Hereditary and sporadic papillary renal carcinomas with c-met mutations share a distinct morphological phenotype.

摘要: Germline mutations of c-met oncogene at 7q31 have been detected in patients with hereditary papillary renal cell carcinoma. In addition, were shown to play a role 13% carcinoma and no family history tumors. The histopathology has not previously described. We analyzed the 103 bilateral archival carcinomas 4 metastases 29 from 6 families germline 5 Twenty-five sporadic tumors prominent architecture without somatic evaluated for comparison. All 75 100% papillary/tubulopapillary showed chromophil basophilic, type 1 histology. Fuhrman nuclear grade 1–2 was seen 23 patients, 3 observed focally 8 patients. Seventeen had multiple adenomas microscopic lesions surrounding parenchyma. Clear cells intracytoplasmic lipid glycogen present 94% small basophilic nuclei, clear areas lacked fine vascular network characteristic conventional (clear) conclude that develop multiple, bilateral, macroscopic lesions. Renal genotype show distinctive phenotype are genetically histologically different other syndromes most architecture. Although all share histology, harbor mutations.