Renal cancer
Surgical
Individuals with HPRCC develop bilateral multifocal renal adenomas and carcinomas.rManagement of renal tumours depends on the lesion size, location, growth rate, number of tumours and the patient’s comorbidities. In general, surgical resection is recommended once a solid lesion exceeds the 3 cm threshold.r Ablation methods, such as radiofrequency ablation (RFA) or cryoablation therapy, are not recommended in patients who are good surgical candidates as previous ablation may complicate subsequent surgical procedures and can also make post-ablation imaging difficult to interpret.r
Surveillance
Given the rarity of HPRCC, there is limited evidence around the timing and type of surveillance. Screening for type 1 papillary renal cell carcinoma can be difficult as tumours are generally hypovascular, can be mistaken for cysts and cause equivocal contrast enhancement.r To reduce cumulative radiation exposure, MRI or high-resolution renal ultrasound are preferred over CT with contrast. The median age of type 1 papillary renal cell carcinoma diagnosis in HPRCC families is 41 years.r However data is limited by small sample size. The youngest reported age of onset of renal cancer in patients with HPRCC is 19 years.r