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Presented by Pedram Argani, M.D. and prepared by Aatur Singhi, M.D., Ph.D.
Case 6: This is 40 year-old female with a perirenal mass.
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1. Question
Week 412: Case 6
This is 40 year-old female with a perirenal mass.images/1alex/101909case6image1.jpg
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images/1alex/101909case6image4.jpgCorrect
Answer: Leiomyosarcoma
Histology: There is a highly pleomorphic spindle cell neoplasm associated with the renal vein. Cells have hyperchromatic nuclei, and demonstrate high mitotic activity including atypical forms. The cells grow in a fascicular pattern, and there is an abundant eosinophilic cytoplasm associated with each cell. These are the typical features of a smooth muscle tumor. The extent of atypia and mitotic activity support a diagnosis of leiomyosarcoma.
Discussion: A sarcomatoid renal cell carcinoma could grow into the renal vein and simulate leiomyosarcoma. A sarcomatoid renal cell carcinoma would be associated with a carcinoma component which would typically label for epithelial markers. A pleomorphic angiomyolipoma is an excellent mimic of leiomyosarcoma when it is composed of predominantly spindle cells. This lesion should label for melanocytic markers HMB45 and melan A. Other components of angiomyolipoma such as fat and dysplastic vessels could also be present. Cellular schwannoma is an excellent mimic of leiomyosarcoma in the retroperitoneum and abdomen. A cellular schwannoma would label for S100 protein diffusely but not for desmin. In addition, while mitotic activity is permitted in a cellular schwannoma, the atypical mitotic forms seen in the current case would not be.
Incorrect
Answer: Leiomyosarcoma
Histology: There is a highly pleomorphic spindle cell neoplasm associated with the renal vein. Cells have hyperchromatic nuclei, and demonstrate high mitotic activity including atypical forms. The cells grow in a fascicular pattern, and there is an abundant eosinophilic cytoplasm associated with each cell. These are the typical features of a smooth muscle tumor. The extent of atypia and mitotic activity support a diagnosis of leiomyosarcoma.
Discussion: A sarcomatoid renal cell carcinoma could grow into the renal vein and simulate leiomyosarcoma. A sarcomatoid renal cell carcinoma would be associated with a carcinoma component which would typically label for epithelial markers. A pleomorphic angiomyolipoma is an excellent mimic of leiomyosarcoma when it is composed of predominantly spindle cells. This lesion should label for melanocytic markers HMB45 and melan A. Other components of angiomyolipoma such as fat and dysplastic vessels could also be present. Cellular schwannoma is an excellent mimic of leiomyosarcoma in the retroperitoneum and abdomen. A cellular schwannoma would label for S100 protein diffusely but not for desmin. In addition, while mitotic activity is permitted in a cellular schwannoma, the atypical mitotic forms seen in the current case would not be.
