Autosomal dominant disease that has material basis in a mutation in the MEN1 tumor suppressor gene and is characterized by over active endocrine glands frequently involving tumors of the parathyroid glands, the pituitary gland, and the pancreas.
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The men 1 gene has been mapped by genetic studies to the long arm of human chromosome 11, region q12-13.
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Genetic analysis of families and tumoral deletion mapping made possible to narrow the men 1 region to a 5 cM interval on chromosome 11q12-13.
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No clinical, laboratory, or MEN 1 feature distinguished patients with meningiomas.
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At diagnosis, all patients were checked for the presence of multipleendocrineneoplasiatypeI syndrome.
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Three patients had atypical carcinoids, one with multiple endocrine neoplasia type 1 syndrome.
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Eight patients had sporadic NETs, and 1 had multiple endocrine neoplasia type 1 syndrome.
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Three patients had atypical carcinoids, one with multiple endocrine neoplasia type 1 syndrome.
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Pituitary adenomas represent one of the key features of multiple endocrine neoplasia type 1.
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She was diagnosed as having multiple endocrine neoplasia type 1 with gastrinoma and liver metastases.
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Eight patients had sporadic NETs, and 1 had multiple endocrine neoplasia type 1 syndrome.
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An association between blood type O and neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) patients was recently suggested.
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Conclusions: Unlike some other syndromes where a genetic mutation can predict clinical course, there is no genotype-phenotype correlation in MEN1 syndrome.
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However, the coexistence of MEN1 syndrome and malignant tumor such as low-grade fibromyxoid sarcoma has not been described in the literature.
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The patient with Zollinger-Ellison and MEN1 syndrome has been followed 3 years after diagnosis and 2 years after total gastrectomy.
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We describe a young woman who fulfilled the clinical and biochemical criteria for MEN1 syndrome, but DNA sequencing did not indicate any MEN1 mutations.