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Multiple endocrine neoplasia type I Clinical genetics and diagnosis

1997; Springer Science+Business Media; Linguagem: Inglês

10.1007/978-1-4615-6355-6_18

2509-8497

Britt Skogseid,

Salivary Gland Tumors Diagnosis and Treatment

Multiple endocrine neoplasia type I (MEN-I) is a well-characterized hereditary syndrome with occurrence of primary hyperparathyroidism (HPT) in combination with pancreatic-duodenal endocrine and anterior pituitary tumors. Other endocrine lesions, such as adrenocortical proliferation; thymic, bronchial, and gastric carcinoids; as well as thyroid adenomas, colloid goiters, differentiated thyroid carcinoma, and lipomas, are over-represented in MEN-I. MEN-I is also referred to as Wermer’s syndrome, because an autosomal-dominant inheritance pattern with high penetrance was first recognized by Wermer in 1954 [1]. Biochemical and genetic screening techniques for MEN-I have been developed, and gene carriers can be identified prior to presentation of an overt disease [2,3]. The pancreatic and duodenal endocrine tumors exhibit a propensity to malignant progression [4,5], while the other two classical MEN-I lesions almost invariably are benign but nevertheless may lead to considerable endocrine morbidity [6–10]. Early diagnosis and timely treatment instituted in order to avoid malignancy and endocrine morbidity comprise an increasing concern in MEN-I.