Practice questions renal neoplasms

Practice questions II
Renal neoplasms
© Jun Wang, MD, PhD

1. Use this case for next three questions. A 45-year-old man presents with left flank discomfort for 4 months. He has a history of seizure and arrhythmia since age of 8. He denies history of other health problems. He does not smoke nor drink alcohol. His father has a history of arrhythmia. His mother has type II diabetes and diabetic nephropathy. He has an irregularly irregular pulse rate at approximately 68 per minute. His vitals signs are otherwise unremarkable. No other abnormalities are noted through physical examination, except a few brown skin fibrous plaque on his face. Radiologic examinations reveal a 5.5 cm solid mass at his left kidney. His right kidney is unremarkable. Cardiac sonographic examination reveal multiple well-circumscribed myocardial mass in the left ventricle. Biopsy of the renal mass reveal distorted vessles with thick walls, scattered adipose tissue and bundles of spindle cells. There is no evidence of cytological atypia or necrosis. Immunohistochemistry studies reveal the spindle cells are positive for vimentin and desmin. What is most likely the diagnosis?
A. Angiomyolipoma
B. Collecting duct carcinoma
C. Papillary adenoma
D. Rhabdomyoma
E. Well differentiated liposarcoma

2. A 45-year-old man presents with left flank discomfort for 4 months. He has a history of seizure and arrhythmia since age of 8. He denies history of other health problems. He does not smoke nor drink alcohol. His father has a history of arrhythmia. His mother has type II diabetes and diabetic nephropathy. He has an irregularly irregular pulse rate at approximately 68 per minute. His vitals signs are otherwise unremarkable. No other abnormalities are noted through physical examination, except a few brown skin fibrous plaque on his face. Radiologic examinations reveal a 5.5 cm solid mass at his left kidney. His right kidney is unremarkable. Cardiac sonographic examination reveal multiple well-circumscribed myocardial mass in the left ventricle. Biopsy of the renal mass reveal distorted vessles with thick walls, scattered adipose tissue and bundles of spindle cells. There is no evidence of cytological atypia or necrosis. Immunohistochemistry studies reveal the spindle cells are positive for vimentin and desmin. What additional markers likely to be expressed by the kidney lesion?
A. CK7
B. HMB45
C. p63
D. TFE-3
E. WT-1

3. A 45-year-old man presents with left flank discomfort for 4 months. He has a history of seizure and arrhythmia since age of 8. He denies history of other health problems. He does not smoke nor drink alcohol. His father has a history of arrhythmia. His mother has type II diabetes and diabetic nephropathy. He has an irregularly irregular pulse rate at approximately 68 per minute. His vitals signs are otherwise unremarkable. No other abnormalities are noted through physical examination, except a few brown skin fibrous plaque on his face. Radiologic examinations reveal a 5.5 cm solid mass at his left kidney. His right kidney is unremarkable. Cardiac sonographic examination reveal multiple well-circumscribed myocardial mass in the left ventricle. Biopsy of the renal mass reveal distorted vessles with thick walls, scattered adipose tissue and bundles of spindle cells. There is no evidence of cytological atypia or necrosis. Immunohistochemistry studies reveal the spindle cells are positive for vimentin and desmin. Mutation of what gene is likely associated with his condition?
A. MET
B. PAX6
C. TSC2
D. VHL
E. WT-1


4. A 63-year-old man presents with left flank pain for 6 months. He denies any history of trauma. His has a history of stage I colon cancer 10 years ago, that was resected. Physical examination reveals a vaguely palpable left abdominal mass. There is no evidence of hepatosplenomegaly. His laboratory results are within normal range. CT scan reveal a 15 cm well circumscribed solid mass at left kidney. Biopsy of the mass reveal cords of cells with small round to oval nuclei and abundant eosinophilic granular cytoplasm. No cytological atypia or necrosis is seen. These cells show patchy reactivity to CK7 and are negative for colloid iron stain, CK20. What is most likely the diagnosis?
A. Adult Wilms tumor
B. Chromophobe renal cell carcinoma
C. Metastatic colon adenocarcinoma
D. Oncocytoma
E. Urothelial carcinoma of renal pelvis


5. Use this case for next three questions. A 42-year-old woman presents with fatigue and progressive abdominal distention for 3 months. She has lost 20 lb unintentionally. She does not have flank or abdominal pain, bowel habit change or hematuria. She was diagnosed with a neuroendocrine tumor of pancreas that was treated with surgery. Physical examination reveals a tender mass at left upper abdomen. No lymphadenopathy, hepatosplenomegaly are noted. Laboratory studies reveal a hemoglobin of 5.5 g/dL (normal 12-16 g/dL). Her other laboratory test results are within normal range. CT scan reveal an 18 cm solid mass at her left kidney. Biopsy of the mass reveal nests of cells with clear cytoplasm and enlarged irregular nuclei with various sizes and darkness of chromatin. Very fine vessels are seen surrounding these nests. Immunohistochemistry studies reveal positive reactivity to both cytokeratin and vimentin, but negative for CD56 and chromogranin. What is most likely the diagnosis?
A. Clear cell renal cell carcinoma
B. Chromophobe renal cell carcinoma
C. Metastatic pancreatic neuroendocrine tumor
D. Oncocytoma
E. Urothelial carcinoma of renal pelvis

6. A 42-year-old woman presents with fatigue and progressive abdominal distention for 3 months. She has lost 20 lb unintentionally. She does not have flank or abdominal pain, bowel habit change or hematuria. She was diagnosed with a neuroendocrine tumor of pancreas that was treated with surgery. Physical examination reveals a tender mass at left upper abdomen. No lymphadenopathy, hepatosplenomegaly are noted. Laboratory studies reveal a hemoglobin of 5.5 g/dL (normal 12-16 g/dL). Her other laboratory test results are within normal range. CT scan reveal an 18 cm solid mass at her left kidney. Biopsy of the mass reveal nests of cells with clear cytoplasm and enlarged irregular nuclei with various sizes and darkness of chromatin. Very fine vessels are seen surrounding these nests. Immunohistochemistry studies reveal positive reactivity to both cytokeratin and vimentin, but negative for CD56 and chromogranin. What genetic mutation is likely detected in this lesion?
A. MET
B. PAX6
C. TSC2
D. VHL
E. WT-1

7. A 42-year-old woman presents with fatigue and progressive abdominal distention for 3 months. She has lost 20 lb unintentionally. She does not have flank or abdominal pain, bowel habit change or hematuria. She was diagnosed with a neuroendocrine tumor of pancreas that was treated with surgery. Physical examination reveals a tender mass at left upper abdomen. No lymphadenopathy, hepatosplenomegaly are noted. Laboratory studies reveal a hemoglobin of 5.5 g/dL (normal 12-16 g/dL). Her other laboratory test results are within normal range. CT scan reveal an 18 cm solid mass at her left kidney. Biopsy of the mass reveal nests of cells with clear cytoplasm and enlarged irregular nuclei with various sizes and darkness of chromatin. Very fine vessels are seen surrounding these nests. Immunohistochemistry studies reveal positive reactivity to both cytokeratin and vimentin, but negative for CD56 and chromogranin. What genetic background is most likely associated with her presentations?
A. Birt-Hogg-Dubé syndrome
B. Hereditary papillary renal carcinoma
C. Tuberous sclerosis
D. von Hippel-Lindau syndrome
E. WAGR syndrome


8. A 77-year-old woman presents with slowing enlarged anterior neck for 6 months. She denies history of voice change, fever or weight loss. She has a history of certain type of kidney tumor 35 years ago that was treated with nephrectomy. Physical examination reveal a slightly enlarged thyroid. No enlarged lymph nodes are seen. Her laboratory test results are within normal range. Sonographic examination of her thyroid reveal a 1.5 cm nodule in the right lobe. Core biopsy reveal nests of cells with small nuclei and clear cytoplasm. No nuclear groove or inclusions are seen. Delicate capillaries are seen surrounding these nests. There is no definitive evidence of invasion. Immunohistochemistry studies reveal these cells are positive for cytokeratin, but negative for thyroglobulin, TTF-1, calcitonin and parathyroid hormones. What is the most likely diagnosis?
A. Follicular adenoma
B. Medullary carcinoma
C. Metastatic renal cell carcinoma
D. Multinodular goiter
E. Papillary carcinoma of thyroid


9. Use this case for next two questions. A 69-year-old woman presents with fever and productive cough with yellowish sputum for 1 week. She denies other symptoms. She has stage I colonic adenocarcinoma and was treated colectomy 10 years ago.  She does not smoke nor drink alcohol. Physical examination reveals a temperature at 38.5 °C, heart rate at 98, and respiratory rate at 25. No other abnormalities are noted. Her laboratory results including liver and renal function tests are within normal range, except slightly elevated white cells with normal differential. Chest X-ray reveal a 2.5 cm nodule at the periphery area of right lower lobe. Biopsy of the nodule reveal nests of atypical cells with eosinophilic granular cytoplasm, slightly pleomorphic nuclei with perinuclear halo. No intercellular bridges or squamous pears are seen. These cells are positive for colloid iron. Per immunohistochemistry studies, these cells are positive for CK7 but negative for CK20 and TTF1. What should be done next for her?
A. Broad spectrum antibiotics
B. Image studies for chest, abdomen and pelvis
C. Molecular studies for EGFR mutation
D. Serum test for HIV infection
E. Sputum culture

10. A 69-year-old woman presents with fever and productive cough with yellowish sputum for 1 week. She denies other symptoms. She has stage I colonic adenocarcinoma and was treated colectomy 10 years ago. She does not smoke nor drink alcohol. Physical examination reveals a temperature at 38.5 °C, heart rate at 98, and respiratory rate at 25. No other abnormalities are noted. Her laboratory results including liver and renal function tests are within normal range, except slightly elevated white cells with normal differential. Chest X-ray reveal a 2.5 cm nodule at the periphery area of right lower lobe. Biopsy of the nodule reveal nests of atypical cells with eosinophilic granular cytoplasm, slightly pleomorphic nuclei with perinuclear halo. No intercellular bridges or squamous pears are seen. These cells are positive for colloid iron. Per immunohistochemistry studies, these cells are positive for CK7 but negative for CK20 and TTF1. What is most likely the diagnosis?
A. Adenocarcinoma of lung
B. Metastatic chromophobe renal cell carcinoma
C. Metastatic colon adenocarcinoma
D. Pulmonary abscess
E. Squamous cell carcinoma of lung


11. Use this case for next two questions. A 72-year-old woman presents with fatigue, loin pain and hematuria for 3 months. She has a history of spontaneous pneumothorax, hypertension, atherosclerosis and coronary artery disease. She was diagnosed with adenocarcinoma of left lung and was treated with surgery and chemotherapy 5 years ago. Physical examination is unremarkable except multiple skin tags. Her CBC a hemoglobin at 11 g/dL (normal 12-16 g/dL), and urinalysis reveals approximately 15 red cell/high power field (normal less than 4/HPF). Her lab results are otherwise unremarkable. CT scan reveal a 1.5 cm well circumscribed lesion at the upper pole of left kidney. Core biopsy reveal nest of cells with small to intermediate sized round to oval nuclei and eosinophilic granular cytoplasm with perinuclear halo. Scarring tissue is focally seen. There is no evidence of necrosis. These cells are positive for colloid iron. Immunohistochemistry studies reveal diffuse reactivity to CK7, but not TTF1. What is most likely the diagnosis?
A. Clear cell renal cell carcinoma
B. Chromophobe renal cell carcinoma
C. Metastatic adenocarcinoma of lung
D. Oncocytoma
E. Urothelial carcinoma of renal pelvis

12. A 72-year-old woman presents with fatigue, loin pain and hematuria for 3 months. She has a history of spontaneous pneumothorax, hypertension, atherosclerosis and coronary artery disease. She was diagnosed with adenocarcinoma of left lung and was treated with surgery and chemotherapy 5 years ago. Physical examination is unremarkable except multiple skin tags. Her CBC a hemoglobin at 11 g/dL (normal 12-16 g/dL), and urinalysis reveals approximately 15 red cell/high power field (normal less than 4/HPF). Her lab results are otherwise unremarkable. CT scan reveal a 1.5 cm well circumscribed lesion at the upper pole of left kidney. Core biopsy reveal nest of cells with small to intermediate sized round to oval nuclei and eosinophilic granular cytoplasm with perinuclear halo. Scarring tissue is focally seen. There is no evidence of necrosis. These cells are positive for colloid iron. Immunohistochemistry studies reveal diffuse reactivity to CK7, but not TTF1. Abnormality of what gene is likely to be seen in this lesion?
A. BHD mutation
B. MET mutation
C. Xp11 translocation
D. VHL mutation
E. WT-1 mutation


13. Use this case for next two questions. A 77-year-old woman presents with vague left flank discomfort for 3 months. She denies any history of trauma. She has history of papillary carcinoma of thyroid at age of 45, and was treated with total thyroidectomy and hormone replacement. Physical examination and laboratory tests are within normal range. CT scan reveal a 7.2 cm poorly circumscribed solid mass at the lower pole of the left kidney. Core biopsy reveal finger-like growth with fibrovascular core lined by cuboid cells with moderate atypia. The lining cells have no nuclear inclusion or grooves. Clusters of foamy histiocytes and foci of calcification or necrosis are seen. Immunohistochemistry studies reveal negative reactivity to thyroglobulin and TTF-1. What is the most likely diagnosis?
A. Chromophobe renal cell carcinoma
B. Metastatic papillary carcinoma of thyroid
C. Papillary adenoma
D. Papillary renal cell carcinoma
E. Urothelial carcinoma of renal pelvis

14. A 77-year-old woman presents with vague left flank discomfort for 3 months. She denies any history of trauma. She has history of papillary carcinoma of thyroid at age of 45, and was treated with total thyroidectomy and hormone replacement. Physical examination and laboratory tests are within normal range. CT scan reveal a 7.2 cm poorly circumscribed solid mass at the lower pole of the left kidney. Core biopsy reveal finger-like growth with fibrovascular core lined by cuboid cells with moderate atypia. The lining cells have no nuclear inclusion or grooves. Clusters of foamy histiocytes and foci of calcification or necrosis are seen. Immunohistochemistry studies reveal negative reactivity to thyroglobulin and TTF-1. Abnormality of what gene is likely to be seen in this lesion?
A. BHD
B. MET
C. TSC2
D. VHL
E. WT-1


15. A 57-year-old woman presents with right flank pain for a month. She denies history of trauma or hematuria. Physical examination and laboratory studies are unremarkable. Sonographic examination reveals a 5.5 cm well circumscribed mass at the central portion of her right kidney. No other abnormalities are noted. Biopsy of the mass reveal solid cords and nests of atypical cells with marked pleomorphism and scan cytoplasm. Whole body scan reveals no other tumors. What is most likely the diagnosis?
A. Angiomyolipoma
B. Chromophobe renal cell carcinoma
C. Collecting duct carcinoma
D. Papillary renal cell carcinoma
E. Urothelial carcinoma of renal pelvis


16. Use this case for next two questions. A 69-year-old man presents with painless hematuria for 2 weeks. He has no other symptoms. His past medical history is unremarkable. He was coal miner for 40 years before retirement. He has been smoking cigarette 1 and a half pack a day for 50 years, and occasionally drink beers and tequila. Physical examination is unremarkable. His CBC is within normal range. Urinalysis reveals more than 15 red blood cells/high power field (normal < 4 RBCs/HPF). Markedly atypical epithelial cell are seen. CT scan reveal a 1.5 cm mass at the mid portion of his left kidney. Retrograde pyelogram demonstrates filling defect in left pelvis. Cystoscopic and ureteroscopic examinations with multiple biopsies show no evidence of neoplasms. Biopsy of the left kidney mass reveal finger like growth with fibrovascular core covered by multiple layers of squamoid cells with marked cytological atypia and active mitosis. Necrosis are seen focally. What is the most likely diagnosis?
A. Clear cell renal cell carcinoma
B. Chromophobe renal cell carcinoma
C. Collecting duct carcinoma
D. Papillary renal cell carcinoma
E. Urothelial carcinoma of renal pelvis

17. A 69-year-old man presents with painless hematuria for 2 weeks. He has no other symptoms. His past medical history is unremarkable. He was coal miner for 40 years before retirement. He has been smoking cigarette 1 and a half pack a day for 50 years, and occasionally drink beers and tequila. Physical examination is unremarkable. His CBC is within normal range. Urinalysis reveals more than 15 red blood cells/high power field (normal < 4 RBCs/HPF). Markedly atypical epithelial cell are seen. CT scan reveal a 1.5 cm mass at the mid portion of his left kidney. Retrograde pyelogram demonstrates filling defect in left pelvis. Cystoscopic and ureteroscopic examinations with multiple biopsies show no evidence of neoplasms. Biopsy of the left kidney mass reveal finger like growth with fibrovascular core covered by multiple layers of squamoid cells with marked cytological atypia and active mitosis. Necrosis are seen focally. What is the most likely findings by UroVysion study in this patient?
A. Aneuploidy of chromosomes 3, 7, 17, and loss of the 9p21
B. BHD mutation
C. Deletion of VHL
D. Mutation of MET
E. Xp11 translocation


18. Use this case for next two questions. A 2-year-old boy presents with fever and decreased activity for 3 days. His past medical history is unremarkable. Physical examination reveal a 7 cm mass at the right upper abdomen. The mass is slightly firm and non-movable. His CBC reveals a hemoglobin at 7.5 g/dL (normal 11.5-14.5 g/dL). No other abnormal laboratory test results are noted. CT scan reveal a poorly demarcated mass in his right kidney. His left kidney is unremarkable.  A right nephrectomy was performed. Pathological examination of the mass reveal scatter irregular glandular structures lined by cells with irregular dark nuclei, nests of epithelioid cells with large irregular nuclei and scant cytoplasm, in a fibrotic background. Necrosis is focally seen. Per immunohistochemistry studies, all the cells are positive WT-1 and negative to S-100. What is the most likely diagnosis?
A. Collecting duct carcinoma
B. Neuroblastoma
C. Xp11 translocation renal cell carcinoma
D. Urothelial carcinoma of renal pelvis
E. Wilms tumor

19. A 2-year-old boy presents with fever and decreased activity for 3 days. His past medical history is unremarkable. Physical examination reveal a 7 cm mass at the right upper abdomen. The mass is slightly firm and non-movable. His CBC reveals a hemoglobin at 7.5 g/dL (normal 11.5-14.5 g/dL). No other abnormal laboratory test results are noted. CT scan reveal a poorly demarcated mass in his right kidney. His left kidney is unremarkable.  A right nephrectomy was performed. Pathological examination of the mass reveal scatter irregular glandular structures lined by cells with irregular dark nuclei, nests of epithelioid cells with large irregular nuclei and scant cytoplasm, in a fibrotic background. Necrosis is focally seen. Per immunohistochemistry studies, all the cells are positive WT-1 and negative to S-100. Abnormality of what gene is likely to be seen in this lesion?
A. BHD
B. MET
C. TSC2
D. VHL
E. WT-1


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