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, and can metastasise. Cysts may be present, but are not common.
- Primary tumours associated with HPRCC do not arise outside the kidneys.
Diagnosis
- An accurate diagnosis is available by genetic testing, as an associated gene for HPRCC has been identified.
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