NCI Definition: Also known as chromophil carcinoma, it represents a minority of renal cell carcinomas. It can be hereditary or sporadic. The sporadic papillary renal cell carcinoma is characterized by trisomy of chromosomes 7, 16, and 17, and loss of chromosome Y. The peak incidence is in the sixth and seven decades. It is classified as type 1 or 2, based on the cytoplasmic volume and the thickness of the lining neoplastic cells. The prognosis is more favorable than for conventional (clear cell) renal cell carcinoma. -- 2003 [1]

Papillary renal cell carcinomas most frequently harbor alterations in MET, CDKN2A, SETD2, NF2, and KDM6A [2].

Most Commonly Altered Genes in Papillary Renal Cell Carcinoma

MET Mutation, CDKN2A Loss, SETD2 Mutation, CDKN2B Loss, and EP300 Mutation are the most common alterations in papillary renal cell carcinoma [2].

Top Alterations in Papillary Renal Cell Carcinoma

Significant Genes in Papillary Renal Cell Carcinoma

FH +

IDH1 +

IDH2 +








TFE3 +

TSC1 +

Disease Details

Chromophil Carcinoma of the Kidney, Kidney renal papillary carcinoma, Papillary (Chromophil) Renal Cell Carcinoma, Chromophil Carcinoma of Kidney, Chromophil Renal Cell Carcinoma
Papillary Adenocarcinoma
Hereditary Papillary Renal Cell Carcinoma
OncoTree Name
Papillary Renal Cell Carcinoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.