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Updated: Jul 7, 2025

The Use of Reverse Phase Protein Arrays RPPA to Explore Protein Expression Variation within Individual Renal Cell Cancers
12:22

The Use of Reverse Phase Protein Arrays RPPA to Explore Protein Expression Variation within Individual Renal Cell Cancers

Published on: January 22, 2013

33.6K

Hereditary papillary renal cell carcinoma

Isa Mulingbayan Jacoba1, Zhichun Lu1

  • 1Boston Medical Center/Boston University Chobanian and Avedisian School of Medicine, Department of Pathology and Laboratory Medicine, United States.

Seminars in Diagnostic Pathology
|December 22, 2023
PubMed

Insights

Hereditary papillary renal cell carcinoma (HPRCC) is linked to specific MET gene mutations. Targeted therapies show promise for treating this kidney cancer subtype.

Area of Science:

  • Oncology
  • Genetics
  • Molecular Biology

Background:

  • Hereditary papillary renal cell carcinoma (HPRCC) is an autosomal dominant syndrome.
  • It presents as bilateral, multifocal, classic papillary renal cell carcinomas.
  • Molecular studies link HPRCC to missense mutations in the MET proto-oncogene's tyrosine kinase domain.

Purpose of the Study:

  • To summarize the molecular basis of HPRCC.
  • To discuss the presence of MET mutations in other renal cell carcinoma variants.
  • To highlight therapeutic strategies for HPRCC.

Main Methods:

  • Review of molecular studies on HPRCC.
  • Analysis of mutation data in sporadic and variant renal cell carcinomas.
  • Evaluation of therapeutic outcomes for kinase inhibitors in HPRCC.

Main Results:

  • Missense mutations in the MET tyrosine kinase domain are specific to HPRCC.
  • MET mutations are also found in sporadic papillary renal cell carcinomas and biphasic squamoid alveolar variant of papillary renal cell carcinoma.
  • Dual MET/VEGFR2 kinase inhibitors and other tyrosine kinase inhibitors demonstrate efficacy in HPRCC systemic therapy.

Conclusions:

  • MET proto-oncogene mutations are key drivers in HPRCC.
  • The presence of MET mutations extends beyond HPRCC to other papillary renal cell carcinoma subtypes.
  • Targeted kinase inhibitors offer a promising therapeutic avenue for HPRCC treatment.
Keywords:
Hereditary papillary renal cell carcinomaMET proto-oncogene

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