Overview

Hereditary papillary renal cell carcinoma (HPRCC) is a hereditary condition that increases the risk of the papillary type of renal cell carcinoma (kidney cancer). There are two types of papillary renal cell tumors: type 1 and type 2. The tumors in HPRCC are type 1 tumors. Individuals with HPRCC have an increased risk of multiple kidney tumors and an increased risk of developing tumors on both kidneys. Currently, no other types of cancer or noncancerous health problems are known to be related to HPRCC.

What causes HPRCC?

HPRCC is a genetic condition. This means that the risk for type 1 papillary renal cell carcinoma can be passed from generation to generation in a family. Mutations in a gene called c-met are linked to HPRCC development. C-met is a gene that encodes for the receptor to hepatocyte growth factor. Research is ongoing to learn more about HPRCC.

How is HPRCC inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. HPRCC follows an autosomal dominant inheritance pattern, in which a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation.

How common is HPRCC?

HPRCC is considered to be rare. The number of people and families who have HPRCC is unknown.

How is HPRCC diagnosed?
HPRCC is suspected when multiple family members have type 1 papillary renal cell carcinoma. Genetic testing to look for mutations in the C-met gene is available for people suspected of having HPRCC.

What are the estimated cancer risks associated with HPRCC?

The specific risk for type 1 papillary renal cell carcinoma in families with HPRCC is unknown. Studies of families with HPRCC show that not everyone who has inherited the condition will develop kidney cancer.

What are the screening options for HPRCC?

There are no specific screening guidelines for families suspected of having HPRCC. Individuals in these families are encouraged to talk with their doctor about screening options for kidney cancer, including ultrasound (which uses sound waves to create a picture of the internal organs), computed tomography (CT or CAT) scan (which creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors), and magnetic resonance imaging (MRI) (which uses magnetic fields, not x-rays, to produce detailed images of the body). Some doctors suggest that individuals who have HPRCC, or a family history that suggests HPRCC, should have yearly screening beginning at age 30.