[Clinical and therapeutic features of MEN II]

Multiple Endocrine Neoplasia type 2 is a genetically transmitted poliendocrinopathy, in which pheochromocytoma (PCC), medullary thyroid carcinoma (MTC) and parathyroid disease may occur and coexist with different rates. Although the clinical behaviour of each component is well known and usually predictable, controversies still exist on the most appropriate therapeutical strategy when patients exhibit synchronous lesions; many elements may influence this choice: the evidence of bilateral (PCC) or multicentric (MTC) lesions, the evaluation of their malignancy, the adoption of therapies other than surgery (alpha receptor blockers in PCC). In this paper the Authors report their experience in diagnosing and treating MEN 2; it is based on 5 patients operated from 1972 to 1993 and on the screening of their relatives for thyroid and/or adrenal lesions. The individual manifestations of the disease, with regard to time and extent of organ involvement, are considerably variable; however, an aggressive approach of MTC, even in presence of a synchronous PCC, as well as a unilateral adrenalectomy (with a close follow up of the patient) when surgical exploration does not show an involvement of the remaining gland, is suggested.