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Presented by George Netto, M.D. and prepared by Zarir E. Karanjawala, M.D., Ph.D.
Case 6: 17 year old African American male with a longstanding history of sickle cell anemia.
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1. Question
Week 326: Case 6
17 year old African American male with a longstanding history of sickle cell anemia was found to have an 11 cm right renal mass. A radical nephrectomy was performed./images/netto100107_6h.jpg
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Answer: Renal medullary carcinoma
Histology: In the first report on this highly aggressive type of renal malignancy, Davis et al. from the AFIP described 34 cases of rare centrally located renal neoplasms involving renal sinuses and calyces. With the exception of one patient, all patients had sickle cell trait. Patients were predominantly African Americans between the ages of 11 and 39 years. Medullary Carcinomas have variable morphologic patterns including a frequently exhibited reticular, yolk sac-like, or adenoid cystic appearance with clusters of poorly differentiated epithelial cells in a highly desmoplastic stroma admixed with neutrophils and lymphocytes.
Discussion: Medullary Carcinomas are usually metastatic at the time of presentation and are almost uniformly lethal within months of diagnosis. A single long-term survival has been reported in the latest series on seven such tumors from Brazil by Watanabe et al.
The differential diagnosis of medullary carcinoma includes collecting duct carcinoma and urothelial carcinoma. Unlike medullary carcinoma, both of the latter tumors are usually positive for high molecular cytokeratin.
Reference(s):
– Davis CJ Jr, Mostofi FK, Sesterhenn IA. Renal medullary carcinoma. The seventh sickle cell nephropathy. Am J Surg Pathol. (1995)19:1-11.
– Watanabe IC, Billis A, Guimarães MS, Alvarenga M, de Matos AC, Cardinalli IA, Filippi RZ, de Castro MG, Suzigan S.Renal medullary carcinoma: report of seven cases from Brazil. Mod Pathol. (2007)20:914-920.Incorrect
Answer: Renal medullary carcinoma
Histology: In the first report on this highly aggressive type of renal malignancy, Davis et al. from the AFIP described 34 cases of rare centrally located renal neoplasms involving renal sinuses and calyces. With the exception of one patient, all patients had sickle cell trait. Patients were predominantly African Americans between the ages of 11 and 39 years. Medullary Carcinomas have variable morphologic patterns including a frequently exhibited reticular, yolk sac-like, or adenoid cystic appearance with clusters of poorly differentiated epithelial cells in a highly desmoplastic stroma admixed with neutrophils and lymphocytes.
Discussion: Medullary Carcinomas are usually metastatic at the time of presentation and are almost uniformly lethal within months of diagnosis. A single long-term survival has been reported in the latest series on seven such tumors from Brazil by Watanabe et al.
The differential diagnosis of medullary carcinoma includes collecting duct carcinoma and urothelial carcinoma. Unlike medullary carcinoma, both of the latter tumors are usually positive for high molecular cytokeratin.
Reference(s):
– Davis CJ Jr, Mostofi FK, Sesterhenn IA. Renal medullary carcinoma. The seventh sickle cell nephropathy. Am J Surg Pathol. (1995)19:1-11.
– Watanabe IC, Billis A, Guimarães MS, Alvarenga M, de Matos AC, Cardinalli IA, Filippi RZ, de Castro MG, Suzigan S.Renal medullary carcinoma: report of seven cases from Brazil. Mod Pathol. (2007)20:914-920.
