Particularités pronostiques, histopathologiques, génétiques et thérapeutiques du carcinome à cellules rénales tubulopapillaire

Abstract

We reviewed papillary renal cell carcinoma (PRCC) epidemiology, radiological and clinical presentations, and specific features of morphological subtypes focusing on genetic defects, risk of local and metastatic recurrence and frequency of multifocality. The MEDLINE database of the US National Library of Medicine was searched for pertinent studies. According to multivariate analyses, PRCC histology was not retained as a prognostic factor. Reported rates of multifocality in PRCC are 22 to 41% but distinction between histological subtype or hereditary forms are barely detailed. Multifocality frequency is independent of size, stage or grade and is not associated with ipsilateral or controlateral recurrence or death from RCC. Thus, PRCC multifocality is not an argument against nephron-sparing surgery. Antiangionenic therapies are being evaluated for the Metastatic PRCC. According to the literature, specific prognostic features of PRCC remain controversial due to the lack of distinction between different PRCC subtypes. Genomic and cytogenetic characterizations have been used to establish an evolving classification of PRCC subtypes and may be a source of new markers that will eventually enable us to precise prognosis and identify targets for new adjuvant therapies.