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.
Links
- American Society of Clinical Oncology HPRCC description
- Familial Renal Cancer: Molecular Genetics and Surgical Management. Barrisford GW, Singer EA, Rosner IL, Linehan WM, Bratslavsky G. International Journal of Surgical Oncology, Vol. 2011. Article ID 658767.
Publication date: September 2012
Review date: September 2014
