Diagnosis and surgical treatment of multiple endocrine neoplasia type 1 / 中国普通外科杂志

ABSTRACT

Objective To discuss the diagnosis and surgical treatment of multiple endocrine neoplasia 1 (MEN1). Methods The clinical data of MEN1 from 1974 to 2005 were retrospectively analyzed. Results There were 17 cases of MEN1 in our group. Among them, 11 cases were from 4 families. MEN1 gene mutation was detected in 14 cases admitted after 1997. There were 11 cases of pHPT, 6 cases of insulinoma, 8 cases of pituitary adenoma, 6 cases of adrenal adenoma, 2 cases of thymic carcinoid and 2 cases of collagenoma. Two patients had respectively 4 glands involved, 3 patients had 3, 7 patients had 2 kinds of gland involved respectively. Four patients had only one kind of gland involved. A 12 year old girl had no clinical symptom and biochemical change. Six patients presented with nephrolithasis, 6 patients had impaired pancreatic endocrine function. Subtotal parathyroidectomy was performed in 4 patients of pHPT, and one patient received parathyroid adenoma enucleation. Insulinomas were enucleated in 4 patients. Two patients underwent thymus tumor extirpation. Conclusions MEN1 varies in symptoms, even those from the same family. Cermline mutation test helps establish diagnosis. Operations should be aiming at tumor resection as well as the improvement of life quality.