[Association of pheochromocytoma and phakomatosis. Apropos of a clinical case].

Of the multiple endocrine syndromes, the association between pheocromochytoma, medullary thyroid carcinoma (MTC) and parathyroid-hyperplasia (Sipple's syndrome, MEN IIa), and the association of MTC, with multiple monocutaneous neuromas and with a characteristic facial appearance (MEN IIb syndrome) are well known. Furthermore in about 10% of the patients affected by Von Recklinghausen's disease (neurofibromatosis) a pheocromochytoma is present. A new classification of this important (even though rare) chapter of endocrine pathology was developed by Pears in 1968 when he hypothesized a common neuroectodermic origin for groups of cells diffused throughout the body and nested in different organs. The association of such different pathological conditions could be justified by a faulty development of these primitive neuroectodermic cells. This study aim to analyse a recently observed case of pheocromochytoma associated with Von Recklinghausen's disease. The Pathogenetic hypotheses of this rare pathological association, the diagnostic methods and the therapeutic procedures will be review.