Hereditary Papillary Renal Cell Carcinoma (HPRCC)

HPRCC is an autosomal dominant disorder.

Signs and symptoms:

  • Increased risk of Type 1 papillary renal cell tumours. These tumours may grow slowly, can be bilateral and multifocal; however they are also malignant and can metastasise. HPRCC are typically small and can be mistaken for kidney cysts; it is important your doctor is knowledgeable about their appearance.
  • Primary tumours associated with HPRCC do not arise outside the kidneys.

Diagnosis

  • An accurate diagnosis is available by genetic testing, as the MET gene has been associated with HPRCC.

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Publication date: September 2012
Review date: September 2015