Chromophobe renal cell carcinoma with liposarcomatous dedifferentiation - report of a unique case.

[pleomorphic liposarcoma]

Sarcomatous transformation of chromophobe renal cell carcinoma (CRCC ) is a well recognized phenomenon . Of the published cases with sarcomatous transformation of CRCC , none have shown liposarcomatous differentiation . Out of a cohort of 250 cases of CRCC , 19 (7 .6 %) showed sarcomatous differentiation . In one case (female , age 46 years ), the sarcomatous component of the tumor displayed histological features of a pleomorphic liposar-coma . Light microscopic examination revealed a biphasic pattern with a chromophobe renal cell carcinoma (CRCC ) and a high -grade sarcomatous component containing large pleomorphic lipoblasts . In several areas both components were intermingled . The conventional CRCC component showed classic histological features with calcifications , medium-sized polygonal cells arranged in solid-alveolar structures with raisinoid nuclei , pale-eosinophilic flocculent cytoplasm with perinuclear haloes . In addition , a microcystic-adenomatous component had luminal spaces filled with erythrocytes . The CRCC was positive with Hale 's colloidal iron -stain whereas the sarcomatous component was negative . The CRCC component was diffusely positive for cytokeratin 7 , parvalbumin and racemase but negative for cy -tokeratin 20 , vimentin , CD 10 , carboanhydrase IX and S 100 -protein . The pleomorphic liposarcomatous component displayed immunereactivity for CD 10 , vimentin , racemase and focally for carboanhydrase IX . The proliferative activity (Mib-1 /Ki-67 ) was 5 % in the CRCC and 30 % in the pleomorphic liposarcomatous component . No immunereactivity for MDM 2 or CDK 4 was detected .This is the first reported case of a sarcomatoid CRCC where the sarcomatous component displayed features of a pleomorphic liposarcoma . The patient died from widespread metastatic disease 12 months after nephrectomy .

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pleomorphic liposarcoma

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