Friday, February 22nd, 2019


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Chromophobe Renal Cell Carcinoma with a Large Hemorrhagic Pseudocystic Appearance Dedifferentiated to a Prominent Rhabdoid Phenotype with a Focal Myxoid Stroma: A Case Report
Authors:  Diana Rodriguez-Villar, M.D., Laura Ruger-Jimenez, M.D., Maria Poch-Arenas, M.D., Rebeca Blanco-Fernandez, M.D., Sergio Fernandez-Pello Montes, M.D., and Raul Rodriguez-Aguilar, M.D., Ph.D.
  BACKGROUND: Renal cell carcinoma nitric oxide synthase (NOS) rarely shows a rhabdoid morphology, and even more uncommonly in the chromophobe subtype. A case of chromophobe renal cell carcinoma (ChRCC) with a partial hemorrhagic pseudocystic appearance and a prominent rhabdoid component is reported. CASE: A 53-year-old man presented with microhematuria, and subsequently a 9-cm renal mass was discovered. Grossly, it was a largely hemorrhagic pseudocystic tumor with peripheral white solid masses corresponding to a ChRCC (NOS and eosinophilic subtype), dedifferentiated to a focal sarcomatoid and a prominent rhabdoid morphology, dyscohesive, sometimes in a myxoid stroma and in an inflammatory background, also present in the large hemorrhagic, necrotic, pseudocystic central area. Clinicopathology and immunohistochemical studies are described.
There is no loss of SMARCB1 integrase interactor 1 expression in the rhabdoid component. Rhabdoid morphology is a dedifferentiated phenotype in different renal neoplasms, related to high grade, high stage, and poor outcome, like the present case, which highlights the importance of adequate sampling.
Keywords:  integrase interactor 1 protein, rhabdoid tumor, SMARCB1 protein, SWI/SNF complex, swi-snf-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1
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