Renal Cell Carcinoma with Sarcomatoid and Rhabdoid Dedifferentiation: Clinico Pathological Significance- Review

Abstract

Introduction Sarcomatoid and/or rhabdoid dedifferentiation are rare histopathological findings which may be heterogeneous in renal cell carcinoma. Sarcomatoid renal cell carcinoma shows marked cytologic atypia and containing enlarged pleomorphic and malignant spindle cells reminiscent of sarcoma. It is highly aggressive with a high metastatic potential and extremely poor prognosis. Case report A 78-year-old male presented with complaints hematuria, pain abdomen, burring maturation of 2 months duration. The patient had a history of hypertension and diabetes mellitus. Computed tomography revealed a well-defined lobulated, exophytic ball-type, isodense lesion, measuring (~ 7.8 x 7.5 x 11.0 cm) arising from mid and lower poles of right kidney involving renal sinus. Features were suggestive of renal cell carcinoma. The patient underwent a right radical nephrectomy. On histopathological findings reported as clear cell RCC, sarcomatoid and rhabdoid dedifferentiation, Fuhrman grade IV. There was no evidence of any metastasis. The immunochemistry was positive for Paired box 8 (PAX8), Carbonic anhydrase 9 (CA9). The BAP1: BRCA1 Associated Protein-1 was retained. Conclusion-Herewith present an uncommon case of highly aggressive tumor clear cell RCC sarcomatoid and rhabdoid differentiation for its clinical, radioimaging, histopathological and immunohistochemitry significance with review.

Keywords

• renal tumors
• renal cell carcinoma
• rhabdoid differentiation
• sarcomatoid differentiation
• paired box 8 (pax8)
• carbonic anhydrase 9 (ca9)