Multiple endocrine neoplasia type 1 (MEN-1), or Wer-mer's syndrome, is a rare autosomal dominant hereditary syndrome with an estimated prevalence of 2-10 per 100,000 inhabitants (1). It typically affects at least two of three major locations: the parathyroid glands, with hyperparathyroidism being the most common (90-97 of cases) and usually the first clinical manifestation of MEN-1 (2); the endocrine pancreas: enteropancreatic tumors occur in 30-80 of patients and they can be functional or non-functional (3). Gastrinomas are the most common functional enteropancreatic tumors; the anterior pituitary gland may be affected in 10-60 of cases (4). The majority of pituitary tumors are pro-lactinomas.However, other lower-frequency endocrine and non-endocrine tumors have been described, such as adrenal cortical tumors in 27-36 of patients (5), carcinoid tumors of the bronchi, gastrointestinal tract and thymus, subcutaneous lipomas, facial angiofibromas, collageno-mas, etc.
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