<総説>多内分泌腺腫瘍症第1型

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  • <Reviews>Multiple Endocrine Neoplasia (MEN) Type I

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In the types of multiple endocrine neoplasia (MEN), MEN type I means involvements of the hypophysis, the parathyroid gland and/or the islet of pancreas. Thirty-six cases of MEN type I reported in the Japanese literature including our own one were reviewed. Although MEN is a genetic disease with a dominant autosomal inheritance, ten sporadic cases were noted. Usually both sexes are affected with approximately the same frequency, but male-to-female ratio was 2 : 3 in our survey. Males in the third and fourth decades and females in the third to the fifth decades were prevalent. The lesions of the parathyroid gland were the most common, and the pituitary lesions were usually benign. The pancreatic lesions are important because its clinical manifestations and relatively high frequency of the malignancy decide the prognosis. Surgical intervention to the pancreatic lesions should be taken into consideration with the characteristics of multiple adenomas, malignancy, microadenomatosis, etc. In the nonfunctioning benign islet-cell tumor(s), if multiple, enucleation may be an effective procedure in order to avoid the destruction of pancreatic exo-and endocrine functions. Careful long-term follow-up is mandatory in the patients suffering from MEN.

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