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Renal medullary carcinoma (RMC) is a very rare disease that is almost exclusive to young black patients with sickle cell trait. Most patients present with metastatic disease at diagnosis and have a grim prognosis. Histologic diagnosis of these tumors can be difficult in the presence of rhabdoid elements and loss of INI1 expression as observed in our case. We describe the first known reported case of a white male adolescent with sickle cell trait, who was treated for a metastatic RMC with reticular pattern of rhabdoid-like cells and absence of INI1 expression, by the European Soft Tissue Sarcoma Study Group (EpSSG) protocol designed for rhabdoid tumors and who achieved a transitory complete response. Although complete remission was achieved, overall survival was not improved.

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Transitory Complete Response to the EpSSG Extracranial Rhabdoid Tumor Protocol in a White Adolescent with a Sickle Trait Harboring a Metastatic Renal Medullary Carcinoma