A case of neurofibromatosis type 1 with an aldosterone-producing adenoma of the adrenal.

Abstract. Biagi P Alessandri M, Campanella G,Castro R, Totteri A, Fanciullacci M (Abbadia SanSalvatore Hospital, Abbadia San Salvatore; Miser-icordia Hospital, Grosseto; University of Florence,Florence, Italy). A case of neurofibromatosis type 1with an aldosterone-producing adenoma of theadrenal (Case Report). J Intern Med 1999; 246:509–512.Neurofibromatosis type 1 is a phacomatosis. Neuro-fibromas are the most common tumours associatedwith the disease, and along with other tumours,make neurofibromatosis type 1 the most commontumour predisposing syndrome in humans. Hyper-tension may be coincidental, but at least two specificneurofibromatosis related causes must be consid-ered, namely neurofibromatous involvement of therenal artery and pheochromocytoma. We havedescribed the first known case of a patient withneurofibromatosis type 1 who developed hyperten-sion due to an aldosterone-producing adenoma ofthe adrenal. The question of whether this associa-tion was coincidental or due to the tumourpredisposition of neurofibromatosis type 1 wasdebated.Keywords: Conn’s syndrome, hypertension, neurofi-bromatosis type 1, tumour predisposition.