Duodenopancreatic Resections in Patients With Multiple Endocrine Neoplasia Type 1

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Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.

OBJECTIVE To evaluate the outcome of an aggressive surgical approach for duodenopancreatic neuroendocrine tumors (PETs) associated with multiple endocrine neoplasia type 1 (MEN1). SUMMARY BACKGROUND DATA The management of PETs is still controversial in the setting of the autosomal dominant inherited MEN1 syndrome. METHODS MEN1 patients that had either biochemical evidence of functioning PET...

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Menin mutations in patients with multiple endocrine neoplasia type 1.

Multiple endocrine neoplasia type 1 (MEN-1) is a familial cancer syndrome with parathyroid, pituitary and enteropancreatic tumors. The disease phenotype segregates with markers on chromosome 11q13. Very recently a new gene was cloned from this region and was found to carry mutations in 14 of 15 unrelated MEN-1 patients. The gene was termed menin and is predicted to code for a tumor suppressor p...

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Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1

Primary hyperparathyroidism may occur as a part of an inherited syndrome in a combination with pancreatic endocrine tumours and/or pituitary adenoma, which is classified as Multiple Endocrine Neoplasia type 1 (MEN-1). This syndrome is caused by a germline mutation in MEN-1 gene encoding a tumour-suppressor protein, menin. Primary hyperparathyroidism is the most frequent clinical presentation of...

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Multiple endocrine neoplasia type 1

Combined clinical and laboratory investigations of MEN-1 have resulted in an increased understanding of this disorder, which may be inherited as an autosomal dominant condition. Defining the features of each disease manifestation in MEN-1 has improved patient management and treatment and has facilitated a screening protocol. Application of the techniques of molecular biology has enabled the ide...

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Multiple endocrine neoplasia type 1.

Multiple endocrine neoplasia-1 (MEN-1) is an autosomal dominant inherited syndrome that occurs due to inactivating mutations of the MEN1 gene locus, coding for a tumor-suppressor protein, menin. The components of MEN-1 are hyperparathyroidism due to multiple parathyroid adenomas, pancreatic neuroendocrine tumors, and pituitary adenomas, in addition to some less common neoplastic manifestations....

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ژورنال

عنوان ژورنال: Annals of Surgery

سال: 2000

ISSN: 0003-4932

DOI: 10.1097/00000658-200006000-00016