Table 1. Diagnostic criteria for BHD.

	Diagnostic Criteria for BHD
Major criteria	At least five fibrofolliculomas or trichodiscomas, at least one histologically confirmed, of adult onset Pathogenic FLCN germline mutation
Minor criteria	Multiple lung cysts: bilateral basally located lung cysts with no other apparent cause, with or without spontaneous primary pneumothorax Renal cancer: early onset (<50 years) or multifocal or bilateral renal cancer, or renal cancer of mixed chromophobe and oncocytic histology A first-degree relative with BHD

Patients should fulfill one major or two minor criteria for diagnosis. Adapted from Menko et al. [38], Lancet Oncology 2009