We report nine patients (seven males and two females, median age 64 years (range 51-79 years)) with a renal cell carcinoma, each of which contained a significant component of neoplastic epithelial cells with a striking microvacuolated (hibernoma-like) cytoplasmic appearance. Tumor sizes ranged from 1.5 to 8.0 cm (mean 4.2 cm, median 4.3 cm).
The basic architecture of the tumors was solid-alveolar in two cases (classified as renal cell carcinoma-not otherwise specified (NOS)) and papillary in seven cases (classified as papillary renal cell carcinoma NOS). The nuclear grade according to the Fuhrman grading system was three in all cases.
By immunohistochemistry, the cells with microvacuolated cytopasm and significantly expressed adipophilin and anti-mitochondrial antigen in a similar cytoplasmic pattern. On ultrastructural examination, the cytoplasm of the neoplastic epithelial cells was packed with distended mitochondria, most of which displayed lamellated cristae.
Numerous microvesicles were dispersed between the mitochondria. No mutations in the succinate dehydrogenase B gene were identified.
Based on our findings, we propose that the mechanism behind this phenomenon is an abnormal intracellular processing of lipids. No aggressive behavior was observed in six out of nine patients with available follow-up information.