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Presented by Dr. Pedram Argani and prepared by Dr. Harsimar Kaur
This is a 7 year old male with a renal mass.
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1. Question
This is a 7 year old male with a renal mass.
Correct
Answer: C
Histologic Description: This is a nested clear cell tumor associated abundant brown melanin pigment, leading to a black gross appearance. The neoplastic cells label for HMB45, but not for epithelial markers such as cytokeratins or for renal tubular markers like PAX8. Break-apart FISH demonstrates a TFE3 gene rearrangement.
Differential Diagnosis: Xp11 translocation renal cell carcinoma may express melanocytic markers, but should express PAX8 and often express some epithelial markers such as cytokeratins or EMA. Melanoma may have a balloon cell appearance, but is unassociated with TFE3 rearrangements. Clear cell sarcoma is typically characterized the gene fusion EWS-ATF1, but not with TFE3 gene rearrangements. It would also label for S100 protein unlike the current lesion.
The lesion illustrated herein was originally reported as melanotic Xp11 translocation renal cancer. With a greater experience, we believe these are closely related to Xp11 translocation PEComas, and represent a variant of that with melanin pigment.
References
Argani P, Illei P, Netto G, Ro J, Cho HY, Dogan S, Ladanyi M, Martignoni G, Aulmann S, Weiss SW. A Distinctive Subset of PEComas Harbor TFE3 Gene Fusions. Am J Surg Pathol 2010; 34: 1395-1406.
Argani P, Aulmann S, Karanjawala Z, Fraser RB, Ladanyi M, Rodriguez MM. Melanotic Xp11 translocation Renal Cancers: A Distinctive Neoplasm with overlapping features of PEComa, Carcinoma, and Melanoma. Am J Surg Pathol 2009; 33: 609-619.Incorrect
Answer: C
Histologic Description: This is a nested clear cell tumor associated abundant brown melanin pigment, leading to a black gross appearance. The neoplastic cells label for HMB45, but not for epithelial markers such as cytokeratins or for renal tubular markers like PAX8. Break-apart FISH demonstrates a TFE3 gene rearrangement.
Differential Diagnosis: Xp11 translocation renal cell carcinoma may express melanocytic markers, but should express PAX8 and often express some epithelial markers such as cytokeratins or EMA. Melanoma may have a balloon cell appearance, but is unassociated with TFE3 rearrangements. Clear cell sarcoma is typically characterized the gene fusion EWS-ATF1, but not with TFE3 gene rearrangements. It would also label for S100 protein unlike the current lesion.
The lesion illustrated herein was originally reported as melanotic Xp11 translocation renal cancer. With a greater experience, we believe these are closely related to Xp11 translocation PEComas, and represent a variant of that with melanin pigment.
References
Argani P, Illei P, Netto G, Ro J, Cho HY, Dogan S, Ladanyi M, Martignoni G, Aulmann S, Weiss SW. A Distinctive Subset of PEComas Harbor TFE3 Gene Fusions. Am J Surg Pathol 2010; 34: 1395-1406.
Argani P, Aulmann S, Karanjawala Z, Fraser RB, Ladanyi M, Rodriguez MM. Melanotic Xp11 translocation Renal Cancers: A Distinctive Neoplasm with overlapping features of PEComa, Carcinoma, and Melanoma. Am J Surg Pathol 2009; 33: 609-619.
