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Presented by Pedram Argani, M.D. and prepared by Jeremy Vincent M.D.
Case 2: This is a 10 year old with a renal tumor.
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Question 1 of 1
1. Question
Week 538: Case 2
This is a 10 year old with a renal tumor.images/vincent/5575_1.jpg
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images/vincent/5575_4.jpgCorrect
Answer: Renal medullary carcinoma
Histology: This is a high grade epithelial neoplasm which has a myxoid, acutely inflamed, desmoplastic stroma and neoplastic cells which have vesicular chromatin, prominent nucleoli, and eosinophilic cytoplasm. Focally, one can appreciate sickle cells in the histologic sections, the tumor cells show loss of labeling for INI-1 protein. These are the typical features of a renal medullary carcinoma.
Discussion: Clear cell carcinomas may have rhabdoid features like the current case. However, they typically do not show loss INI-1 protein, and are uncommon in this age group. Rhabdoid tumor of the kidney overlaps greatly with renal medullary carcinoma at the morphologic and immunohistochemical levels, in that both show loss of INI-1 protein. However, rhabdoid tumor of the kidney typically affects patients less than 3 years of age, and is unassociated with sickle cell trait. Primitive neuroectodermal tumor is a serious clinical concern in this age group; however, this is a round cell tumor unlike the current lesion which has open, vesicular chromatin and prominent nucleoli.
Incorrect
Answer: Renal medullary carcinoma
Histology: This is a high grade epithelial neoplasm which has a myxoid, acutely inflamed, desmoplastic stroma and neoplastic cells which have vesicular chromatin, prominent nucleoli, and eosinophilic cytoplasm. Focally, one can appreciate sickle cells in the histologic sections, the tumor cells show loss of labeling for INI-1 protein. These are the typical features of a renal medullary carcinoma.
Discussion: Clear cell carcinomas may have rhabdoid features like the current case. However, they typically do not show loss INI-1 protein, and are uncommon in this age group. Rhabdoid tumor of the kidney overlaps greatly with renal medullary carcinoma at the morphologic and immunohistochemical levels, in that both show loss of INI-1 protein. However, rhabdoid tumor of the kidney typically affects patients less than 3 years of age, and is unassociated with sickle cell trait. Primitive neuroectodermal tumor is a serious clinical concern in this age group; however, this is a round cell tumor unlike the current lesion which has open, vesicular chromatin and prominent nucleoli.
