MULTIPLE ENDOKRINE NEOPLASIE TYP II. DIAGNOSTIK, THERAPIE UND PROGNOSE

From 1979 to 1986, seven patients with multiple endocrine neoplasia (MEN) type IIa and three with type IIb were treated. Nine had a C-cell carcinoma, one had C-cell hyperplasia. None had hyperparathyroidism. Three patients had multiple mucous neuromas. Six patients had proven pheochromocytoma: adrenalectomy was performed in these six (unilateral or bilateral depending on whether the tumor was uni- or bilateral). All ten patients had a total thyroidectomy - three later required neck dissection for regional lymphnode metastases. One patient died from the consequences of diffuse liver metastases of a C-cell carcinoma. Extensive family screening is necessary with patients who have MEN type II, in order to discover early any occult disease carriers. In addition, MEN type II should be excluded in all patients who have C-cell carcinoma, pheochromocytoma or hyperparathyroidism.