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Renal Cell Carcinoma, Papillary, 1; RCCP1

Renal Cell Carcinoma, Papillary, 1; RCCP1

OMIM number: 605074

Comments closing date: 04/05/2020

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Renal cell carcinoma papillary (RCCP): is an uncommon histological type of renal cell carcinoma accounting for approximately 10 to 15% of all renal cell tumours. It is a subtype of renal cell carcinoma tending to show a tubulo-papillary architecture formed by numerous, irregular, finger-like projections of connective tissue. Renal cell carcinoma is a heterogeneous group of sporadic or hereditary carcinoma derived from cells of the proximal renal tubular epithelium. RCCP has been found to be caused by mutations in the MET gene. It is inherited in an autosomal dominant fashion. Multiple tumours of varying size can occur in both kidneys of affected family members. The MET gene is a type of proto-oncogene and a type of receptor tyrosine kinase gene.

Review date: 6 April 2022