Table 3 Consensus recommendations on surveillance in BHD syndrome.
Recommendations | Strength | |
|---|---|---|
Rec. 13 | Surveillance for renal cell carcinoma should be lifelong. | strong |
Rec. 13a | Surveillance for renal cell carcinoma should be started at age 20. | strong |
Rec. 13b | Surveillance for renal cell carcinoma should be conducted every 1–2 years. | strong |
Rec. 14 | Surveillance for renal cell carcinoma should preferably be conducted using MRI, but ultrasound can be used if MRI is not available/appropriate. | strong |
Rec. 14a | MRI with IV contrast should be used unless there are contraindications for contrast use. | strong |
Rec. 15 | Following the detection of a renal tumour, the frequency of imaging follow-up should increase in order to monitor growth rate and plan intervention. | strong |
Rec. 16 | Surveillance for colon polyps and/or cancers should follow local standard population or family history-based screening guidelines. | moderate |
Rec. 17 | Surveillance for thyroid cancers, salivary cancers and melanomas should not be performed as part of the routine follow-up of patients with BHD syndrome, but should be based on family history. | strong |
