Advanced papillary renal cell carcinoma: Epidemiology, genomic drivers, current therapies, and ongoing trials

Patrick L Sweeney; Albert Jang; Shams K Halat; Sumanta K Pal; Pedro C Barata

doi:10.1016/j.ctarc.2022.100639

Advanced papillary renal cell carcinoma: Epidemiology, genomic drivers, current therapies, and ongoing trials

Cancer Treat Res Commun. 2022:33:100639. doi: 10.1016/j.ctarc.2022.100639. Epub 2022 Sep 22.

Authors

Patrick L Sweeney¹, Albert Jang¹, Shams K Halat², Sumanta K Pal³, Pedro C Barata⁴

Affiliations

¹ Deming Department of Medicine, Tulane University School of Medicine, New Orleans, LA, USA.
² Department of Pathology and Laboratory Medicine, Tulane University School of Medicine, New Orleans, LA, USA.
³ City of Hope Comprehensive Cancer Center, Duarte, CA, USA.
⁴ Deming Department of Medicine, Tulane University School of Medicine, New Orleans, LA, USA; Tulane Cancer Center, New Orleans, LA, USA; University Hospitals Seidman Cancer Center, Case Comprehensive Cancer Center, Cleveland, Ohio, USA. Electronic address: pedro.barata@UHhospitals.org.

PMID: 36162322
DOI: 10.1016/j.ctarc.2022.100639

Abstract

Papillary renal cell carcinoma (PRCC) is the most common subtype of non-clear cell renal cell carcinoma. PRCC can be subdivided into types 1 and 2 based on histology, each associated with different genetic mutations. The MET gene is commonly altered in type 1 PRCC while multiple alterations are involved in type 2 PRCC. PRCC is an aggressive cancer with a predominance in male and black patients and poor prognosis. Due to its rarity, there was a lack of convincing prospective data to guide treatment; hence, therapies were previously extrapolated from clear cell renal cell carcinoma with mixed results. More recently, some phase 2 trials focused on PRCC have been promising. Tyrosine kinase inhibitor (TKI) monotherapy is considered the standard of care, and combination strategies with TKIs and immune checkpoint inhibitors are emerging. Genetic profiling and large-scale clinical trials are needed to inform targeted treatment of PRCC.

Keywords: Cabozantinib; Immune checkpoint inhibitor; Nivolumab; Non-clear cell renal cell carcinoma; Papillary renal cell carcinoma; Sunitinib; Tyrosine kinase inhibitor.

MeSH terms

Carcinoma, Renal Cell* / drug therapy
Carcinoma, Renal Cell* / epidemiology
Carcinoma, Renal Cell* / genetics
Genomics
Humans
Kidney Neoplasms* / drug therapy
Kidney Neoplasms* / epidemiology
Kidney Neoplasms* / genetics
Male
Mutation
Prospective Studies