Practice questions I Tumors of vessels

Practice questions I
Tumors of vessels
© Jun Wang, MD, PhD

1. A 2-year-old girl presents with a rapidly growing reddish mass on her left face, covering eye-lid, left side of nose, and upper lip. The mass has small foci of ulceration. Purulent exudates are seen. Microbiology culture of the purulent exudates grows non-hemolytic white raised colonies gram-positive, coagulase-negative cocci that form clusters. These bacteria are sensitive to novobiocin. Biopsy of the lesion reveals packed small channel-like spaces lined by benign flat epithelial cells that is positive for CD31. Scattered neutrophilic and lymphoplasmacytic infiltration is seen. What is the most likely diagnosis?
A. Angiosarcoma
B. Bacillary angiomatosis
C. Capillary hemangioma
D. Hemangioblastoma
E. Kaposi sarcoma

2. A 1-year-old boy presents with an ulcerated lesion on his right ear. The lesion is a 2.5 cm well-demarcated, red nodule with peripheral telangiectasia and central ulceration. No other abnormalities are seen. Biopsy of the lesion reveal proliferation of capillaries without significant cytological atypia. Focally there are neutrophilic infiltration and abscess formation. What is the most like outcome of this lesion?
A. Early metastasis
B. Invading into central nervous system through internal auditory meatus
C. Malignant transformation
D. Regression in a few years
E. Sepsis due to infection

3. A 42-year-old man presents with slowly increasing abdominal girth for 3 years. He experiences worsening anorexia and dyspnea on exertion. He denies history of liver, pancreas or biliary system disease. Physical examination reveal non tender mass 4 cm below right costal margin. There is no evidence of ascites. His laboratory tests including liver functions, renal functions and AFP are within normal range. Sonographic examination reveals a 15 cm sharply demarcated mass at the periphery of right lobe of his liver. Biopsy of the mass reveals largely dilated spaces lined by flat cells. Blood components are seen in these spaces. No cytological atypia is seen. These flat cells are positive for CD31. What is the most likely diagnosis?
A. Angiosarcoma
B. Capillary hemangioma
C. Cavernous hemangioma
D. Hepatocellular carcinoma
E. Kaposi sarcoma

4. Use this case for the next three questions. A 47-year-old man presents with headache and upper extremity numbness and weakness for 2 months. Her past medical history is unremarkable. Physical examination confirms reduce strength of both arms. Her laboratory tests are within normal range. Radiologic examination reveals a 0.5 cm well demarcated cerebellar mass. The mass was resected and microscopically it composed of closed packed growth of capillaries contain atypical stromal cells with large dark nuclei. There is no necrosis. Additional radiological work up identified multiple cystic lesions in her liver and both kidneys. What is the most likely diagnosis of the cerebellar lesion?
A. Angiosarcoma
B. Capillary hemangioma
C. Glioblastoma
D. Hemangioblastoma
E. Oligodendroglioma

5. A 47-year-old man presents with headache and upper extremity numbness and weakness for 2 months. Her past medical history is unremarkable. Physical examination confirms reduce strength of both arms . Her laboratory tests are within normal range. Radiologic examination reveals a 0.5 cm well demarcated cerebellar mass. The mass was resected and microscopically it composed of closed packed growth of capillaries contain atypical stromal cells with large dark nuclei. There is no necrosis. Additional radiological work up identified multiple cystic lesions in her liver and both kidneys. Abnormality of what gene is the most likely associated with these findings?
A. APC
B. NF1
C. PRKAR1A
D. TSC
E. VHL

6. A 47-year-old man presents with headache and upper extremity numbness and weakness for 2 months. Her past medical history is unremarkable. Physical examination confirms reduce strength of both arms . Her laboratory tests are within normal range. Radiologic examination reveals a 0.5 cm well demarcated cerebellar mass. The mass was resected and microscopically it composed of closed packed growth of capillaries contain atypical stromal cells with large dark nuclei. There is no necrosis. Additional radiological work up identified multiple cystic lesions in her liver and both kidneys. Elevated risk of what diseases is associated with her conditions?
A. Acute pancreatitis
B. End stage kidney disease
C. Hepatocellular carcinoma
D. Metastatic glioblastoma
E. Renal cell carcinoma

7. An 8-year-old girl presents with easy gingiva bleeding and a rapidly growing mass on her left buccal mucosa for a month. The mass has doubled the size recently. She has a history of sickle cell anemia. Physical examination reveals a 2 cm ulcerated red mass at the right buccal mucosa. No other abnormality is seen. Her lab tests are consistent with sickle cell anemia but otherwise unremarkable. The lesion was removed and microscopically it has a lobular appearance of packed capillaries separated by fibrotic tissue. Scattered neutrophils are seen. No significant cytological atypia is noted. What is the diagnosis?
A. Angiosarcoma
B. Aphthous ulcers
C. Bacillary angiomatosis
D. Kaposi sarcoma
E. Pyogenic granuloma

8. A 69-year-old man presents with a painful papule at his left distal ring finger. His has a history of chronic lymphocytic leukemia 3 years ago that was being followed without therapy. He is otherwise healthy. Physical examination reveals a 5 mm tender purple papule at the dorsal distal interphalangeal joint. The growth appears to be limited to deep dermis. A punch biopsy was performed and reveals a growth of relatively monotonous round cells surrounding vascular spaces. These cells have uniform nuclei and moderate amount of cytoplams. Per immunohistochemistry, these cells are positive for smooth muscle actin and CD34, but negative for CD3, CD20 and CD45. What is the diagnosis?
A. Bacillary angiomatosis
B. Diffuse large B cell lymphoma
C. Glomus tumor
D. Pyogenic granuloma
E. Small lymphocytic lymphoma

9. Use this case for the next three questions. A 39-year-old man presents with shortness of breath, intractable cough, hemoptysis. He has fever, night sweating, and diffuse skin lesions for 6 months and a 35 lb weight loss in one year. He has a history of recurrent respiratory and skin infections. He is an intravenous drug user and has been HIV positive for 9 years. He is currently receiving HAART therapy. He has a 20 pack year history of cigarette smoking, and drinks beers 3 to 5 cans a day for 10 years. Physical examination non-blanching brownish plaques on his face, chest, and lower legs. Thrush is seen in his oral mucosa. Breath sounds were decreased bilaterally. No other abnormalities are noted. Laboratory tests reveals a hemoglobin of 8.5 g/dl (14 – 17g/dl) and CD4 count at 78 cells/mm3 (Normal 500 – 1500 cells/mm3). Chest x-ray showed multiple nodular opacities in both lung fields. CT guided biopsy of the lung lesions reveals atypical spindle cell proliferation with narrow spaces. Extravasated red blood cells are seen. Per immunohistochemistry studies, these cells are positive for CD31 and smooth muscle actin, but negative for cytokeratin. What is the diagnosis of the lung lesions?
A. Angiosarcoma
B. Interstitial pneumonitis
C. Kaposi sarcoma
D. Pneumocystis jirovicii pneumonia
E. Sarcomatoid carcinoma

10. A 39-year-old man presents with shortness of breath, intractable cough, hemoptysis. He has fever, night sweating, and diffuse skin lesions for 6 months and a 35 lb weight loss in one year. He has a history of recurrent respiratory and skin infections. He is an intravenous drug user and has been HIV positive for 9 years. He is currently receiving HAART therapy. He has a 20 pack year history of cigarette smoking, and drinks beers 3 to 5 cans a day for 10 years. Physical examination non-blanching brownish plaques on his face, chest, and lower legs. Thrush is seen in his oral mucosa. Breath sounds were decreased bilaterally. No other abnormalities are noted. Laboratory tests reveals a hemoglobin of 8.5 g/dl (14 – 17g/dl) and CD4 count at 78 cells/mm3 (Normal 500 – 1500 cells/mm3). Chest x-ray showed multiple nodular opacities in both lung fields. CT guided biopsy of the lung lesions reveals atypical spindle cell proliferation with narrow spaces. Extravasated red blood cells are seen. Per immunohistochemistry studies, these cells are positive for CD31 and smooth muscle actin, but negative for cytokeratin. What is associated with his lung lesions?
A. Alcohol
B. Cigarette smoking
C. EB virus
D. Human Herpes Virus 8
E. Human papilloma virus

11. A 39-year-old man presents with shortness of breath, intractable cough, hemoptysis. He has fever, night sweating, and diffuse skin lesions for 6 months and a 35 lb weight loss in one year. He has a history of recurrent respiratory and skin infections. He is an intravenous drug user and has been HIV positive for 9 years. He is currently receiving HAART therapy. He has a 20 pack year history of cigarette smoking, and drinks beers 3 to 5 cans a day for 10 years. Physical examination non-blanching brownish plaques on his face, chest, and lower legs. Thrush is seen in his oral mucosa. Breath sounds were decreased bilaterally. No other abnormalities are noted. Laboratory tests reveals a hemoglobin of 8.5 g/dl (14 – 17g/dl) and CD4 count at 78 cells/mm3 (Normal 500 – 1500 cells/mm3). Chest x-ray showed multiple nodular opacities in both lung fields. CT guided biopsy of the lung lesions reveals atypical spindle cell proliferation with narrow spaces. Extravasated red blood cells are seen. Per immunohistochemistry studies, these cells are positive for CD31 and smooth muscle actin, but negative for cytokeratin. What is the most likely diagnosis of his skin lesions?
A. Angiosarcoma
B. Dermatophytosis
C. Hemangioma
D. Kaposi sarcoma
E. Squamous cell carcinoma

12. Use this case for the next two questions. A 65-year-old man presents for follow up for liver cirrhosis. He has a history of alcoholic liver disease for 15 years. He used to drink whisky 2 glasses per day for 30 years, but quitted after his diagnosis of alcoholic liver disease. He does not smoke cigarette. His history is otherwise unremarkable. Physical examination is unremarkable except multiple foci of dilated small vessels radiating from a central core on his upper chest. Pressing the center core results in the disappearance of the radial vessels, and rapid refill is seen when the compression is relieved. Laboratory tests results are consistent with impaired liver functions. Sonographic examination reveals liver cirrhosis. What is the diagnosis for the skin lesions?
A. Hemangioma
B. Nevus flammeus
C. Port-wine stain
D. Pyogenic granuloma
E. Spider telangiectasia

13. A 65-year-old man presents for follow up for liver cirrhosis. He has a history of alcoholic liver disease for 15 years. He used to drink whisky 2 glasses per day for 30 years, but quitted after his diagnosis of alcoholic liver disease. He does not smoke cigarette. His history is otherwise unremarkable. Physical examination is unremarkable except multiple foci of dilated small vessels radiating from a central core on his upper chest. Pressing the center core results in the disappearance of the radial vessels, and rapid refill is seen when the compression is relieved. Laboratory tests results are consistent with impaired liver functions. Sonographic examination reveals liver cirrhosis. What is associated with the skin lesions?
A. Bartonella bacteria infection
B. Human herpes virus 8
C. Hyperestrinism
D. Portal hypertension
E. Trauma

14. An 8-year-old boy presents with erupt of skin lesions on his face and chest for 1 month. He has a history of Burkitt lymphoma and is currently receiving chemotherapy. Physical examination reveals many dark multiple brown papules and nodules with partial erosion and crusting, up to 1.1 cm in greatest dimension. No other abnormality is seen. Biopsy of these lesion reveal proliferation of dilated small vessels lined by mildly atypical endothelium. Neutrophilic infiltration is seen in the background. Gram stain reveals scattered Gram-negative rod. What is the diagnosis?
A. Angiosarcoma
B. Bacillary angiomatosis
C. Kaposi sarcoma
D. Pyogenic granuloma
E. Recurrent Burkitt lymphoma





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