PMID: 8949733Sep 21, 1996Paper

Characteristic pathological associations in multiple endocrine neoplasia type 1

La Presse médicale
J P ChigotP Benlian

Abstract

Multiple endocrine neoplasia type 1 (MEN 1) is an inherited disorder characterised by slow progressing tumors of the parathyroids, of the endocrine pancreas and of the anterior pituitary. A genetic locus predisposing to this disease has been localised on chromosome 11. Predictive diagnosis of carriers of the defective gene is possible in families using genetic markers at this locus. However, this analysis presupposes a precise identification of affected subjects. Moreover, expression of the disease may vary from one family to the other. The aim of the present study was to define the typical clinical features of the syndrom. We assessed retrospectively 26 cases of MEN 1 identified during 20 years in the same medico-surgical center. Among 11 men and 15 women, all those who had a genealogical investigation had a positive family history of MEN 1. Bifocal and trifocal tumors were the main patterns of associations, and were diagnosed at a mean age of 48.6 years. Parathyroid involvement was most frequent and earliest (96% of cases). The second most frequent was pancreatic involvement (69.2% of cases) predominantly manifesting with gastrinomas (N = 13). Multifocal tumors were usually diagnosed before or within 5 years following diagnos...Continue Reading

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