The majority of uveal melanoma diagnoses occur in adulthood, with 75% diagnosed over age 44 years.r Median age at diagnosis is 51 years. Whilst cases as young as age 11 years are reported in the literature, absolute risks under age 18 years are small. BAP-1 related uveal melanoma may be a more aggressive phenotype.r
Renal cell carcinoma
The pathology is usually clear cell in type,r and median age at diagnosis is 50 years, with 75% of diagnoses reported over age 40 years.r Annual renal ultrasound (ideally high resolution) is advised and consider alternating with abdominal MRI every two years. Reporting of haematuria is advised, though there is no evidence for performing routine urinalysis.
The median age at diagnosis of mesothelioma is 55 years, with 75% of diagnoses over age 46-48 years.r Annual clinical examination by a health professional is advised. There is no evidence for surveillance imaging to detect early tumours, though if abdominal imaging for renal cancer is being performed, then assessment for mesothelioma could be considered. Alternatives to MRI include use of low dose CT scan for chest surveillance, though there is no evidence to assess efficacy for mesothelioma surveillance for BAP-1 pathogenic variant carriers.
There is evidence to support an increased risk of malignant mesothelioma in individuals with BAP1-TPDS who have environmental asbestos exposure.r
Cutaneous melanoma and non-melanoma skin cancers
Benign BAP-1-related skin lesions (BIMT) can occur in younger children (age <18 years). Expert dermatologist review before biopsy is advised. Only cutaneous lesions suspected to be malignant require biopsy. The median age at diagnosis for cutaneous melanoma is 39 years, with 75% of diagnoses occurring after age 29 years.r Annual full-body dermatologic examinations are advised beginning around age 18-20 years. Non-melanoma skin cancer has a median age at diagnosis of 44 years, with 75% of diagnoses over age 39 years.