area in the upper part of the sinus, with no contrast enhancement of the remainder of the sinus (Figure, D)

On paracoronal images, there was no cleavage plane between the hyperenhancing area in the sphenoid sinus and the bright signal of the internal carotid artery (ICA) in the cavernous sinus


Angiolymphoid hyperplasia with eosinophilia

Sclerosing mucoepidermoid carcinoma with eosinophilia

A man in his 30s from Burma presented with a slow-growing right facial mass after resection 14 years prior

His medical history was significant for atopic dermatitis

He had no previous treatment with radiation or immunosuppressive medications

On examination, he had a surgical scar, an indurated malformed auricle, a superficial right parotid mass infiltrating the overlying skin, and a 1.5-cm postauricular node

Postgadolinium magnetic resonance imaging (MRI) showed infiltration into the cartilaginous tissue of the external auditory canal and ipsilateral cervical adenopathy (Figure, A)

Serum laboratory test results revealed an eosinophil count of 1109 cells/μL (reference range, 50-350 cells/μL), and an IgE level of 5.44 μg/mL (reference range, <0.27 μg/mL)

Flow cytometry was negative for signs of leukemia or lymphoma

Fine-needle aspiration (FNA) revealed a mixed leukocyte population and was negative for malignant neoplasm, necrosis, and granulomatous disease

An excisional biopsy of the postauricular node was performed

Within the lymphoid tissue there was prominent follicular hyperplasia and a diffuse infiltrate of eosinophils (Figure, B)

There was an increase in small blood vessels within the germinal centers

While some follicles had well-defined mantle zones, others demonstrated a spectrum of lytic changes accompanied by intrafollicular infiltrates of eosinophils (Figure, C)

The stroma was densely fibrotic with perivascular sclerosis (Figure, D)

A, Magnetic resonance image (MRI) of a right parotid mass and pathologic preauricular and retroauricular lymph nodes

B-D, Histopathologic images, hematoxylin-eosin


A man in his 20s presented with a 5-week history of hoarseness and foreign body sensation in his throat

His voice was breathy and raspy, and he projected his voice poorly

He denied dyspnea, dysphagia, odynophagia, weight loss, and lymphadenopathy

He had no clinically significant medical, surgical, or social history

Flexible Lindholm laryngoscopy revealed a large, right-sided, supraglottic, submucosal mass expanding the aryepiglottic fold and false vocal fold with hypomobility of the right vocal fold (Figure, A)

The lesion extended posterolaterally to the aryepiglottic fold and obscured visualization of the glottis

The right laryngeal ventricle was effaced, but the mucosal surface of both true vocal cords appeared normal

A computed tomographic (CT) scan to determine the extent of the lesion revealed a cystic mass with no protrusion out of the thyrohyoid space or into the subglottic airway (Figure, B and C)

Intraoperatively, the lesion was fully visualized with a Lindholm laryngoscope (Figure, A)

The mass was completely removed using a pulsed potassium titanyl phosphate laser to make mucosal incisions just lateral to the aryepiglottic fold and cold instrumentation to manipulate and remove the mass

Strategic needle decompression and angled endoscopes facilitated complete visualization and removal of the mass

The paraglottic space was closed via endoscopic suturing

The patient tolerated oral intake and was discharged home within 24 hours

Considerable voice improvement and vocal fold mobility was noted at 6 weeks, with normal vocal fold movement, normal mucosal pliability, and complete glottic closure achieved at 1 year (Figure, D)


A woman in her 40s with relapsed grade 2 follicular lymphoma receiving therapy with idelalisib, 150 mg twice daily for the past 5 months, presented with 3 days of vomiting, cramping abdominal pain, and diarrhea

She initially had 10 to 12 watery bowel movements a day, which progressed to passage of bright red blood per rectum

She reported no sick contacts, recent travel, recent antibiotic exposure, or nutritional supplement intake

She denied use of tobacco, alcohol, or illicit drugs

Vital signs were notable for elevated temperature (37.9°C), tachycardia (heart rate, 112/min), respiratory rate of 16/min, and blood pressure of 105/60 mm Hg

Physical examination showed a soft nondistended abdomen without organomegaly and normal bowel sounds

She had diffuse tenderness to deep palpation

Laboratory evaluation revealed normal hematological indices, renal function, and hepatic function

Computed tomography of the abdomen and pelvis demonstrated colonic wall thickening without perforation and stable prior pelvic lymphadenopathy

No pathogenic organisms or parasitic ova were detected on stool culture

Results of a stool Clostridium difficile toxin assay were negative

Flexible sigmoidoscopy demonstrated grossly congested and erythematous mucosa in the rectosigmoid and descending colon

Histopathology from the biopsies of one of the affected areas is shown in the Figure


A woman in her 50s with poorly controlled type 2 diabetes and insulin-like growth factor 1 deficiency presented to the dermatology clinc reporting a 1- to 2-year history of continual outbreaks of “flowers” on her skin

The lesions were mildly pruritic and would typically occur in crops of 3 to 4 on the arms, legs, trunk, and buttocks

Individual lesions would last a few months before spontaneously resolving without scarring

The patient denied new or progressively worsening systemic symptoms, including fevers, chills, night sweats, or rapid unexpected weight loss

She had a history of basal cell skin cancer but denied a history of internal cancer, as well as new medications

Pertinent daily medications included metformin hydrochloride, sitagliptin phosphate, insulin glargine, somatostatin, and antihypertensives

Physical examination revealed an obese but otherwise healthy-appearing white woman with multiple small 1.5-cm monomorphic and somewhat octagonal groupings of folliculocentric, erythematous, scaly, and hyperkeratotic papules at different stages of development distributed across arms, legs (Figure, A and B), trunk, and buttocks

Skin examination otherwise had unremarkable results, including normal scalp, palms, soles, and nails, as well as no palpable cervical, axillary, or inguinal lymphadenopathy

Two scoop shave biopsies of representative lesions were performed (Figure, C and D)

Folliculocentric, octagonal, erythematous, and scaly papules on the left thigh

C and D, Shave biopsy specimen (hematoxylin-eosin)


A woman in her 30s presented with a 3-year history of painful nodules on her lower extremities, neck, and jawline

Her medical history included seasonal allergies, and she did not take any prescription medications

She felt well and denied weight loss, night sweats, and any other systemic symptoms

Physical examination revealed scattered skin-colored papules on the right neck, a solitary 8-mm skin-colored nodule on the right jawline, and three 2- to 3-cm pink firm nodules on the right thigh (Figure, A and B)

A shave biopsy was obtained from the nodule on the right jawline (Figure, C), and the thigh nodules were excised


Annular elastolytic giant cell granuloma

A woman in her 50s presented to the dermatology clinic with a 3-year history of a slightly pruritic steadily growing plaque on her right arm

She had consulted a dermatologist 18 months prior, who performed a biopsy of the lesion that was inconclusive

Her medical history was relevant for multiple consultations with several neurologists in the past 2 years for intermittent moderate-to-severe unilateral headache with an unsatisfactory response to nonsteroidal antiinflammatory drugs and amitriptyline

She was not taking any medication at the time of consultation

Clinical examination revealed a 12-cm-wide, soft, nontender plaque with a raised, nodular, and slightly desquamative erythematoviolaceous border and a clear, atrophic center with an increase in wrinkle depth on her upper right arm (Figure, A)

There were no other mucosal or skin lesions

Her neurological and mental state examination showed no abnormalities

No physical examination was performed specifically to the head and neck

A biopsy specimen that included the border of the lesion was obtained (Figure, B and C)

A, Annular plaque with raised borders and an atrophic and wrinkled center on the right arm

B, Hematoxylin-eosin stain (original magnification x20)

C, Orcein stain (original magnification x40)


A 1-day-old male infant in the neonatal intensive care unit was evaluated for a diffuse rash present at delivery

The patient was born at full term via spontaneous vaginal delivery after an uncomplicated pregnancy with APGAR scores of 8 and 9 at 1 and 5 minutes, respectively

His mother was varicella nonimmune and had no active rashes including in the perineal area

Physical examination of the patient revealed widespread papulovesicular, crusted lesions on the arms, legs, face, scalp, and trunk (Figure 1)

The palms, soles, and oral mucosa were spared

Further workup included Tzanck smear, direct fluorescent antibody test for varicella, herpes virus culture, syphilis IgG antibody test, fungal culture, and bacterial culture

While results were pending, the patient started empirical treatment with ampicillin, gentamicin, and acyclovir


Eroded gallstone into the abdominal wall

A 51-year-old man presented with a 2-day history of right upper quadrant abdominal pain

Ultrasonography of the abdomen’s right upper quadrant showed gallbladder wall thickening with gallstones in the neck of the gallbladder consistent with acute calculous cholecystitis

He subsequently underwent a laparoscopic cholecystectomy

The critical view of safety1  was obtained, and the gallbladder was removed from the fossa without difficulty

On extraction of the gallbladder through the umbilical incision, a large stone in the gallbladder could be palpated

After several attempts of extraction, the retrieval bag perforated and stones spilled into the abdomen

Not all the spilled stones could be retrieved

The patient had an uncomplicated postoperative course and was discharged to home on postoperative day 2. Two weeks after surgery, he was evaluated for persistent mild abdominal pain in clinic

Computed tomography (CT) of the abdomen showed a 2.5-cm calcified mass with adjacent inflammatory changes in the left hemiabdomen that was most consistent with a lost gallstone (Figure 1A)

He returned to the emergency department 1 month later with a 2-day history of progressive abdominal pain, nausea, and vomiting

Results of a physical examination revealed mild abdominal tenderness in the left lower quadrant

A second abdominal CT was performed (Figure 1B)

A, Postoperative computed tomography (CT) of the abdomen demonstrating a lost gallstone in the left hemiabdomen (arrowhead)


A young adult presented with a progressively growing abdominal lump that left him feeling a dull ache and heavy

Ten weeks previously, he was hospitalized and conservatively managed for acute pancreatitis

He had a history of prolonged alcohol abuse

A physical examination revealed stable vital signs and a normothermic, nontender, nonpulsatile, fixed lump in his upper abdomen (Figure, A)

A routine blood test revealed leukocytosis (white blood cell count of 12 000/μL[to convert to ×109 per liter, multiply by 0.001]), and his liver and kidney function test results were unremarkable

His serum amylase level was 487 U/L (normal range, 28-100 U/L [to convert to microkatals per liter, multiply by 0.0167])

A contrast-enhanced computed tomography (CT) scan of his abdomen was performed (Figure, B)

Fixed and nonpulsatile abdominal lump (A) and contrast-enhanced computed tomography (CT) scan of the abdomen (B) (axial view)

The scan reveals a homogeneous collection of fluid (black arrowhead) encysted by a well-defined enhancing wall (yellow arrowhead)


An ex utero intrapartum treatment (EXIT) procedure was performed for a neonate born by cesarean delivery at 37 weeks of gestation with a prenatally diagnosed neck mass

Initial laryngoscopy with a Parsons laryngoscope identified airway deviation secondary to mass effect, with intubation ultimately achieved with assistance of a bronchoscope

Examination revealed a large neck mass extending from the mandible to the clavicles, with mass effect causing neck extension and rotation

The mass was multicystic, with prominent vasculature, and a small area of skin necrosis

Following birth, the mass continued to grow, with inability to palpate the sternal notch by day of life 4 (Figure, A)

The patient underwent a magnetic resonance image (MRI) on day of life 3 which showed a 15.7 × 10.0 × 14.4-cm midline neck mass with solid and cystic components

Flow voids were present throughout the mass

The mass was isointense to hypointense on T1, and isointense to hyperintense on T2. The patient also underwent a computed tomographic (CT) scan, which demonstrated calcifications present within the multicystic neck mass

Owing to continued growth and risk of airway compromise, the patient was taken to the operating room on day 5 of life for resection of the mass and tracheostomy

Pathologic results showed a multinodular 19-cm mass, 80% solid and 20% cystic, with multiple tissue types present (Figure, B-D)


A man in his 50s presented with a 5-year history of a left jaw mass discovered incidentally on a computed tomographic (CT) image

Fine-needle aspiration was interpreted as a “well-differentiated squamous cyst

” Routine laboratory test results and hematologic markers were normal

Examination revealed a 5-cm, minimally tender mass deep to the left parotid gland

Cranial nerve examination findings were normal, and no overlying skin or mucosal changes were noted

CT imaging (Figure, A and B) revealed a well-defined hypodense mass within the left masticator space measuring 5.0 × 3.5 cm, resulting in smooth remodeling of the left mandibular ramus

The second left mandibular molar appeared to be missing

On magnetic resonance imaging (MRI), the lesion was T2 hyperintense, mildly T1 hyperintense, without enhancement, with remodeling of left mandibular alveolar ridge

No definite site of mandibular or maxillary attachment was noted (Figure, C and D)

He underwent resection via a combined transoral and transantral (Caldwell-Luc) approach

The mass was noted to be associated with an empty socket of the second mandibular molar, was not well circumscribed, and was notably adherent to some surrounding tissues, requiring meticulous dissection from the inferior alveolar nerve, temporomandibular joint capsule, and skull base

Pathologic assessment demonstrated a squamous lined cyst with parakeratosis

Imaging of the face of a patient with a left jaw mass

A and B, Computed tomographic (CT) images


A woman in her 70s had a 2-week history of frontal headaches and forehead nodules (Figure, A) with no associated fevers, vision changes, or neurologic symptoms

Her medical history was significant for extensive craniofacial polyostotic fibrous dysplasia (FD), breast adenocarcinoma, and stage IV clear cell renal cell carcinoma with metastasis to the ipsilateral adrenal gland treated with radical nephrectomy

Physical examination was remarkable for soft, tender nodules at the forehead and right orbit with right proptosis

Neurologic examination results were normal, except for hypesthesia in a V1 distribution

Sinus computed tomography showed a soft-tissue mass with involvement of multiple craniofacial bones with orbital, anterior, and posterior table defects

Magnetic resonance imaging with contrast (Figure, B) showed interval growth at the frontal bones in the region of previously diagnosed FD

A fine-needle aspiration (FNA) biopsy established the diagnosis

Romanowsky-stained direct smears (Figure, C) showed loose billowing clusters of epithelial cells involved by strands of magenta extracellular tissue (black arrowhead)

Cytoplasm was abundant with coarse and fine textures

Nuclei were large and round, occasionally with conspicuous nucleoli (blue arrowheads)

Hematoxylin-eosin staining (Figure, D) showed cells with abundant clear cytoplasm and nuclei with varying nucleolar prominence (blue arrowheads)

Immunohistochemical stains were positive for pancytokeratin, vimentin, CD10 and PAX-8. A carbonic anhydrase IX (CA-IX) stain was noncontributory

C, Original magnification ×400. D, Original magnification ×40.


Basal cell carcinoma and squamous cell carcinoma

Eccrine syringofibroadenoma and squamous cell carcinoma

Eccrine poroma and squamous cell carcinoma

A man in his 70s presented with a 3- to 4-month history of a small cutaneous lesion on his left temple

The lesion was growing in size and had begun to drain

Prior to the visit, he had been evaluated in emergency care for the same complaint and was given a prescription of oral antibiotics

Afterward he was referred to a local dermatologist, who performed a biopsy of the lesion

His medical history was significant for emphysema but no previous skin cancers or surgery

On physical examination, the patient had a 2.5 × 2-cm, singular, umbilicated, ulcerated papular lesion of the left temple with overlying crusts and surrounding erythema and induration (Figure, A)

Computed tomography with contrast of the soft tissue revealed an abnormal mass in the site of the left temporal scalp lesion

The mass appeared homogenous and was not particularly enhanced by contrast (Figure, B)

There was no accompanying surrounding lymphadenopathy

A preoperative biopsy and low-power histological analysis revealed an epidermal proliferative process

vertically oriented anastomosing strands of basaloid epithelium were seen arising from multiple points along the epidermis (Figure, C)

In this case, there was concurrent well-differentiated carcinoma (Figure, C)

Ductal differentiation was observed on high power, and the mass had an intervening fibrovascular stroma (Figure, D)

B, Computed tomographic image with contrast, soft-tissue window

C and D, Micrographs of tissue biopsy, hematoxylin-eosin

C, Original magnification ×4. D, Original magnification ×40.


A man in his 20s was evaluated for a 9-month history of a nonhealing submental ulcer that formed where a laceration caused by an assault had recently closed

The area bled, expressed serosanguineous drainage, and was prone to crust formation

He reported poor dental health and no exposure to tuberculosis

He smoked cigarettes daily and consumed alcohol socially

Significant medical history included excision of a mass at the ulcer site 8 years before that healed uneventfully

Surgical pathologic examination revealed areas of histiocytes surrounded by lymphocytes and a focus of central necrosis

Acid fast bacilli and fungal stains and cultures were negative

There was a 1.5 × 1.5-cm ulcerated, submental lesion within the mid-portion of a scar

It was nontender, bled with manipulation, and appeared to be fixed to the underlying mandible

Examination of the oral cavity revealed caries and periodontal disease

There was no palpable cervical adenopathy

The patient received a course of antibiotic therapy

When symptoms persisted, he underwent a biopsy

Because the biopsy was concerning for malignancy, an excision with frozen section margins was then performed

The lesion was present within old scar tissue and had an infiltrative growth pattern

Microscopically there were epithelioid or spindle-shaped tumor cells with atypical vesicular nuclei and pale eosinophilic cytoplasm with areas of necrosis (Figure, A)

Tumor cells stained strongly positive for EMA, CD34, and pankeratin and demonstrated loss of INI-1 immunostaining (Figure, B and C)

B, Strong cytoplasmic staining with cytokeratin AE1/AE3 immunostain


A teenage girl presented to the emergency department with hand and foot swelling and pain in areas of sun exposure after playing golf outside

She had a history of similar yet milder skin eruptions with sun exposure over the prior 2 years

No other family members were similarly affected

The physical examination findings were notable for acral edema and well-demarcated, tender petechial patches on the dorsal surface of the hands and feet (Figure, A and B)

A punch biopsy specimen from the affected area of her hand was obtained (Figure, C and D)

In the clinical images, edema with petechial eruption is evident on the hands (A) and feet (B)

In the pathologic images, the hematoxylin-eosin–stained specimens from affected areas of the dorsal aspect of the hands show subtle thickening of vessel walls (C), in addition to extravasation of erythrocytes and fibrin thrombus formation (D)


Multiple mucosal neuromas in multiple endocrine neoplasia type 2b

An adolescent girl with no relevant family history was referred to the dermatology pediatric unit for evaluation of multiple asymptomatic lesions on her tongue that had gradually developed since the first years of life

The patient had a marfanoid habitus, disproportion of limbs, and an enlarged face

Both lips were enlarged and felt nodular (Figure, A)

Multiple pink-whitish and translucent nodules were observed on the lateral and anterior surface of the tongue (Figure, B)

The rest of her physical examination did not reveal other cutaneous abnormalities, except for mild acne

Biopsy results from tongue tissue are shown in the Figure, C and D

A, Both upper and lower lips were enlarged and felt nodular

B, Oral examination revealed the presence of multiple small nodules located on the anterior and lateral surface of the tongue

C and D, Histopathological findings from one of the nodules located on the tongue


A 32-year-old male with a history of end-stage liver disease secondary to alcoholic cirrhosis was transferred to our hospital with a 2-week history of a skin rash that developed at an outside facility where he was being treated for hepatic encephalopathy

He reported that the lesions began on his face, then spread to his thighs and were mildly pruritic

He did not have any other involvement and was in no pain

On physical exam, multiple 1 to 3 cm purple papules with central black and hemorrhagic crusting were noted on his forehead, bilateral cheeks, and anterior thighs (Figure 1, A and B)

On his left flank, he had a large polycyclic, atrophic hyperpigmented plaque with a slightly erythematous and scaly border (Figure 1C)

The scalp, oral mucosa, back, chest, abdomen, arms, palms and soles were all spared

Multiple toenails were noted to be dystrophic with hyperkeratotic subungual debris

Over the next few days, the crusted papules and nodules eroded leaving smooth erythematous plaques with punched-out centers

Testing for the human immunodeficiency virus (HIV) and hepatitis was negative

Punch biopsy specimens from the left anterior thigh were taken for histopathologic analysis and fungal, bacterial, and atypical mycobacterial tissue cultures

A skin culture was obtained of the polycyclic plaque on his left flank

A, Discrete and coalescing papules and nodules on the forehead and cheeks with central black and hemorrhagic crust

B, Morphology on the thighs was similar to the face with friable, firm purple crusts overlying smoother dermal plaques

C, Left flank with a large polycyclic, atrophic hyperpigmented plaque with a slightly erythematous and scaly border


An 80-year-old man presented to the hospital with new-onset slurred speech and left-sided facial droop

Magnetic resonance imaging scans of the head were visually interpreted to show an acute infarct involving the posterior right frontal lobe (Figure 1)

An echocardiogram did not reveal evidence of an embolic source

The patient was discharged from the hospital 2 days later and was treated with aspirin and clopidogrel bisulfate

Sixteen days later, the patient presented to the hospital again with a 2-day history of numbness and weakness in his left arm

His medical history was significant for hypertension, hyperlipidemia, coronary artery disease after placement of a stent 7 years prior, right-lung empyema with decortication 8 years prior, and chronic obstructive pulmonary disease

His family history was negative for neurological disease but positive for myocardial infarction and colon cancer

He had a remote smoking history of 10 pack-years and no history of alcohol or illicit drug use

General physical examination findings were noncontributory

Significant neurological examination findings included fluent but dysarthric speech, dysphagia, marked left facial droop, and difficulty with multistep commands

A motor examination revealed decreased left upper extremity strength (3/5 proximally and 4/5 distally), conspicuous loss of fine motor coordination, dysmetria, and dysdiadochokinesis

Hemogram and inflammatory markers were within normal limits (white blood cell count of 5600/μL [to convert to ×109 per liter, multiply by 0.001], erythrocyte sedimentation rate of 0 mm/h, and C-reactive protein level of 5 mg/L [to convert to nanomoles per liter, multiply by 9.524])

Carotid arteries were unremarkable via ultrasonography

Magnetic resonance imaging of the brain demonstrated an intra-axial necrotic lesion with adjacent vasculogenic edema primarily involving the right posterior frontal and anterior parietal lobes

Magnetic resonance imaging (MRI) of the brain at the time of initial presentation revealing hyperintensity in the right posterior frontal lobe (A) and hypointensity involving the right posterior frontal lobe (B)

FLAIR indicates fluid-attenuated inversion recovery


An 84-year-old woman presented to the emergency department with 2 days of progressive abdominal distention and nausea

She also had anorexia, and she had crampy abdominal pain during the past few days

Similar symptoms occurred previously but were self-relieved

In triage, her body temperature was 37.3°C, and she had a normal heart rate

Physical examinations were notable for distended abdomen and hyperactive bowel sounds but negative for the peritoneal sign

She reported dull pain below the pelvic bone as we palpated gently over the abdomen

No gross abnormalities were present in the perineal area

Her medical history was unremarkable, except for hypertension, and she denied having any abdominal operations

Her laboratory results indicated the following: white blood cell count, 11670/μL (to convert to ×109/L, multiply by 0.001)

sodium level, 132 mEq/L (to convert to mmol/L, multiply by 1)

and creatinine, 0.6 mg/dL (to convert to µmol/L, multiply by 88.4)

Abdominal radiography revealed segmental dilatation of the small bowel

She was kept in the emergency department for observation

However, she experienced little improvement in her primary symptoms

Further evaluation with computed tomography of the abdomen and pelvis was performed (Figure 1)

A, Computed tomographic scan showing small-bowel ileus

B, Computed tomographic scan showing a mass lesion in the right lower pelvic wall


Retroperitoneal fibrosis (Ormond disease)

A man aged 54 years, who had not seen a physician for nearly 10 years, presented to the emergency department with a 1-month history of generalized weakness and bilateral lower extremity swelling that had progressed to his upper abdomen

Physical examination revealed at least 4 lower extremity edemas and an abdomen that was large, taut, and distended (Figure 1A)

A computed tomographic scan of the chest, abdomen, and pelvis was obtained (Figure 1B)

A, In addition to lower extremity edemas, the abdomen of the patient was markedly taut and distended

B, Representative computed tomography images with intravenous and oral contrast

Note the patient’s descending colon was severely displaced by the retroperitoneal mass and was located on the patient’s right side

The mass enveloped the left kidney without evidence of hydronephrosis


Pityriasis lichenoides et varioliformis acuta

An African American boy aged 7 years presented with right ankle pain, overlying soft tissue erythema, and swelling

He was diagnosed with cellulitis and prescribed 10 days of sulfamethoxazole and trimethoprim

Although his right ankle pain and swelling initially improved, he presented 1 week later with left ankle and right knee pain and swelling

He had a tender, nonerythematous, subcutaneous 1-cm nodule above the right olecranon

Laboratory results were notable for an elevated erythrocyte sedimentation rate of 44 mm/h, a C-reactive protein level of 0.84 mg/L (to convert to nanomoles per liter, multiply by 9.524), an antistreptolysin O titer of 1150 IU/mL, and an anti-DNase B titer of 961 U/mL

The results of the rapid strep test and throat culture were normal, but given his constellation of symptoms, he was diagnosed with acute rheumatic fever

Echocardiogram and electrocardiogram were normal, and he was treated with amoxicillin

Four days after discharge, he developed new painful skin lesions on his elbows, forearms, and legs

He remained afebrile but continued to complain of persistent left ankle and right knee pain

On examination, he had a multitude of skin lesions, including a raised follicular rash on both shins

painful flesh-colored raised nodules on the right knee, left index finger, and elbows

and painful raised circular violaceous papulonecrotic lesions with surrounding erythema and induration on the bilateral shins, left knee, right upper arm (Figure, A), and bilateral buttocks

Admission laboratory results included a hemoglobin concentration of 9.3 g/dL (to convert to grams per liter, multiply by 10), indicating normocytic anemia

a white blood cell count of 11 500/μL (to convert to × 109/L, multiply by 0.001), with 64% neutrophils

an elevated erythrocyte sedimentation rate of 46 mm/h

and a C-reactive protein level of 0.57 mg/L

Punch biopsy was obtained from a lesion (Figure, B), which showed a dermal neutrophilic infiltrate with normal Gram and Grocott methenamine silver stains

A, Painful raised circular violaceous papulonecrotic lesion with surrounding erythema on child’s right upper arm

Hematoxylin-eosin stain reveals dense neutrophilic infiltrate in the dermis as well as papillary dermal edema (original magnification × 40)


A young woman presented with a 4-month history of a 2.0-cm mass in her right supraclavicular region

She had a cough and fatigue at the time, but no other symptoms of upper respiratory tract infection at onset

She attributed her fatigue to staying up late studying and had not tried any treatments, including antibiotics

She denied any trauma, night sweats, fever, chills, weight loss, generalized pruritus or lymphadenopathy in other areas of her body, any voice change, dysphagia, odynophagia, hoarseness, hemoptysis, hematemesis, or weight loss

Her family history was positive for breast cancer in her mother, and the patient was a lifelong nonsmoker

Physical examination revealed palpable nontender, freely movable mass in the right supraclavicular region

Palpation of the parotid, submandibular glands, and thyroid gland did not reveal any suspicious masses or tenderness

No mucosal lesions, scars, or masses were found on intraoral examination, with clear oropharynx

Computed tomography revealed a 2.0-cm, right-sided supraclavicular mass just anterior and superior to the right subclavian vein with central patches of hypoattenuation (Figure, A)

Results from initial ultrasonography-guided fine-needle aspiration were nondiagnostic

Subsequent ultrasonography-guided core needle biopsy on supraclavicular aspirate showed a benign spindle cell proliferation arranged in a tissue-culture–like pattern with a myxoid to collagenous stroma (Figures, B and C)

Immunohistochemical stains demonstrated the cells to be positive for actin and negative for cytokeratin and S100 protein


A young boy presented with a 2-month history of a progressively enlarging right facial mass

The swelling persisted despite a course of oral antibiotics prescribed by the primary care team

His parents denied any recent fevers, night sweats, weight loss, pain, respiratory distress, nasal bleeding, or significant rhinorrhea

The physical examination revealed a right infraorbital and maxillary swelling, with deformity of the right lateral nasal dorsum

The remainder of the physical examination was unremarkable

Flexible fiber-optic sinonasal endoscopy revealed a normal appearance of the nasal mucosa with no obvious obstruction, mass lesion, rhinorrhea, or bleeding

Vision was grossly normal with no diplopia, and sensation of the right cheek was intact

A contrasted maxillofacial computed tomographic (CT) scan and magnetic resonance imaging (MRI) were performed, revealing an expansile right maxillary mass, associated with roots of the upper molar and premolar teeth, with erosion through the anterior maxillary wall and some thinning and displacement of the inferior orbital wall (Figure, A)

The lesion was excised via a sublabial approach using both open and endoscopic techniques for better visualization

Histopathologic analysis revealed a bland, monotonous proliferation of stellate spindled cells with a variable extracellular matrix ranging from loose myxoid (Figure, B) to fibromyxoid and collagenous (Figure, C)

There was also permeation of adjacent bone marrow spaces (Figure, D)

B-D, Histopathologic images (hematoxylin-eosin, original magnification ×4)


A woman in her 50s with a history of ulcerative colitis presented with severe diffuse sore throat associated with fevers, chills, night sweats, and neck swelling of 2 weeks’ duration

Results from a rapid strep test were negative, and observation was initiated for presumed viral pharyngitis

Progression of neck swelling led to empirical treatment for bacterial pharyngitis

A neck computed tomographic scan with contrast revealed large bilateral asymmetric conglomerations of necrotic cervical lymph nodes measuring up to 9 cm in the greatest dimension (Figure, A)

Four weeks after symptom onset, the patient was referred to the otolaryngology service

Initial evaluation, including fiber-optic laryngoscopy, revealed the presence of significant bilateral tender lymphadenopathy in the absence of any lesions in the upper aerodigestive tract

A core biopsy specimen yielded negative gram stain and aerobic and anaerobic bacterial, fungal, and acid-fast bacillus cultures

Polymerase chain reaction (PCR) of nodal contents was negative for Bartonella henselae

Necrotic skin changes and persistent purulent neck drainage through the needle biopsy site required incision and drainage as well as incisional biopsy of the left side of her neck

Operative findings included abundant purulent debris and considerable necrosis of skin and neck musculature

All the cultures once again returned with no growth

Final pathologic examination showed necrotizing granulomatous inflammation (Figure, B) with no monoclonal lymphocyte population on flow cytometry

Results from a test for human immunodeficiency virus and angiotensin-converting enzyme testing were negative

Francisella tularensis serum antibody titers were 1:640.


Granulomatosis with polyangiitis (Wegener granulomatosis)

A young African American man presented with a 6-month history of nasal congestion, facial pressure, purulent rhinorrhea, and hypo-osmia that had not improved despite multiple courses of antibiotics and steroids

His medical history was significant for chronic rhinosinusitis, allergic rhinitis, and asthma, for which he took fluticasone propionate and salmeterol (Advair) and albuterol

A review of systems revealed epiphora and ocular crusting for 4 months

Findings on nasal endoscopy included bilateral turbinate hypertrophy and significant nasal crusting

A computed tomographic (CT) scan of the sinuses revealed diffuse mucosal thickening consistent with pansinusitis (Figure, A)

All laboratory results from an autoimmune workup were normal for angiotensin-converting enzyme (ACE) level, antineutrophil cytoplasmic antibodies, antinuclear antibody, and immunoglobulin levels

The patient was lost to follow-up but returned several months later with 6 weeks of progressive bilateral parotid swelling (Figure, B)

His parotid glands were diffusely enlarged and tender, and his facial function was normal

Radiologically, no masses or sialoliths were seen (Figure, C)

His only associated symptoms were fevers and fatigue

A history of recent tuberculosis exposure was obtained

Test results for SSa/SSb levels, human immunodeficiency virus (HIV), polymerase chain reaction (PCR), mumps titers, rapid plasma reagin, amylase, cytomegalovirus PCR, and quantiferon testing were negative

A superficial parotid biopsy revealed noncaseating granulomatous inflammation (Figure, D)


A nonsmoking man in his 40s with hypertension and diabetes mellitus type 2 reported a 1-year history of a mass in the region of his left parotid gland that had been steadily increasing in size

There was no associated pain, facial numbness, or weakness

Multiple aspirations of the mass had been performed, which would temporarily decrease the size

On examination, the mass was freely mobile, the facial nerve was intact bilaterally, and there was no palpable lymphadenopathy

Results from fine-needle aspiration 6 months prior to presentation were consistent with a cystic oncocytic neoplasm, and computed tomography revealed a 1.9 × 1.5 × 2.1-cm, well-circumscribed ovoid lesion in the anterior aspect of the superficial lobe of the left parotid gland (Figure, A)

A left superficial parotidectomy was performed under general anesthesia with facial nerve dissection and facial nerve monitoring

Postoperative pathological analysis revealed a cystic, cribriform, papillary neoplastic growth with low-grade cytologic features and nearly complete myoepithelial rims (Figure, B and C)

Calponin and p63 staining were positive in the myoepithelium surrounding the tumor nests (Figure, D), S-100 was positive within the tumor, and the Ki-67 rate was approximately 5%

Extracellular mucin and occasional mucous cells were also noted

Margins were clear, and there was no evidence of perineural spread or lymphovascular invasion

The patient tolerated the procedure well, recovered appropriately, and was free of complications at his first follow-up appointment


A man in his 60s with a medical history of hypertension, cerebrovascular accident, colon cancer, and remote cocaine use presented with numerous pruritic crusted papules and nodules on the face and genitals

The eruption presented abruptly over a 2-week course, and the patient attributed it to contact with plants while working in his yard

He denied having similar lesions in the past

He had used over-the-counter topical antibiotic and antihistamine creams, which alleviated the pruritus

He denied recent sexual intercourse, penile discharge, fevers, chills, nausea, or vomiting

Physical examination of the bilateral cheeks, glabella, dorsal nose, and chin revealed multiple skin-colored papules, nodules, and vegetative plaques ranging in size from 4 to 6 mm in diameter, with central umbilication, erosions, and overlying yellow crust (Figure, A)

There were similar lesions on the scrotum and inner thighs, with sparing of the penis (Figure, B)

There was also a 1-cm ulceration on the left lower lip

One 4-mm punch biopsy specimen was obtained from a nodule on the left medial cheek, bisectioned, and sent for both hematoxylin–eosin staining and tissue cultures for bacteria, fungi, and atypical mycobacteria (Figure, C and D)

A, Multiple crusted and ulcerative papules and nodules on the face

B, Multiple ulcerated healing papules on the scrotum and umbilicated papules on the left inner thigh

C, Low-power image (hematoxylin-eosin, original magnification ×40)

D, A Warthin-Starry silver impregnation stain (original magnification ×40)


Granulomatous manifestation of inflammatory bowel disease

A woman in her 50s with a history of Crohn disease (CD) was referred for evaluation of painful perianal skin growths

Physical examination revealed a tender perianal plaque with a papular, cobblestone-like surface (Figure, A)

She was taking no medications and had not had active gastrointestinal symptoms of CD in several years

A biopsy specimen from the perianal plaque was obtained (Figure, B and C)

A, Multiple flesh-colored perianal papules and a pebbly plaque in a middle-aged woman with Crohn disease

B, A biopsy specimen of the perianal plaque was obtained, and histopathology revealed a dermal perivascular and nodular infiltrate composed of aggregates of histiocytes and (C) numerous multinucleate giant cells surrounded by mature lymphocytes

Acid-fast and silver stains were negative (not shown)


A man in his 70s presented with a 4.0-cm linear plaque with an 8.0-mm erythematous nodule on one end on his right upper abdomen (Figure, A)

The lesion appeared 2 months prior and had been mildly itchy

The patient lived in a farm community that used well water for drinking

He denied wading in local water sources and had not traveled abroad recently

The patient denied systemic symptoms, and further physical examination results were unremarkable

Recent laboratory blood work was notable for peripheral eosinophilia

A punch biopsy was performed (Figure, B)

A, Photograph shows a serpiginous erythematous plaque on the abdomen

B, Histopathological analysis of a punch biopsy specimen demonstrates eosinophilic cuticle (external tegument) and calcareous bodies (basophilic internal structures)

C, Histopathological analysis of a punch biopsy specimen


A 57-year-old woman presented to the emergency department with a 1-day history of upper abdominal pain

The pain was localized to the epigastrium and had acute onset  that prevented her from working for the rest of the day

There was no association between the pain and eating

No history of nausea, vomiting, diarrhea, melena, hematochezia, constipation, or fevers was reported

She had no alcohol abuse or smoking history

Dyslipidemia and a transient ischemic attack 3 years before were her only medical history

She had never had a colonoscopy or a gastroscopy

Her medication included atorvastatin and acetylsalicylic acid

The only past operation she reported was for treating carpal tunnel syndrome

She was a sheep breeder and was in close contact with animals for 8 to 12 hours every day

During physical examination, the heart and the lungs were normal

Her abdomen was soft with tenderness in the epigastrium

The laboratory evaluation results revealed normocytic anemia (hematocrit level of 28.1% [to convert to proportion of 1.0, multiply by 0.01] and hemoglobin level of  9.3 g/dL [to convert to grams per liter, multiply by 10]) including a normal white blood cell count of 7600/µL (to convert to ×109 per liter, multiply by 0.001)

Results from a complete metabolic panel were normal

An abdominal radiography was performed (Figure 1)

Anteroposterior abdominal x-ray film in erect position

In the right side of the mid to lower quadrant, at the level of L5 vertebral body, there is a rounded radio-opaque lesion (arrowhead) that seems to contain a second smaller sclerotic lesion within its central part (a lesion-in-lesion appearance)


A man in his mid-50s was referred to our hospital because he had a cystic mass in his spleen that was discovered incidentally during a routine physical examination

He had no history of abdominal trauma, infection, or surgery

A physical examination revealed mild tenderness over the posterior left upper quadrant

A precontrast computed tomographic scan revealed a large, well-defined, cystic mass (7 by 6 cm) with mural calcification in the spleen (Figure 1A), and the cyst was not enhanced in the arterial phase

The laboratory test results, including carcinoembryonic antigen and carbohydrate antigen 19-9 levels, were all within normal ranges, except for a mild increase in the carbohydrate antigen 72-4 level, which was 11.89 U/mL (normal range, 0-6.9 U/mL)

A, Computed tomography (CT) revealed a large, well-defined, cystic mass with mural calcification in the spleen

B, The surface of the cyst was extremely dense and appeared to have an eggshell-like calcification

the cyst was filled with about 300 mL of yellowish opalescent fluid containing numerous cholesterol-like crystals (arrowhead)

During a laparotomy, a huge hard mass, which was partially covered by fibrous and necrotic tissue, was found emerging from the lower pole of the spleen

A total splenectomy was performed, and a gross pathologic examination revealed that the lower end of the spleen was occupied by a cyst, the surface of which was extremely dense and appeared to have an eggshell-like calcification (Figure 1B)

The cyst was filled with about 300 mL of yellowish opalescent fluid containing numerous cholesterol-like crystals (Figure 1B)


An otherwise healthy 2-month-old boy was brought to the dermatologist by his parents for a severe but apparently asymptomatic rash characterized by redness and scaling

This condition had been present for 4 weeks and included areas of the scalp, face, chest, abdomen, back, axillae, arms, legs, perineum, and buttocks

There were no aggravating factors, and no family member in contact with the patient had a rash with similar characteristics

The parents tried moisturizers and topical antiyeast medications with no change in the rash

The physical examination findings were notable for a well-appearing infant with pink to brown nummular patches and plaques with thick white scale on the scalp, cheeks, trunk, back, axillae, inguinal crease, scrotum, and umbilicus and posterior to the ears (Figure 1)

The patient had no lymphadenopathy or hepatosplenomegaly

He was up to date on all immunizations and was full term

A, Infant at presentation, with pink to brown round scaly patches and plaques widespread on the trunk including umbilicus and axillae

B, Pink and brown scaly patches on the abdomen within the inguinal crease


A woman in her 50s was admitted after several months of progressive dyspnea on exertion and severe lower extremity edema

She had undergone aortic valve replacement for aortic stenosis, 2-vessel coronary artery bypass, and permanent pacemaker placement 1 year before presentation

She had a history of mantle chest radiotherapy for Hodgkin lymphoma at age 17 years, which was curative

After successful intravenous diuresis, neck examination at approximately 60° (Figure 1A and Video) revealed a regular carotid pulsation and discernible internal jugular venous pulse

After examination of the neck at rest, the patient took a small breath and held it for a few seconds

Hemodynamic data at left and right heart catheterization are shown in Figure 1B

A, Neck examination at 60° shows the location of the carotid and internal jugular pulsations

B, High-fidelity micromanometer simultaneous recording of the left ventricular (LV) and right atrial (RA) pressures


An elderly man with history of squamous cell carcinoma of the forehead presented with 6-month history of a slowly enlarging left facial mass

He did not have facial pain, weakness, numbness, trismus, or difficulty chewing

He had no history of dysphagia and/or odynophagia, hoarseness, vision changes, dry mouth, decreased ipsilateral sweating, weight loss, fevers, chills, or a history of tobacco or alcohol use

Physical examination revealed a palpable nontender mass in the left parapharyngeal space

Cervical lymphadenopathy was not palpable

Nasopharyngoscopy revealed slight bulging of the lateral pharyngeal wall into the oropharynx with no discrete masses seen

Computed tomography–guided fine-needle aspiration biopsies were nondiagnostic

Axial T2-weighted MRI showed a lesion arising from the left parapharyngeal space, arising from the deep lobe of the parotid gland

The lesion caused loss of the fat plane between the parapharyngeal space and masticator space with subsequent inflammation of the pterygoid space

The pharyngeal mucosa of the left lateral pharyngeal wall was effaced owing to mass effect (Figure, A)

The mass was surgically resected in its entirety

Hematoxylin-eosin staining showed sections of confluent amorphous eosinophilic masses with surrounding foreign-body giant cell reaction and lymphoplasmacytic infiltrates (Figure, B)

Sheets of plasma cells were positive for κ and CD138 and negative for λ, consistent with clonal κ light-chain restricted plasma cell infiltrates around amorphous tissue (Figure, C)

Congo red stain showed apple-green birefringence demonstrated on polarized microscopy (Figure, D)

A, Magnetic resonance imaging (MRI) of a lesion in the left parapharyngeal space


A man in his 70s with a history of acute myeloid leukemia (AML), diabetes, hypertension, and coronary artery disease presented with a 3-month history of hoarseness, throat pain, dysphagia, and odynophagia, which began after he was intubated for 13 days for neutropenic sepsis

Twenty-nine months prior to presentation, the patient had been diagnosed as having myelodysplastic syndrome, which 7 months later progressed to AML

During treatment for AML with low-dose cytarabine, his course was complicated by neutropenic sepsis requiring intubation, resulting in his head and neck symptoms

His physical examination was notable for a “breathy” voice with no respiratory distress or cervical lymphadenopathy

Flexible nasal endoscopy revealed an immobile right vocal fold with broad submucosal fullness on the right false vocal cord and arytenoid

Computed tomography (CT) demonstrated a right-sided laryngeal mass extending from the level of the free margin of the epiglottis to the level of the glottis (Figure, A)

In the operating room, during direct laryngoscopy a polypoid mass was seen based on the right arytenoid, also involving the right false vocal cord (Figure, B)

Frozen section confirmed polypoid ulcerated tissue with granulation consistent with an inflammatory process, and histopathologic analysis demonstrated a mixed infiltrate containing numerous mononuclear myeloid cells positive for myeloperoxidase, CD34, CD68, and CD33 but negative for CD117 (Figure, C and D)

There are small numbers of scattered CD3+ T-cells and CD20+ B-cells

C, Neoplastic cells admixed with lymphoplasmacytic inflammatory cells

D, Neoplastic cells strongly express myeloperoxidase


A woman in her 30s presented with a lump on her tongue that had been progressively enlarging for several years without a precipitating event or symptoms

The size now caused intermittent gagging and a foreign-body sensation in her throat

She denied pain and was an otherwise healthy nonsmoker

Examination of the oral cavity revealed an approximately 1-cm, pedunculated mass of the posterior dorsal tongue just lateral to the circumvallate papillae

The lesion was mobile and well-circumscribed without ulceration or surrounding mucosal abnormalities

It was nontender to palpation without drainage or discharge

Flexible fiber-optic laryngoscopy showed no other lesions

Neck ultrasonography showed a normal thyroid gland

Excisional biopsy was performed with the patient under local anesthesia, and the entire lesion was sent for pathologic examination

Grossly, the firm, polypoid mass measured 0.5 × 0.5 × 0.8 cm

Histopathologic examination revealed a discrete proliferation of mature bone with overlying mildly hyperplastic stratified squamous epithelium without surrounding inflammation (Figure, A)

The core was composed of dense lamellar osseous matrix with numerous Haversian canals and associated regularly dispersed osteocytes in lacunae (Figure, B)


A woman in her 30s presented with chronic bilateral nasal congestion in the absence of facial pressure, pain, paresthesia, bleeding, dysphagia, or voice change

Physical examination demonstrated no lymphadenopathy

The oropharynx and oral cavity were clear

Anterior rhinoscopy demonstrated thick, clear to yellow mucus bilaterally in the nasal cavities and bilateral inferior turbinate hypertrophy

There was right to left nasal septal deviation

Rigid nasal endoscopy demonstrated moderate mucosal edema responsive to topicalization

Thick, clear to yellow mucus was evacuated with suction from the middle meatus and sphenoethmoidal recess, revealing a large, lobulated, flesh-colored mass filling the entire nasopharynx with an irregular surface and firm consistency

The fossae of Rosenmuller were effaced bilaterally

There was no overt surface ulceration or bleeding

Computed tomography (CT) revealed a well-defined, round, 2-cm midline posterior nasopharyngeal mass, situated between the longus colli muscles (Figure, A)

The lesion appeared hypodense on CT, without calcification, enhancement, or bony erosion

Magnetic resonance imaging (MRI) (Figure, B-D) demonstrated the lesion to be hypointense on T1 sequence and hyperintense on T2 sequence with mild heterogeneous enhancement

Endoscopic biopsy of this mass was performed


Epidermolysis bullosa with mottled pigmentation

Focal dermal hypoplasia (Goltz syndrome)

A young girl presented to establish care for skin lesions on her trunk and extremities that had been present since birth

Her mother described an intermittent inflammatory reaction localized only to the patient’s abnormal skin lesions that was reproducible in febrile states and was concerned about asymptomatic, stable, dark brown lesions that had developed in areas of prior blistering and scarring on the patient’s right arm, left knee, and right upper back

There was no history of developmental delay, eye abnormalities, or seizures

Examination revealed irregular, dark brown, well-demarcated macules with regular pigment networks on dermoscopy within prominent soft, yellow, papular protrusions in a Blaschkolinear arrangement on her upper arms, particularly her right arm

The right flank demonstrated linear, atrophic pink plaques with focal erosions and crusting, evidencing recent inflammation described by the patient’s mother (Figure, A)

Additional irregular, well-demarcated, evenly pigmented dark brown macules were present within pink, atrophic scars located on the right side of the trunk, the right upper back, and the left knee

She had well-healed surgical scars on the hands, syndactyly of the toes, and ventral pterygium and dystrophy of the fingernails and toenails (Figure, B)

A, Irregular, dark brown, well-demarcated macules within prominent soft, yellow, papular protrusions in a Blaschkolinear arrangement on the upper arms

linear, atrophic pink plaques with focal erosions and crusting are seen on the right flank

B, Extracutaneous features show syndactyly of the toes and ventral pterygium and dystrophy of the toe nails

C, Hematoxylin-eosin staining of skin biopsy demonstrates close proximity of subcutaneous fat to the epidermis with attenuated collagen (original magnification × 20)

Previous treatment had included surgical excision of a frequently symptomatic focus of affected skin with subsequent adjacent tissue transfer

Additionally, she had received dental implantation of frontal upper incisors

Histopathologic slides from the prior excision of affected skin were reviewed (Figure, C)


Linear and whorled nevoid hypermelanosis

A 14-day-old girl presented with vesicular lesions over her trunk and lower extremities

According to her mother, lesions had started 2 days prior

Physical examination revealed vesicles filled with clear fluid disposed in a linear arrangement forming whorls and swirls (Figure, A)

A, Linear vesicles on the calf of the newborn patient

The lesions appeared on the 13th day of life

B, After 15 months, the lesions had evolved into small hyperpigmented macules in a linear arrangement

C and D, A biopsy specimen of a vesicle was obtained

Herpes simplex and varicella zoster virus polymerase chain reactions were negative

Syphilis, cytomegalovirus, and toxoplasma serologies were negative

Bacterial and fungal cultures of the vesicles were negative

A biopsy sample of a vesicle was obtained

Over time, lesions healed leaving residual grayish hyperpigmentation (Figure, B)


A 55-year-old man presented with eruptive and painless reddish-yellow papules in the axillae (Figure, A and B)

He endorsed polyuria and polydipsia but was otherwise asymptomatic

The patient’s medical history included hypertension, and review of his family medical history was noncontributory

Recent extensive laboratory data revealed mildly elevated triglycerides (209 mg/dL [to convert to mmol/L, multiply by 0.0113]) but otherwise were all within normal limits

On physical examination, numerous coalescing reddish-yellow pedunculated papules were present in both axillae (Figure, A and B)

Shave biopsy of one of the left axillary papules was performed (Figure, C and D) with additional immunohistochemistry positive for CD68 antibody staining

A and B, Numerous coalescing reddish-yellow pedunculated papules in the right and left axillae

C, Histopathological analysis of a biopsy specimen taken from the left axilla shows histiocytes with foam cells, scattered lymphocytes, and few plasma cells

D, a classic wreath like Touton cell and foam cells with histiocytes


The first episode of cluster headache episodes

Tolosa-Hunt syndrome, a retrobulbar inflammation

A space-occupying mucocele of the nasopharynx

An acute episode of angle-closure glaucoma

A 24-year-old woman was referred to our clinic for evaluation and treatment of a first-ever episode of headaches that occurred 16 days before the consultation

She reported a strong unilateral headache located in her left cheek, eye, and forehead that evolved during the evening hours, peaked at night, and decreased again until the morning, without any remarkable trigger

This first episode went along with ipsilateral lacrimation, a red eye, and periorbital swelling but without ptosis, which she documented with her smartphone (Figure 1)

During a painful night, she could not sleep

Pain remitted, but the swelling increased until the next morning and slowly decreased over approximately 36 hours

Recurrent but less intense episodes of several hours’ duration occurred during the 2 subsequent nights with no symptoms during the following 14 days

The patient reported no nasal congestion, nausea, vomiting, or phonophobia or photophobia

The patient reported being an infrequent smoker, with about 2 cigarettes a month

The patient during the first pain episode, with a red left eye, lacrimation, and periorbital swelling

She had no history of impaired vision, diplopia, facial trauma, prior head or neck surgery, or allergies

Examination of her head and neck showed no particularities, periorbital edema or proptosis, or enlarged lymph nodes

The neurological examination was unremarkable, with normal visual acuity, symmetrically reactive pupils, and unimpaired ocular movements, as was a differential hemogram, which showed normal white blood cell count of 5500/μL (to convert to ×109/L, multiply by 0.001)


A woman in her 40s with diabetes consulted our emergency department owing to a pain in the right groin that had worsened since its onset 3 days before

The pain radiated throughout the right leg and was related to nausea without vomiting

The patient’s history revealed chronic constipation, relieved by the use of laxatives

The patient never noticed any inguinal protrusion or mass

Furthermore, results of an abdominal physical examination were also normal, and no neurological or vascular deficits were found

The abdominal computed tomographic scans performed in the emergency department are shown in the Figure


Fibrosis owing to chronic peptic ulceration

A 72-year-old woman presented with a 6-month history of a dull, epigastric pain, 10-kg weight loss (from 52 kg to 42 kg), and a 6- to 8-week history of nausea and vomiting

Physical examination showed no notable abnormalities

Blood test results revealed anemia (hemoglobin 9.8 g/dL [to convert to grams per liter, multiply by 10]) but were otherwise unremarkable

Upper gastrointestinal series showed subtotal occlusion in the second part of the duodenum (Figure 1 A)

Computed tomography of the abdomen confirmed intraluminal narrowing of the duodenum without signs of a mass (Figure 1 B, arrowhead)

Gastroduodenoscopy revealed a duodenal ulcer with pinpoint stenosis that could not be passed or dilated

Multiple biopsies were negative for cancer

Total parenteral nutrition was started and the patient was scheduled for surgery

A, Coronal upper gastrointestinal series demonstrate subtotal occlusion in the second part of the duodenum

B, Axial computed tomography image shows intraluminal narrowing of the duodenum without signs of a mass (arrowhead)


A healthy girl aged 4 years presented with a 5-day history of a pruritic rash on her arms, neck, axillae, and face

The rash started as small pink bumps on her arms that spread to involve the axillae, neck, and upper chest

Four days prior to arrival, she developed significant redness and pruritus of her face

On arrival, her parents noted black streaks on her right arm, prompting an urgent emergency department evaluation

Treatment prior to presentation included diphenhydramine, cetirizine, and a 20-mg dose of prednisone without improvement

The patient was otherwise healthy with normal development and growth

She had a history of mild atopic dermatitis and was not taking any medications

She had no known history of drug allergy or contact allergy

The patient lived with her 2 siblings and both parents, who were all asymptomatic

She had no pets and no recent travel, and her vaccinations were up to date

On physical examination, the patient was actively scratching the involved skin

The bilateral arms, axillae, anterior neck, and face had poorly defined, blanching pink-red patches and edematous plaques without epidermal change (Figure, A)

Jet black, well-defined, and slightly shiny linear streaks with peripheral erythema were noted on her right forearm, with smaller circular, black macules on the ipsilateral biceps and axilla (Figure, B)

A, Poorly defined, blanching pink-red patches and edematous plaques on the upper chest, neck, proximal arms, and face

B, Jet black, well-defined, and slightly shiny linear streaks with peripheral erythema on the right forearm


A man in his 20s had a lump on the right side of his forehead

His medical history was notable for bilateral retinoblastoma when he was an infant, which was treated with right-eye enucleation and left-eye irradiation

As a teenager, he developed an advanced malignant small blue cell tumor, which was interpreted as an olfactory neuroblastoma and treated with radiation and chemotherapy

On examination, the patient had a hard, fixed, rounded 2-cm lesion in the right supraorbital region

Computed tomography (CT) showed an irregular mass in the right frontal, maxillary, and ethmoid sinuses with surrounding sclerotic and erosive bony changes

A fine-needle aspirate (FNA) revealed atypical, mitotically active cells

Bone shavings obtained operatively were interpreted as chronic sinusitis with sclerotic bone

The surgical specimen showed multinucleated giant cells and bizarre stromal cells suggestive of treatment effect (Figure, A) and normal-appearing bone

A second opinion was reported as chronic sinusitis and atypical stromal cells

When the patient returned 2 months later with increasing pain, endoscopy revealed a polypoid lesion occupying the biopsied site

On repeated operative biopsy, histologic examination showed similar giant cells and atypical stromal cells (Figure, B)

However, other areas demonstrated osteoid formation with fine calcifications and severely atypical spindle cells with mitotic figures (Figure, C), as well as osteoid-entrapped, malignant-appearing cells (Figure, D)


A man in his mid-60s was referred to the otolaryngology surgery department for evaluation of an incidental deep nasal septal mass discovered on a noncontrast head computed tomographic (CT) study ordered for stroke-like symptoms after a colonoscopy

His only symptom was frontal and ethmoid sinus pressure

He denied difficulty breathing, postnasal drip, rhinorrhea, epistaxis, or anosmia and had no history of sinus or upper respiratory infections

His extensive medical history was notable for congestive heart failure, Ménière’s disease after multiple endolymphatic shunts, a benign parotid tumor (which had been excised), and chronic abdominal pain secondary to pancreatic steatorrhea

Flexible nasal endoscopy confirmed a central posterior bulge in the posterior nasal cavity with normal overlying mucosa and clear nasopharynx

The noncontrast CT bone window showed an expansile anterior nasal septum mass measuring 1.9 × 2.4 cm with internal matrix mineralization (Figure, A-C)

Biopsy and magnetic resonance imaging (MRI) were recommended as baseline studies for follow-up, but were not completed

A complete septectomy was performed, which involved near-complete resection of the quadrangular cartilage and resection of the adjacent anterior portion of the periosteum of the vomer bone

A histological specimen is shown in the Figure, D

A-C, Computed tomographic images without contrast of the head using a bone algorithm

D, Histopathological image from the nasal cavity mass


A woman in her 20s with a history of a benign retropharyngeal mass was followed with serial examinations and imaging after a debulking procedure performed 1 year prior

She reported return of her symptoms after 11 months to include mouth breathing, snoring, and nasal obstruction with hyponasality and eustachian tube dysfunction

She also began to experience transient upper extremity paraplegias

She required hospital admission with steroid and anti-inflammatory treatment for partial resolution of symptoms

On examination, she was noted to have abnormal asymmetry of the posterior nasopharyngeal wall by flexible fiber-optic laryngoscopy without any overlying mucosal changes

Magnetic resonance imaging revealed a complete recurrence and enlargement of the retropharyngeal mass with dimensions of 4.2 (anterior to posterior) × 6.3 (transverse) × 5.1 (craniocaudal) cm

The mass was heterogeneously hyperenhancing on T1-weighting and centered at the atlantoaxial joint causing erosion of the upper dens, anterior arch of C1, and the basion (Figure)

A, T1-weighted magnetic resonance imaging (MRI)

C, Fat-saturated T1-weighted postcontrast MRI

D, Computed tomographic image (CT), bone windows

Arrowhead indicates the anteromedial aspect of the left foramen transversarium of C1.


A woman in her 60s was evaluated in the neurosurgery intensive care unit for purulent discharge from her right ear

She had undergone an emergency craniotomy for brain decompression the day before

She was receiving intravenous antibiotics and steroids and was sedated and mechanically ventilated

Computed tomography (CT) of the temporal bone detected middle ear, mastoid, and infralabyrinthine involvement (Figure, A)

Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain demonstrated meningitis and a large cerebellar lesion with rim enhancement

Moreover, diffusion-weighted imaging (DWI) images and apparent diffusion coefficient (ADC) maps were consistent with cerebellar abscess (Figure, B-D)

ADC indicates apparent diffusion coefficient

FLAIR, fluid attenuated inversion recovery


A young, previously healthy girl presented with a 10-day history of progressive, left-sided facial palsy accompanied by ipsilateral hearing loss and decreased facial sensation

She had not experienced headaches, disequilibrium, or tinnitus

She had no history of trauma, and there was no response to steroids

Findings from a physical examination were notable for left House-Brackmann grade III/VI facial palsy, hypoesthesia in left trigeminal nerve distribution, and no left audiologic responses to bone or air stimuli

A non–contrast-enhanced computed tomographic (CT) scan showed a left petrous apex expansile lesion involving the internal auditory canal (Figure, A)

Magnetic resonance imaging (MRI) showed a well-circumscribed lesion (Figure, B) extending into the fundus of the internal auditory canal (Figure, C)

There was a fluid-fluid level in the lesion (Figure, B), consistent with presence of blood products

The displaced adjacent cranial nerve V (Figure, C) extended along the surface of the lesion

After the administration of an intravenous contrast agent, there was a nodular focus of enhancement at the anterior aspect of the lesion (Figure, D) and linear areas of enhancement at the periphery and of internal septations (Figure, D)

The lesion was completely excised via a middle fossa craniotomy

Postoperatively, the patient regained full function of all cranial nerve deficits, including the hearing loss


A white woman in her 60s with a medical history of multiple joint replacements related to osteoarthritis presented with left leg pain

The symptoms started approximately 3 months prior and had progressively worsened with interval difficulty weight bearing and ambulating up and down stairs

The patient noted night sweats 2 weeks prior to presentation but denied any associated fevers, unintentional weight loss, fatigue, or change in appetite

Physical examination revealed limited range of motion of the left leg related to pain

There was no cervical, axillary, or inguinal lymphadenopathy

Laboratory evaluation demonstrated a hemoglobin level of 10.3 g/dL and a lactic acid dehydrogenase level of 599 u/L

Serum protein electrophoresis testing did not detect a monoclonal protein

A plain radiograph of the left femur showed a 15-cm area of moderate destruction in the proximal femoral shaft extending distally from the level of the lesser trochanter with concern for impending fracture (Figure 1A and B)

A computed tomography (CT) scan of the chest, abdomen, and pelvis did not visualize any metastatic disease, and results were negative for lymph node enlargement

A nuclear medicine bone scan demonstrated increased uptake in the left femur with no other focal abnormalities

The patient underwent a CT-guided needle biopsy of the left femur (Figure 1C)


A Japanese man in his 80s presented with a 3-year history of erythematous papules on the bilateral cheeks without any additional symptoms

The lesions had repeatedly recurred despite topical steroid therapy

Physical examination revealed papules and plaques ranging up to 1 cm in diameter on the lateral cheeks and behind the ears and neck along with a red-brown hue (Figure, A)

Complete blood cell count and comprehensive metabolic panel results were unremarkable

However, serum protein electrophoresis revealed polyclonal hypergammaglobulinemia

Antinuclear antibody and anti-Ro/SS-A and anti-La/SS-B antibodies were undetectable by indirect immunofluorescence or double immunodiffusion methods

Computed tomography (CT) revealed bilateral parotid gland swelling together with mild lymphadenopathy in the mediastinum and hilus

A skin biopsy specimen was obtained from a papule on the neck for further analysis (Figure, B-D)

A, Indurated papules and plaques are present on the lateral cheeks and behind the ears and neck along with a red-brown hue

B and C, Hematoxylin-eosin–stained skin biopsy specimens taken from papules show nodular inflammatory cell infiltration consisting of dense lymphocytes admixed with plasma cells and eosinophils

D, Papule specimen under IgG4 immunohistochemical stain


A woman in her 60s with acute myeloid leukemia (AML), status post–nonallogenic bone marrow transplant and subsequent relapse, was admitted to the hospital with blast crisis (white blood cell count, 72 500/μL

Flow cytometry showed myeloid blasts, most of which (≥90%) expressed CD13, CD33, CD117, and partial CD11b (≥50%)

Blasts were also “highly positive” for myeloperoxidase

Stains were weak for CD4 and were negative for CD34 and HLA-DR

Cytogenetic studies revealed a normal karyotype with NPM1 and FLT3-ITD mutations

Dermatology was consulted for evaluation of 5-day history of new tender lesions on the scalp

The patient did not have a history of recent travel or trauma

Physical examination revealed several tender purpuric papules and plaques with central necrosis and hemorrhagic crusting on the scalp (Figure, A)

Similar lesions were present on the right dorsal hand and left lower leg

On the right forearm, there was a tender erythematous plaque with overlying vesicles and mild hemorrhagic crusting

On the left medial thigh, there was a large indurated, tender red-brown plaque

Wound cultures and polymerase chain reaction studies from the right forearm were negative for herpes simplex virus and varicella zoster virus infections

Punch biopsy specimens from lesions on the scalp, right forearm, and left thigh were sent for histopathological and immunohistochemical assessment and tissue culture (Figure, B-D)

A, Clinical image of tender purpuric papules and plaques with central necrosis on the scalp

B-D, Histologic images of a punch biopsy specimen from a scalp lesion