Pathology, Therapy and Prognosis of Papillary Renal Carcinoma

Abstract

Papillary renal cell carcinoma (pRCC) accounts for approximately 10% of renal parenchymal tumors. There are two pRCC subtypes reported in several studies, but at present, there is limited molecular evidence to validate this pRCC subtyping in the daily routine. The utility of subtyping pRCC is based on reports describing that pRCC subtype is an independent predictor of outcome, with type 1 tumors showing significantly better survival than type 2 tumors. In this article, we summarize the relevant knowledge on pRCC regarding tumor features: clinical presentation, histopathology, electron microscopy, immunohistochemistry, cytogenetics, genetic/molecular and prognosis. We present an overview of the currently available pRCC treatment options and some of the new promising agents.

Renal cell carcinoma: Review of etiology, pathophysiology and risk factors

“…24,25 ). Two familial syndromes are associated with increased risk of papillary-type RCC: hereditary papillary RCC is an autosomal dominant syndrome characterized by multifocal, bilateral, type 1 -RCC caused by mutation of the MET gene on 7q31.…”

Renal cell carcinoma: the search for a reliable biomarker

“…chRCC and oncocytomas can both express CK7, but in different staining patterns. In chRCC it is strongly and diffusely positive, while in oncocytoma it only shows focal positivity or no staining at all (44,46,48). In addition, another stain that can be used to differentiate between chRCC and oncocytoma is CD117 (also known as c-kit), which is a stem cell factor receptor.…”

“…Papillary type I is associated with MET alterations, and cytogenetics shows frequent gains in chromosome 7p and 17p, loss of chromosome Y, and variable gains in chromosomes 3q, 8p, 12q, 16q and 20q (48,59). Papillary type II is more heterogeneous and may be associated with silencing of CDKN2A, mutations in SETD2 and NRF2-ARE pathways, TFE3 fusions, as well as some CpG island methylator phenotypes.…”

“…Papillary type II is more heterogeneous and may be associated with silencing of CDKN2A, mutations in SETD2 and NRF2-ARE pathways, TFE3 fusions, as well as some CpG island methylator phenotypes. Cytogenetics shows allelic imbalance of one or more chromosomes, including 1p, 3p, 5, 6, 8, 9p, 10, 11, 15, 18 and 22 (48,54,58,60,61,64,65). Patients with hereditary papillary renal cancer (HPRC) develop bilateral multifocal type I tumors, while those with HLRCC, which is caused by a germline mutation of the fumarate hydratase gene, develop papillary type II tumors that characteristically have eosinophilic cytoplasm and large inclusion-like nucleoli (66,67).…”

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“…In addition, it is the most common histological subtype in pediatric RCC and has been reported in 18% of dialysis patients (Morabito et al, 2010). However, diagnosis, treatment, and prognosis of PRCC are now mostly based on histological features, whose subtyping remains unsatisfactory (Fernandes and Lopes, 2015). Recent studies have introduced several novel biomarkers for RCC diagnosis and prognosis, such as urine aquaporin-1 and perilipin-2 (Farber et al, 2017;Cao et al, 2018;Song et al, 2019).…”