Table 2.

Consensus recommendations relevant to the clinical management of BHD syndrome.

Recommendations		Strength
Rec. 10	FLCN variants should not be considered as ‘pneumothorax-only’ variants.	strong
Rec. 11	All FLCN variants should be considered as significantly increasing renal tumour risk and lead to appropriate renal surveillance being offered.	strong
Rec. 12	Currently there is not sufficient evidence of an increased risk for other tumours observed in families with BHD syndrome (e.g. colorectal cancer, malignant melanoma, thyroid cancer, etc.).	moderate