Clear Cell Papillary Renal Cell Carcinoma and Renal Angiomyoadenomatous Tumor

Autor: Gabriella Nesi, Guido Sauter, Andreas G. Nerlich, Hans-Ulrich Schildhaus, Martina Storz, Nikolaus GaBler, Peter Schraml, Adriana von Teichman, Rainer Grobholz, Falko Fend, Karl-Friedrich Deml, Helmut E. Gabbert, Eva Comperat, Joseph V. Bonventre, Benoit Lhermitte, Seife Hailemariam, Ruth Knüchel, Thomas Rüdiger, Barbara Fleige, Holger Moch, Raoul Hinze
Rok vydání: 2015
Předmět:
Zdroj: American Journal Surgical Pathology
ISSN: 0147-5185
DOI: 10.1097/pas.0000000000000456
Popis: Clear cell papillary renal cell carcinoma (ccpRCC) and renal angiomyoadenomatous tumor (RAT) share morphologic similarities with clear cell (ccRCC) and papillary renal cell carcinoma (pRCC). It is a matter of controversy whether their morphologic, immunophenotypic and molecular features allow the definition of a separate renal carcinoma entity. The aim of our project was to investigate specific renal immunohistochemical biomarkers involved in the hypoxia-inducible factor pathway and mutations in the VHL gene to clarify the relationship between ccpRCC and RAT. We investigated 28 ccpRCC and 9 RAT samples by immunohistochemistry using 25 markers. VHL gene mutations and allele losses were investigated by Sanger sequencing and fluorescence in situ hybridization (FISH). Clinical follow-up data were obtained for a subset of the patients. No tumor recurrence or tumor-related death was observed in any of the patients. Immunohistochemistry and molecular analyses led to the reclassification of three tumors as ccRCC and TFE3 translocation carcinomas. The immunohistochemical profile of ccpRCC and RAT samples was very similar but not identical, differing from both ccRCC and pRCC. Especially, the parafibromin and hKIM-1 expression exhibited differences in ccpRCC/RAT compared with ccRCC and pRCC. Genetic analysis revealed VHL mutations in 2/27 (7%) and 1/7 (14%) ccpRCC and RAT samples, respectively. FISH analysis disclosed a 3p loss in 2/20 (10 %) ccpRCC samples. ccpRCC and RAT have a specific morphologic and immunohistochemical profile but they share similarities with the more aggressive renal tumors. Based on our results, we regard ccpRCC/RAT as a distinct entity of renal cell carcinomas.
Databáze: OpenAIRE