There are several ways in which to manage the BAP1-TPDS:
Treatment of manifestations: For the most part, treatments for BAP1-TPDS tumors are the same as those used in standard practice but this may vary depending on the country’s treatment guidelines/recommendations, or even from center to center. However, because of the increased aggressiveness of uveal melanoma caused by BAP1 mutations, all uveal melanomas in patients with the BAP1-TPDS should be managed as the more aggressive class 2 or monosomy 3 tumors. It has been reported that mesothelioma in patients with the BAP1-TPDS tend to be less aggressive and respond better to treatment. We do not know if other tumors associated with BAP1 also exhibit a more, or less, aggressive disease course.
Prevention: For eye melanoma, no data exist regarding the benefit of sunglasses to reduce risk. For mesothelioma, people should avoid asbestos exposure and smoking. For skin cancer, limit sun exposure, use sunscreen and protective clothing, and have regular dermatologic examinations.
Surveillance recommendations:
- Uveal melanoma: yearly dilated eye examinations and imaging by an ocular (eye) oncologist beginning around age 11 years.
- Mesothelioma: no screening methods exist; however, an annual physical examination is recommended. If an abdominal MRI is to be performed as recommended for kidney cancer, consider evaluation of the peritoneum and pleura as well. Although some physicians recommend spiral chest CT, others do not, given the possible increased risk of cancer from CT radiation exposure.
- Skin cancer and BAP1-inactivated melanocytic tumors: annual full body dermatologic examinations beginning around age 20 years.
- Kidney cancer: annual abdominal ultrasound examination; consider annual urinalysis and abdominal MRI every two years.
