[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.
Giorgetti
P L
PL
Istituto di Chirurgia Generale e Cardiovascolare, Università degli Studi di Milano.
Pizzocari
P
P
De Monti
M
M
Rampoldi
V
V
Rignano
A
A
Vandone
P L
PL
Miani
S
S
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D010673:Pheochromocytoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D014057:Tomography, X-Ray Computed
Associazione di feocromocitoma e facomatosi. A proposito di un caso clinico.