Renal cancers in HLRCC metastasise early and are aggressiverr. Precise risk of renal cancer is unclear and occurs in only 20-35% of HLRCC families. Youngest reported age at diagnosis is age 10 and with metastatic renal cancer is age 18. Average age at diagnosis with renal cancer is mid-40's. Screening in childhood is not recommended since absolute risk is less than 1%. Discuss option of screening from earlier age in certain families e.g. young onset, strong family history of renal cancer.
Annual MRI (or CT with contrast if MRI contraindicated) from age 18. Ultrasound is less sensitive for screening than MRI. HLRCC associated renal cancer can metastasise when very smallr therefore renal ultrasound is considered insufficient imaging. Annual CT may cause excessive radiation exposure. There is no evidence screening detects renal cancer at an early stage or improves survival.
Although most patients are likely to have a solitary renal cancer, total nephrectomy is recommended.r
Uterine leiomyoma (fibroids)
There is no evidence for surveillance.
There is no evidence for risk reducing surgery.
There is no evidence for annual skin review.