Overview
CANCER
GENETIC CANCER RISK
Renal
High Risk
Other
High Risk
Skin
Elevated Risk
CANCER TYPE
AGE RANGE
CANCER RISK
RISK FOR GENERAL POPULATION
Renal
To age 80
16-21%, with a strong tendency towards young ages of diagnosis, median age of diagnosis is estimated to be 44
1.4%
Uterine Leiomyosarcoma
To age 80
Possibly elevated risk
<0.1%
Skin Leiomyosarcoma
To age 80
Possibly elevated risk
<0.1%
Other - Non-malignant features of HLRCC (women only)
All ages
Most women with HLRCC will develop multiple uterine fibroids at young ages
NA
Other - Non-malignant features of HLRCC
All ages
Individuals with HLRCC are likely to develop skin leiomyomas
NA
CANCER TYPE
PROCEDURE
AGE TO BEGIN
FREQUENCY
Renal
Abdominal imaging with MRI (preferred) or CT, with and without contrast
8 to 10 years
Annually
Since mutation carriers are at an increased risk for more aggressive kidney cancer, this information may be considered when choosing management options for individuals with any suspicious renal lesion or a diagnosis of renal cancer
NA
NA
Uterine Leiomyosarcoma
Gynecologic exam, with ultrasound and other imaging as needed to evaluate for severity of uterine fibroids and to monitor for changes suggestive of sarcoma
Age 20, or younger if symptoms are present
Annually
Skin Leiomyosarcoma
Monitoring of leiomyomas for changes suggestive of sarcoma
At time of diagnosis of HLRCC
Annually
Other - Non-malignant features of HLRCC
Monitoring and treatment for uterine fibroids
At time of diagnosis of HLRCC
Annually
Monitoring and treatment for skin leiomyomas as necessary
At time of diagnosis of HLRCC
Annually
For Patients With A Cancer Diagnosis
For patients with a gene mutation and a diagnosis of cancer, targeted therapies may be available as a treatment option for certain tumor types (inhibitors of VEGF and EGFR)
NA
NA