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Title: |
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. | |||||||||||||||||||
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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.
Conclusion: 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. |
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| 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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