Mystery Quiz

Mystery Quiz

December 21, 2012

Elizabeth Mulaikal MD, Vivian Hayashi MD, Robert Smith MD

The patient is a 55 year old African American male with a 60 pack year history of tobacco use and AIDS,who presented with 1 month of intermittent fevers and weight loss. His most recent CD4 count and viral load were 2/cmm and 50,623 copies/mL, respectively. Prior opportunistic infections included pneumocystis pneumonia and thrush. He was previously homeless, but currently resides in a Single Room Occupancy Housing. Upon presentation he complained of occasional night sweats, but no shortness of breath, cough, sputum production, or hemoptysis. Vital signs were notable for a fever of101.5F, but the patient was otherwise normotensive with a room air saturation of 98%. On physical examination he appeared cachectic and the lung fields were clear. Labs showed a WBC count of 3.6k/cmm with 47% neutrophils and 13% bands, and an LDH of 364 U/L.


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Mystery Quiz- The Answer

June 29, 2012

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The answer to the mystery quiz is thymoma associated with myasthenia gravis. The clue to the case is the intermittent dysphagia and chewing difficulty. If one considers myasthenia in the differential, then an otherwise grossly normal appearing chest radiograph may be viewed more closely, with attention directed to the upper, anterior mediastinum. The chest radiograph shows the ascending aorta (image 3, arrow); on the lateral film, the retrosternal space, which normally contains air, appears opacified due to a soft tissue density (image 4, asterisk). The anterior ascending aorta on the lateral chest radiograph is not clearly seen because its border is obliterated by the mass which is in contact with it (image 5, arrow). The aorta also appears enlarged (image 5, arrowhead) which accounts for its prominence as seen on the PA chest radiograph. The mass appears encapsulated but the rough border on its left lateral aspect signifies possible invasion (image 6, arrow). Calcification, reported in 10-41% of patients, is also seen within the mass. Although more common in invasive disease, calcification can also be found in a significant minority of patients with benign disease.

Thymoma is the most common anterior mediastinal neoplasm in adult patients. About half of patients present with an incidental finding on chest imaging while the remaining half present with symptoms, most often muscle weakness associated with myasthenia gravis. This condition characteristically presents with waxing and waning weakness over long periods of time. Less commonly, patients present with other paraneoplastic syndromes such as pure red cell aplasia.

Our patient had an elevated level of acetylcholine receptor antibodies. (1.58, where a level >0.40 is considered positive). The resected specimen showed Type B Thymoma, B1 (lymphocyte-rich, organoid histology, as seen in low and high magnification, image 7 and 8 respectively). In addition, pathology revealed Stage la given the presence of microscopic transcapsular invasion (image 6, arrow). Based on the Masaoka staging classification, the pathology findings confer a predicted five year survival of 86-95%. Due to the presence of capsular invasion, adjuvant radiotherapy was also undertaken for our patient. Overall, there is a continuum of pathology from thymoma to the less common thymic carcinoma, with heterogeneous histology seen. Pathology, however, does correlate with biologic behavior and invasiveness.

Mystery Quiz

June 19, 2012

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The patient is a 61 year old man admitted to the psychiatry service for depression. During the hospitalization the patient reported a three year history of dysphagia and occasional right sided chest pain not clearly associated with exertion. He first noted difficulty chewing and swallowing turkey on Thanksgiving Day. The symptoms were intermittent thereafter and involved solid foods but not liquids. His chewing and swallowing difficulty abated for a year but returned one year later and persisted until presentation. The patient denied fevers, breathlessness, chest pain, limb weakness, speech abnormalities, numbness or visual changes. Past medical history included depression treated with citalopram, hyperlipidemia treated with simvastatin, and tobacco abuse. Exam was notable for obesity, normal vital signs, clear chest, and non-focal neurologic exam.

Chest radiographs are shown below:


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Mystery Quiz- The Answer

December 14, 2011
Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The answer to the mystery quiz is Kaposi’s sarcoma. The CXR shows bilateral confluent airspaceopacities which have a wide differential diagnosis in this case. The CT narrows the differential.  Specifically, the opacities appear to emanate from the central hilar areas, cuff the airways, and fan out into the more distal airspaces (Images 3 and 4).  This appearance is very suggestive of Kaposi’s sarcoma. CT scans may also reveal mediastinal lymphadenopathy and large pleural effusions which are not present in our case.  The absence of fever and chronicity of one month argue against bacterial pathogens such as pneumococcus and legionella.  Of note, there is an absence of cavitation as seen in necrotizing infections, scant ground glass opacity as found in pneumocystis pneumonia, and an absence of predominant nodularity as might be seen in pulmonary lymphoma.  Kaposi’s sarcoma was the most common neoplastic disease of patients with HIV infection and low CD4 counts early in the AIDS epidemic. It results from an additional infection with HHV-8. Inflammatory pathways involve vascular tissue; pathology typically shows spindle cells sometimes accompanied by blood in the vascular spaces.  Also, the lesions are commonly seen in the large airways as red-to-purple plaques, sometimes spreading throughout all the visualized airways.  There is still debate about whether Kaposi’s sarcoma is truly a neoplasm given the cellular multiclonality that is often reported.  Cutaneous Kaposi’s is by far the most common presentation, and pulmonary involvement is seen in about 30% of cases.  The GI tract is also commonly involved in as many as 40% of cases.  Kaposi’s was seen much more frequently in the 1980s but inexplicably declined in incidence even before the HAART era which further hastened the decline.

Our patient underwent bronchoscopy which revealed red plaques throughout most of the airways .  The transbronchial biopsy showed spindle cells that stained positive for CD 34, a marker of early hematopoietic and vascular-associated tissue.  No pathogens were found on BAL or biopsy. These findings confirmed the diagnosis of Kaposi’s sarcoma.  The patient was treated with ten cycles of liposomal doxorubicin along with HAART.  His recovery was quite dramatic, as can be seen on a CT scan six years after his presentation (Image 5).  The patient also gained weight and increased his CD4 count to nearly 300 cells/cmm.

Mystery Quiz

December 9, 2011

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The patient is a 52 year old African-American man who presented with breathlessness and weight loss over one month along with watery stools during the week prior to admission. He had a history of untreated HIV infection, a CD4 cell count of 5/cmm, oral candidiasis, and peri-rectal HSV infection. The patient reported occasional cough with variable sputum production but denied fever, night sweats, hemoptysis, recent travel, or sick contacts. His risk factor for HIV infection was having sex with other men. The physical was notable for cachexia, normal temperature, blood pressure of 87 mmHg systolic, respiratory rate of 25 bpm, O2 saturation of 89% breathing room air, coarse breath sounds somewhat decreased at the left base, and unrevealing dermatologic exam. Labs showed a WBC of 2k/cmm, 23% bands, albumin 1.8 gm/dL and an LDH of 422 U/L.


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Mystery Quiz

November 30, 2011

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The patient is a 52 year old African-American man who presented with breathlessness and weight loss over one month along with watery stools during the week prior to admission. He had a history of untreated HIV infection, a CD4 cell count of 5/cmm, oral candidiasis, and peri-rectal HSV infection. The patient reported occasional cough with variable sputum production but denied fever, night sweats, hemoptysis, recent travel, or sick contacts. His risk factor for HIV infection was having sex with other men. The physical was notable for cachexia, normal temperature, blood pressure of 87 mmHg systolic, respiratory rate of 25 bpm, O2 saturation of 89% breathing room air, coarse breath sounds somewhat decreased at the left base, and unrevealing dermatologic exam. Labs showed a WBC of 2k/cmm, 23% bands, albumin 1.8 gm/dL and an LDH of 422 U/L.

CXR and CT are shown below:


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Mystery Quiz- The Answer

June 17, 2011

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The answer to the mystery quiz is lymphangitic carcinomatosis (LC). The diffuse interstitial and infiltrative pattern seen on CXR is best appreciated on the CT of the chest. On Images 4, 5 and 6, interlobular septal thickening is apparent (arrow) and is due to tumor infiltration of interstitial lymphatics. Also evident on these images (arrowhead) is diffuse peribronchial thickening due to tumor infiltration of the airways. Image 6 (open arrowhead) additionally shows pleural nodules, likely representing tumor infiltration of pleural lymphatics, with associated peripheral interstitial thickening leading to them (Kerley B line). The images reflect two paths for pulmonary lymphatic drainage: the interstitial lymphatics which drain centrally to hilar and mediastinal nodes and the more peripheral interstitium which also drains centrally but via lymphatics in the visceral pleura. Image 7 (arrow) shows significant mediastinal lymphadenopathy.

LC is an unusual manifestation of pulmonary metastases. Patients often present with advanced disease and marked tachypnea that may be disproportionate to the abnormality seen on imaging. The most common associated primary tumors are adenocarcinomas arising from breast, stomach, pancreas, prostate and bronchus. Pathogenesis is thought to be due to: (1) hematogenous spread with secondary involvement of pulmonary lymphatics; (2) possible retrograde spread from mediastinal nodes; or (3) antegrade spread to mediastinal nodes from blocked lymphatics.

Our patient remained quite breathless throughout the hospital course, requiring morphine for relief of dyspnea and supplemental oxygen. Pathology revealed tumor cells that had a signet ring morphology filling lymphatics (Image 8). Immunostaining showed expression of cytokeratin 7, CEA, and mucin (Image 9), but not cytokeratin 20, TTF-1, and CD 45. These results pointed to the upper GI tract including the stomach, pancreas, or biliary tree as the primary site of the tumor. Serum alpha-feto protein and prostate specific antigen were not elevated but carcinoembyonic antigen level was quite elevated at 83 ng/ml (ref. range 0-4.5). Upper endoscopy and MRCP were unrevealing. Due to severe respiratory compromise, the patient was unable to undergo colonoscopy, precluding further work-up for a possible GI tumor as well as treatment with chemotherapy. About three weeks after admission, the patient was discharged to a home hospice program.

Mystery Quiz

June 16, 2011

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The patient is a 62 year old man presenting with increasing breathlessness for the past six weeks. The patient first noted symptoms of mild shortness of breath and cough productive of yellow sputum that he attributed to his yearly winter cold. The sputum cleared and at presentation the patient had largely unproductive cough. A construction worker until six months earlier, the patient’s unlimited exercise tolerance was now limited to minimal exertion. He denied current fever, hemoptysis, night sweats, arthralgias, rash, and exposure to people with TB, but did note recent bilateral ankle swelling. The patient denied recent travel. Upon further questioning, the patient said that he maintained up to twenty finches in his apartment and had kept them until two weeks prior to admission. The patient had a 45 pack year smoking history but stopped 2 weeks earlier due to worsening shortness of breath. On exam, the patient was tachypneic at rest with pulse oximetry showing 90% saturation while breathing ambient air, a heart rate of 110 bpm and a chest exam that was unremarkable.

CXR and Chest CT are shown below:

 


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Mystery Quiz- The Answer

February 11, 2011

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The answer to the mystery quiz is pulmonary artery aneurysm. The chest CT shows a very round lesion in the left lower lobe with possible surrounding ground glass opacity. This perfectly round shadow suggests a vascular origin (Image 4, arrow). The apparent vascularity is further suggested by the sequential axial cuts on the CT (Image 5) which show a tubular dilatation (Image 5b) that bifurcates distally (Image 5d & e). This impression was confirmed by subsequent CT with contrast that shows marked enhancement of the lesion (Image 6). Additional coronal views (Image 7, arrow) show how the lobar pulmonary artery gives rise to the aneurysm. Importantly, the distal extension of the vessel shows a continuation of the pulmonary artery (Image 7, arrowhead) denoting a pulmonary artery aneurysm. In contrast, an arterio-venous malformation would have a feeding vessel from the pulmonary artery and an outgoing vessel flowing into the pulmonary venous system. In hindsight, another look at the lateral film reveals the tubular nature of the aneurysm (Image 8, arrows).

A pulmonary artery pseudo-aneurysm would be seen in an area of prior lung destruction due to an inflammatory process with subsequent scarring and fibrosis such as seen in pulmonary tuberculosis. Inflammation and scarring of lung tissue encroaches upon the pulmonary artery and thereby disrupts the three layered anatomy of the pulmonary artery. This process accounts for the formation of a pseudo-aneurysm. An additional aspect of this process includes new vessel formation arising from the bronchial artery circulation creating anastamoses with the pulmonary artery circulation. In our case, no such anastamoses are seen and the surrounding lung architecture is preserved, making it more likely that the lesion is a true pulmonary artery aneurysm.

Solitary pulmonary artery aneurysms are usually associated with congenital heart disease and pulmonary artery hypertension due to patent ductus arteriosis or any left-to-right intra-cardiac shunt. Most often, they are found in the proximal pulmonary artery, unlike the more peripheral location seen in our case. Other causes include acquired mycotic aneurysms due to bacterial endocarditis or septic thrombophlebitis, collagen vascular diseases, and Behcet’s syndrome. None of the foregoing associations was found in our case. Thus, the origin of the vascular abnormality is unclear.

Planned management included bronchoscopy to identify the origin of the hemoptysis but the patient declined further work-up. It was not clear whether the vascular abnormality was the source of bleeding but the ground glass opacification in the area of the lesion, possibly representing hemorrhage, increased the likelihood that the aneurysm was indeed the source. There are no explicit guidelines for treatment, but lung resection or embolization by IR should be considered. A follow-up chest CT three years later showed the lesion to be stable and the patient reported no further episodes of hemoptysis.

Mystery Quiz

February 4, 2011

Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

The patient is an 84 year old man who presents with small amounts of hemoptysis for one week prior to admission. He is a life-long non-smoker who began having cough with sputum production for one year without fever, chills, nightsweats, orthopnea, weight loss, arthralgias or rash. The patient denied unusual occupational exposures, history of prior TB, travel, or owning pets. The past medical history was significant for coronary bypass surgery, hypertension, atrial fibrillation treated with aspirin, and diabetes mellitus. A recent echocardiogram was unremarkable.

Chest film and chest CT are shown below:


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Mystery Quiz- The Answer

November 13, 2010

Dana Clutter, MD

Edited by Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

Faculty peer reviewed

The answer to the mystery quiz is bacillary angiomatosis (BA). BA is a disease that most frequently affects individuals infected with the human immunodeficiency virus (HIV) and typically presents with multiple cutaneous papules and nodules. Visceral manifestations also occur and can involve the bone, lungs, lymph nodes, spleen, liver (termed peliosis hepatis) and the central nervous system.(1) Since the relatively recent first description of the disease in 1983, the two causative organisms, Bartonella quintana and Bartonella henselae, have been identified. The main risk factors for B. henselae infection are contact with flea-infested cats and cat scratches, while those for B. quintana are lice infestation and homelessness. Erythromycin or doxycycline are the first line treatments.(2)

There are multiple clinical forms of cutaneous lesions in bacillary angiomatosis including dermal and subcutaneous nodules. The more common dermal form, present in 60% of patients, is characterized by multiple erythematous nodules/plaques that may be quite friable. An epithelial collarette may develop around some of these lesions. The subcutaneous form, present in approximately 50% of patients, features nodules which are often tender beneath skin that may be normal in color, dusky, or inflamed.(1)

Because it is frequently difficult to distinguish BA from other diseases, particularly Kaposi’s sarcoma (KS) and pyogenic granuloma, by clinical means alone, a biopsy is usually performed to establish the diagnosis. Histologically, biopsies of cutaneous lesions demonstrate lobular proliferation of small blood vessels lined by large endothelial cells. An infiltrate of scattered neutrophils is another important clue to the diagnosis. There may be an epithelial collarette and varying amounts of edema. A silver stain can help to visualize clumps of Bartonella bacteria.(1) A serologic test for Bartonella is available; however, up to 25% of culture-positive patients with advanced acquired immunodeficiency syndrome (AIDS) never develop anti-Bartonella antibodies.(1)

The differential diagnosis of BA includes other vascular lesions, such as pyogenic granulomas and angiomas, and given that the patient has advanced HIV/AIDS, it is particularly important to include KS and atypical mycobacterial infections.

In this case, there are several clinical clues to the diagnosis of BA. The facial lesion demonstrates a typical erythematous nodule surrounded by a collarette which is present in both BA and pyogenic granuloma, but not KS. The lesion is friable, which is characteristic of BA, where a firmer texture is expected in KS.(3) The lesion on the forearm represents a subcutaneous lesion, a finding common in BA and KS and rarely in pyogenic granuloma. Although these three diseases can affect immunocompetent patients, BA and KS have a strong predilection for patients with advanced AIDS. Thus, our patient with a CD4+ T-cell count of 1 cell/cmm has an increased risk of both diseases. Although insufficient sample remained for silver staining, pathology was most consistent with BA. The dramatic response to erythromycin pictured below in both the facial and forearm lesions also strongly supports the diagnosis of BA (Images 1 and 2).

Dr. Clutter is a first year resident at NYU Langone Medical Center.

Faculty Peer Reviewed by Jennifer Stein, MD, Assistant Professor in the Department of Dermatology, NYU Langone Medical Center.

1. Cotell S, Noskin G. Bacillary angiomatosis: clinical and histologic features, diagnosis and treatment. Arch Intern Med. 1994 Mar; 154: 524-528.

2. Centers for Disease Control and Prevention. Guidelines for Prevention and Treatment of Opportunistic Infections in HIV-Infected Adults and Adolescents. MMWR 2009; 58(No. RR-4):1-203.

3. Wong R. Bacillary angiomatosis and other Bartonella species infections. Semin Cutan Med Surg. 1997 Sep; 16(3): 188-199.

Mystery Quiz

November 10, 2010

Dana Clutter, MD

Edited by Vivian Hayashi MD and Robert Smith MD, Mystery Quiz Section Editors

A 25 year old woman infected with HIV presents to an HIV/AIDS clinic in Kampala, Uganda, for evaluation of cutaneous lesions on her face, arms and back. Aside from the disfiguring nature of her lesions, she reports being in her usual state of health. She denies any constitutional symptoms and all of her vital signs are stable. Her current CD4+ T cell count is 1 cell/cmm and she has never been on antiretroviral therapy. Two lesions from her initial presentation are pictured below. They were tender and had a rubbery consistency. A punch biopsy of a representative lesion was performed. Out of concern for superinfection of several lesions, including the pictured facial lesion, erythromycin was started.


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Dr. Clutter is a 1st year resident at NYU Langone Medical Center.