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Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad
This case talks about a 50 year old female with a renal mass.
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1. Question
Case 1: This is a 50 year old female with a renal mass.
Diagnosis:
Correct
Answer: B
Histologic Description: This is a cystic renal neoplasm which contains a cellular spindle cell stroma and cysts which are lined by bland renal tubular epithelial cells. The stroma has a fascicular pattern, lacks significant mitotic activity, and is associated with hyalinized collagen, and thus is reminiscent of ovarian stroma. The cellular stroma entraps native renal tubules at its periphery. The stroma labeled diffusely for estrogen receptor and in a patchy fashion for inhibin, supporting the diagnosis of mixed epithelial stromal tumor.Differential Diagnosis: Angiomyolipoma with epithelial cysts also might label with hormone receptors and contains entrapped native renal tubular epithelium forming cysts; however, the stroma is that of a stromal predominant angiomyolipoma, and thus would label for HMB45 and muscle markers. Cystic partially differentiated nephroblastoma would be exclusively cystic and lack solid areas. The stroma would contain primitive nephroblastic elements similar to those seen in Wilms tumor, though limited to the thin septa of the lesion. Primary renal synovial sarcoma would feature greater mitotic activity, typically would not label for estrogen receptor, and would demonstrate the characteristic SS18-SSX1/2 gene fusions of that entity.
Incorrect
Answer: B
Histologic Description: This is a cystic renal neoplasm which contains a cellular spindle cell stroma and cysts which are lined by bland renal tubular epithelial cells. The stroma has a fascicular pattern, lacks significant mitotic activity, and is associated with hyalinized collagen, and thus is reminiscent of ovarian stroma. The cellular stroma entraps native renal tubules at its periphery. The stroma labeled diffusely for estrogen receptor and in a patchy fashion for inhibin, supporting the diagnosis of mixed epithelial stromal tumor.Differential Diagnosis: Angiomyolipoma with epithelial cysts also might label with hormone receptors and contains entrapped native renal tubular epithelium forming cysts; however, the stroma is that of a stromal predominant angiomyolipoma, and thus would label for HMB45 and muscle markers. Cystic partially differentiated nephroblastoma would be exclusively cystic and lack solid areas. The stroma would contain primitive nephroblastic elements similar to those seen in Wilms tumor, though limited to the thin septa of the lesion. Primary renal synovial sarcoma would feature greater mitotic activity, typically would not label for estrogen receptor, and would demonstrate the characteristic SS18-SSX1/2 gene fusions of that entity.
