Renal medullary carcinoma (RMC) is a highly aggressive renal cell carcinoma, arising in the collecting system, and requiring careful correlation with status of sickle cell trait. A panel of international experts has recently proposed provisional diagnostic terminology, renal cell carcinoma, unclassified, with medullary phenotype (RCCU-MP), based on encountering an extraordinarily rare tumor with RMC morphology and immunophenotype in an individual proven not to have a hemoglobinopathy. In Jul 14, 2017, Deepika Sirohi and others published an article in << Human Pathology >> which title is“Renal Cell Carcinoma, Unclassified with Medullary Phenotype: Poorly-Differentiated Adenocarcinomas Overlapping with Renal Medullary Carcinoma”, extend this observation to a cohort of five such tumors, morphologically similar to RMC, lacking SMARCB1 expression by immunohistochemistry, but each without evidence of a hemoglobinopathy. In summary, this first series of RCCU-MPs documents tumors with morphologic, immunophenotypic, and prognostic features of RMC, occurring in individuals without sickle cell trait. While greater biologic and molecular understanding is needed, the available evidence points to these cases representing a sporadic counterpart to sickle cell trait-associated RMC.
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