CANCER TYPE
AGE RANGE
CANCER RISK
RISK FOR GENERAL POPULATION
Uveal Melanoma
Percentage of mutation carriers affected
25%-36%
<0.1% to age 80
Malignant Mesothelioma
Percentage of mutation carriers affected
20%-25%
<0.1% to age 80
Cutaneous Melanoma
Percentage of mutation carriers affected
13%-17%
1.6% to age 80
Renal
Percentage of mutation carriers affected
5%-10%
1.4% to age 80
Basal Cell Skin Cancer
Percentage of mutation carriers affected
6%-10%
20% to age 70
Other - Non-malignant features of BAP1-TPDS
All ages
BAP1-TPDS is associated with benign skin tumors known as BIMTs (see Overview)
NA
BAP1
Associated Syndrome
BAP1-Tumor Predisposition Syndrome (BAP1-TPDS).
Core Cancer Risk(s)
Renal and Skin Cancer
Inheritance
Autosomal Dominant
Prevalence
Unknown
CANCER TYPE
PROCEDURE
AGE TO BEGIN
FREQUENCY
Uveal Melanoma
Currently there are no specific medical management guidelines for uveal melanoma risk in mutation carriers. However, expert groups have suggested consideration of screening including eye examinations and imaging by an ocular oncologist. It may also be desirable to avoid risk factors for uveal melanoma, such as arc welding and exposure to sunlight without UV protective sunglasses
11 years
Annually
Malignant Mesothelioma
Currently there are no specific medical management guidelines for malignant mesothelioma risk in mutation carriers. However, expert groups have suggested consideration of individualized monitoring for symptoms of malignant mesothelioma, and/or Consideration of abdominal MRI or CT imaging
30 years
NA
Cutaneous Melanoma
Currently there are no specific medical management recommendations for skin 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
18 years
Annually
Renal
Abdominal MRI (preferred) or CT, with and without IV contrast
30 years, or 10 years younger than the earliest renal cancer diagnosis in the family
Every 2 years
Basal Cell Skin Cancer
Currently there are no specific medical management recommendations for basal cell skin cancer 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
18 years
Annually
Other - Non-malignant features of BAP1-TPDS
Currently there are no specific medical management guidelines for BIMTs in mutation carriers. However, expert groups have suggested it may be appropriate for these tumors to be monitored by a dermatologist along with monitoring for melanoma and basal cell cancer.
18 years
Annually