Pheochromocytoma and Neurofibromatosis Type 1: An Exceptional Association

Abstract

The association of a pheochromocytoma to a neurofibromatosis type 1 is a rare association. We relate the observation of a 62 years female patient who had, sinced a young age, showed cafe au lait spots and neurofibromas on the entire body, and been monitored for the last 4 years for high blood pressure with headaches, excessive sweating and palpitations. Dosage of urine catecholamine metabolites has revealed a rate elevated to 20 times the normal. Adrenal tomodensitometry has revealed a voluminous heterogeneous mass occupying the right adrenal loge, presenting a narrow contact with the inferior vena cava, and with the upper pole of the right kidney, measuring 10.5 x 9.3 x 8.7 cm, along with a small heterogeneous mass of the left adrenal. Under the scope of the neurofibromatosis, fundus photography has been performed and showed Lisch nodules, while cerebral tomodensitometry results were normal. After undergoing bilateral andrenalectomy, anatomopathological examination has revealed a completely excised pheochromocytoma on the right adrenal, and a macro-nodular adrenal hyperplasia on the left adrenal. Metaiodobenzylguanidine scan has not showed any ectopic placement of the pheochromocytoma, and urinary methoxy derivatives were normal post-surgery. Screening and adequate surgical support of the pheochromocytoma, for patients with neurofibromatosis type 1 presenting high blood pressure, allows to avoid complications.