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CANCER

GENETIC CANCER RISK

Other

High Risk

Renal

High Risk

Skin

High Risk

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CANCER TYPE

PROCEDURE

AGE TO BEGIN

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%

0.03% 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%

1.3% 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

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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

Consider abdominal MRI (preferred) or CT, with and without IV contrast

30 years

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