

CANCER
GENETIC CANCER RISK
Renal
High Risk
Skin
Elevated Risk
Other - Non-malignant features of BHDS
High Risk

CANCER TYPE
AGE RANGE
CANCER RISK
RISK FOR GENERAL POPULATION
Renal
To age 80
10%-37%
1.4%
Melanoma
To age 80
Elevated risk for skin melanoma
1.6%
Other - Non-malignant features of BHDS
All ages
BHDS is associated with a high risk for lung cysts and life-threatening spontaneous pneumothorax (lung collapse), as well as benign skin tumors.
N/A
FLCN
Associated Syndrome
Birt-Hogg-Dube Syndrome (BHDS).
Core Cancer Risk(s)
Renal Cancer and Melanoma
Inheritance
Autosomal Dominant
Prevalence
Unknown

CANCER TYPE
PROCEDURE
AGE TO BEGIN
FREQUENCY
Renal
Abdominal imaging with MRI (preferred) or CT, with and without contrast
Age 20, or earlier if there is a family history of renal tumor before age 30 years
Every 1 to 3 years
Melanoma
Currently there are no specific medical management recommendations for melanoma risk in mutation carriers. However, the increased risk for melanoma warrants consideration of risk-reduction strategies, including frequent self-examination of the skin, consideration of clinical skin examinations, and minimizing exposure to the sun and other sources of UV radiation.
Individualized
N/A
Other - Non-malignant features of BHDS
Evaluation for lung cysts and attention to the risk for pneumothorax, as well as dermatological consultation
At diagnosis
Individualized