Adrenal pheochromocytoma and jejunal neurofibroma in type 1 neurofibromatosis: report of a case
De Toma, G.; Letizia, C.; Cavallaro, G.; Polistena, A.; Cotesta, D.; Petramala, L.; Porciello, R.; Giustini, S.; Calvieri, S.
Il Giornale di Chirurgia 28(5): 199-202
2007
ISSN/ISBN: 0391-9005 PMID: 17547785 Document Number: 613968
Neurofibromatosis type 1 (NF1), known as von Recklinghausen's disease, is characterized by presence of café au lait spots, and neurofibromas in the skin or along the course of peripheral nerves. Diagnosis, despite extreme clinical variability, is defined by established diagnostic criteria. Clinical status is frequently complicated by systemic disorders and neoplasias. A case of a patient affected by NF1, with hypertension due to adrenal pheochromocytoma and with jejunal neurofibroma, is reported. Variability in clinical presentation of NF1 with possible manifestation of severe systemic benign and malignant diseases requires strict follow-up and specific screening of extra-cutaneous lesions.