multiple endocrine neoplasia type 1

Summary

Summary: A form of multiple endocrine neoplasia that is characterized by the combined occurrence of tumors in the PARATHYROID GLANDS, the PITUITARY GLAND, and the PANCREATIC ISLETS. The resulting clinical signs include HYPERPARATHYROIDISM; HYPERCALCEMIA; HYPERPROLACTINEMIA; CUSHING DISEASE; GASTRINOMA; and ZOLLINGER-ELLISON SYNDROME. This disease is due to loss-of-function of the MEN1 gene, a tumor suppressor gene (GENES, TUMOR SUPPRESSOR) on CHROMOSOME 11 (Locus: 11q13).

Top Publications

  1. Gracanin A, Dreijerink K, van der Luijt R, Lips C, Höppener J. Tissue selectivity in multiple endocrine neoplasia type 1-associated tumorigenesis. Cancer Res. 2009;69:6371-4 pubmed publisher
    The phenotype of the multiple endocrine neoplasia type 1 (MEN1) syndrome cannot be explained solely by the expression pattern of the predisposing gene MEN1 and its encoded protein, menin...
  2. Kim H, Park J, Kim C, Kang E, Cha B, Lim S, et al. A case of multiple endocrine neoplasia type 1 combined with papillary thyroid carcinoma. Yonsei Med J. 2008;49:503-6 pubmed publisher
    This is the first report of papillary thyroid carcinoma combined with multiple endocrine neoplasia type 1 (MEN1) in Korea...
  3. Pieterman C, Schreinemakers J, Koppeschaar H, Vriens M, Rinkes I, Zonnenberg B, et al. Multiple endocrine neoplasia type 1 (MEN1): its manifestations and effect of genetic screening on clinical outcome. Clin Endocrinol (Oxf). 2009;70:575-81 pubmed publisher
    Effect of genetic screening on outcome in multiple endocrine neoplasia type 1 (MEN1) remains unclear. Expression of MEN1 is described using currently available diagnostic techniques...
  4. Norton J, Jensen R. Role of surgery in Zollinger-Ellison syndrome. J Am Coll Surg. 2007;205:S34-7 pubmed
  5. Lemos M, Thakker R. Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene. Hum Mutat. 2008;29:22-32 pubmed
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of tumors of the parathyroids, pancreas, and anterior pituitary...
  6. Chen G, A J, Wang M, Farley S, Lee L, Lee L, et al. Menin promotes the Wnt signaling pathway in pancreatic endocrine cells. Mol Cancer Res. 2008;6:1894-907 pubmed publisher
    Menin is a tumor suppressor protein mutated in patients with multiple endocrine neoplasia type 1. We show that menin is essential for canonical Wnt/beta-catenin signaling in cultured rodent islet tumor cells...
  7. Montenegro F, Lourenço D, Tavares M, Arap S, Nascimento C, Massoni Neto L, et al. Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center. Clinics (Sao Paulo). 2012;67 Suppl 1:131-9 pubmed
    ..Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical ..
  8. Balogh K, Racz K, Patocs A, Hunyady L. Menin and its interacting proteins: elucidation of menin function. Trends Endocrinol Metab. 2006;17:357-64 pubmed
    The multiple endocrine neoplasia type 1 (MEN1) gene is a tumor suppressor gene encoding a 610 amino acid nuclear protein, menin...
  9. Costa M, Domenice S, Toledo R, Lourenço D, Lourenço D, Latronico A, et al. Glucose-dependent insulinotropic peptide receptor overexpression in adrenocortical hyperplasia in MEN1 syndrome without loss of heterozygosity at the 11q13 locus. Clinics (Sao Paulo). 2011;66:529-33 pubmed
    ..New prospective studies will be able to clarify the exact role of the glucose-dependent insulinotropic peptide receptor in the molecular pathogenesis of MEN1 adrenocortical lesions. ..

More Information

Publications62

  1. Thakker R. Multiple endocrine neoplasia type 1 (MEN1). Best Pract Res Clin Endocrinol Metab. 2010;24:355-70 pubmed publisher
    b>Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal-dominant disorder characterised by the occurrence of tumours of the parathyroids, pancreas and anterior pituitary...
  2. Davenport C, Agha A. The role of menin in parathyroid tumorigenesis. Adv Exp Med Biol. 2009;668:79-86 pubmed
    ..the almost universal occurrence of parathyroid tumors accompanying the inactivation of menin in multiple endocrine neoplasia Type 1 (MEN1) syndrome and the high rate of somatic menin gene mutations seen in sporadic parathyroid ..
  3. Akerstrom G, Stalberg P. Surgical management of MEN-1 and -2: state of the art. Surg Clin North Am. 2009;89:1047-68 pubmed publisher
    ..Prophylactic thyroidectomy based on DNA testing in the MEN-2 syndrome is considered one of the greater achievements in cancer treatment, because it may be performed before thyroid carcinoma development and provides cure for the patient...
  4. Gauger P, Doherty G, Broome J, Miller B, Thompson N. Completion pancreatectomy and duodenectomy for recurrent MEN-1 pancreaticoduodenal endocrine neoplasms. Surgery. 2009;146:801-6; discussion 807-8 pubmed publisher
  5. Busygina V, Kottemann M, Scott K, Plon S, Bale A. Multiple endocrine neoplasia type 1 interacts with forkhead transcription factor CHES1 in DNA damage response. Cancer Res. 2006;66:8397-403 pubmed
    b>Multiple endocrine neoplasia type 1 (MEN1) is a cancer susceptibility syndrome affecting several endocrine tissues...
  6. Yokoyama A, Cleary M. Menin critically links MLL proteins with LEDGF on cancer-associated target genes. Cancer Cell. 2008;14:36-46 pubmed publisher
    ..Conversely, a subset of menin mutations in multiple endocrine neoplasia type 1 patients abrogate interaction with LEDGF while preserving MLL interaction but nevertheless ..
  7. Horvath A, Stratakis C. Clinical and molecular genetics of acromegaly: MEN1, Carney complex, McCune-Albright syndrome, familial acromegaly and genetic defects in sporadic tumors. Rev Endocr Metab Disord. 2008;9:1-11 pubmed publisher
    ..In conclusion, we attempt to present an overall, integrative approach to the human molecular genetics of both familiar and sporadic pituitary tumors...
  8. Powell A, Alexander H, Pingpank J, Steinberg S, Skarulis M, Bartlett D, et al. The utility of routine transcervical thymectomy for multiple endocrine neoplasia 1-related hyperparathyroidism. Surgery. 2008;144:878-83; discussion 883-4 pubmed publisher
    ..5 or 4 parathyroid glands and transcervical thymectomy (TCT). We reviewed our experience with initial operation for primary HPT to determine the outcome and utility of routine TCT...
  9. Harding B, Lemos M, Reed A, Walls G, Jeyabalan J, Bowl M, et al. Multiple endocrine neoplasia type 1 knockout mice develop parathyroid, pancreatic, pituitary and adrenal tumours with hypercalcaemia, hypophosphataemia and hypercorticosteronaemia. Endocr Relat Cancer. 2009;16:1313-27 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized in man by parathyroid, pancreatic, pituitary and adrenal tumours...
  10. Schreinemakers J, Pieterman C, Scholten A, Vriens M, Valk G, Rinkes I. The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review. World J Surg. 2011;35:1993-2005 pubmed publisher
    ..The optimal surgical approach for patients with primary hyperparathyroidism (pHPT) and multiple endocrine neoplasia 1 (MEN1) is controversial. We sought to determine the optimal type of surgery for pHPT in MEN1...
  11. Belar O, de la Hoz C, Pérez Nanclares G, Castano L, Gaztambide S. Novel mutations in MEN1, CDKN1B and AIP genes in patients with multiple endocrine neoplasia type 1 syndrome in Spain. Clin Endocrinol (Oxf). 2012;76:719-24 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder mostly owing to a genetic defect in MEN1 gene. Not all patients with MEN1 phenotype present a defect in this gene...
  12. Yip L, Ogilvie J, Challinor S, Salata R, Thull D, Yim J, et al. Identification of multiple endocrine neoplasia type 1 in patients with apparent sporadic primary hyperparathyroidism. Surgery. 2008;144:1002-6; discussion 1006-7 pubmed publisher
    ..We instituted a 6-question panel (6Q) to routinely screen for MEN1 preoperatively...
  13. Barbe C, Murat A, Dupas B, Ruszniewski P, Tabarin A, Vullierme M, et al. Magnetic resonance imaging versus endoscopic ultrasonography for the detection of pancreatic tumours in multiple endocrine neoplasia type 1. Dig Liver Dis. 2012;44:228-34 pubmed publisher
    In multiple endocrine neoplasia type 1, the main risk factor for metastases is pancreatic tumour size...
  14. Berna M, Hoffmann K, Serrano J, Gibril F, Jensen R. Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature. Medicine (Baltimore). 2006;85:295-330 pubmed
    ..Most clinical variables (multiple endocrine neoplasia type 1 status, presence or absence of the most common symptoms, prior medical treatment) are not ..
  15. Kann P, Balakina E, Ivan D, Bartsch D, Meyer S, Klose K, et al. Natural course of small, asymptomatic neuroendocrine pancreatic tumours in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study. Endocr Relat Cancer. 2006;13:1195-202 pubmed
    ..and experience with EUS imaging is limited, there is no clear evidence for clinical management in multiple endocrine neoplasia type 1 (MEN1)...
  16. Veldman M, Reading C, Farrell M, Mullan B, Wermers R, Grant C, et al. Percutaneous parathyroid ethanol ablation in patients with multiple endocrine neoplasia type 1. AJR Am J Roentgenol. 2008;191:1740-4 pubmed publisher
    The objective of our study was to show the efficacy and safety of percutaneous ethanol ablation in managing recurrent primary hyperparathyroidism in patients with multiple endocrine neoplasia type 1 (MEN1) after subtotal parathyroidectomy.
  17. Newey P, Jeyabalan J, Walls G, Christie P, Gleeson F, Gould S, et al. Asymptomatic children with multiple endocrine neoplasia type 1 mutations may harbor nonfunctioning pancreatic neuroendocrine tumors. J Clin Endocrinol Metab. 2009;94:3640-6 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) is characterized by the occurrence of parathyroid, pituitary, and pancreatic tumors...
  18. Schott M, Sagert C, Willenberg H, Schinner S, Ramp U, Varro A, et al. Carcinogenic hypergastrinemia: signet-ring cell carcinoma in a patient with multiple endocrine neoplasia type 1 with Zollinger-Ellison's syndrome. J Clin Endocrinol Metab. 2007;92:3378-82 pubmed
    ..Mutations in the epithelial cadherin (E-cadherin) gene have been shown to be associated with the occurrence of diffuse gastric carcinomas in affected families...
  19. Balasubramanian D, Scacheri P. Functional studies of menin through genetic manipulation of the Men1 homolog in mice. Adv Exp Med Biol. 2009;668:105-15 pubmed
  20. Salmeron M, Gonzalez J, Sancho Insenser J, Fornos J, Goday A, Perez N, et al. Causes and treatment of recurrent hyperparathyroidism after subtotal parathyroidectomy in the presence of multiple endocrine neoplasia 1. World J Surg. 2010;34:1325-31 pubmed publisher
    ..parathyroidectomy (SPTX) is the treatment of choice for hyperparathyroidism in a patient with multiple endocrine neoplasia type 1 (HPT-MEN-1)...
  21. Imamura M, Komoto I, Ota S, Hiratsuka T, Kosugi S, Doi R, et al. Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients. World J Gastroenterol. 2011;17:1343-53 pubmed publisher
    To search for the optimal surgery for gastrinoma and duodenopancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1.
  22. Dreijerink K, Höppener J, Timmers H, Lips C. Mechanisms of disease: multiple endocrine neoplasia type 1-relation to chromatin modifications and transcription regulation. Nat Clin Pract Endocrinol Metab. 2006;2:562-70 pubmed
    b>Multiple endocrine neoplasia type 1 (MEN1) is a hereditary tumor syndrome characterized by tumors of the parathyroid glands, the pancreatic islets, the pituitary gland, the adrenal glands, as well as by neuroendocrine carcinoid tumors, ..
  23. Giusti F, Tonelli F, Brandi M. Primary hyperparathyroidism in multiple endocrine neoplasia type 1: when to perform surgery?. Clinics (Sao Paulo). 2012;67 Suppl 1:141-4 pubmed
    ..In rare circumstances, it is associated with familial syndromes, such as multiple endocrine neoplasia type 1. This syndrome is caused by a germline mutation in the multiple endocrine neoplasia type 1 gene ..
  24. Tonelli F, Giudici F, Cavalli T, Brandi M. Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy. Clinics (Sao Paulo). 2012;67 Suppl 1:155-60 pubmed
    Usually, primary hyperparathyroidism is the first endocrinopathy to be diagnosed in patients with multiple endocrine neoplasia type 1, and is also the most common one...
  25. Trouillas J, Labat Moleur F, Sturm N, Kujas M, Heymann M, Figarella Branger D, et al. Pituitary tumors and hyperplasia in multiple endocrine neoplasia type 1 syndrome (MEN1): a case-control study in a series of 77 patients versus 2509 non-MEN1 patients. Am J Surg Pathol. 2008;32:534-43 pubmed publisher
    ..All types of mutation were observed, including frameshifts, nonsenses, missenses, and 1 case of germline MEN1 encompassing large deletion, strongly suggesting the absence of any phenotype-genotype correlation...
  26. Sakurai A, Suzuki S, Kosugi S, Okamoto T, Uchino S, Miya A, et al. Multiple endocrine neoplasia type 1 in Japan: establishment and analysis of a multicentre database. Clin Endocrinol (Oxf). 2012;76:533-9 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) is less well recognized in Asian countries, including Japan, than in the West. The clinical features and optimal management of MEN1 have yet to be clarified in Japan...
  27. Almeida M, Stratakis C. Solid tumors associated with multiple endocrine neoplasias. Cancer Genet Cytogenet. 2010;203:30-6 pubmed publisher
    ..The Carney complex is yet another form of MEN that is characterized by skin tumors and pigmented lesions, myxomas, schwannomas, and various endocrine neoplasias...
  28. Stratakis C, Tichomirowa M, Boikos S, Azevedo M, Lodish M, Martari M, et al. The role of germline AIP, MEN1, PRKAR1A, CDKN1B and CDKN2C mutations in causing pituitary adenomas in a large cohort of children, adolescents, and patients with genetic syndromes. Clin Genet. 2010;78:457-63 pubmed publisher
    ..Thus, germline AIP or MEN1 gene mutations are frequent among pediatric patients with GH- or PRL-secreting PA but are significantly rarer in pediatric CD; PRKAR1A mutations are not present in PA outside of Carney complex. ..
  29. Balogh K, Patocs A, Hunyady L, Racz K. Menin dynamics and functional insight: take your partners. Mol Cell Endocrinol. 2010;326:80-4 pubmed publisher
    ..number of recent findings and novelties, menin, the protein encoded by the gene responsible for multiple endocrine neoplasia type 1 syndrome, still remains a mystery...
  30. Coutinho F, Lourenço D, Toledo R, Montenegro F, Toledo S. Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Clinics (Sao Paulo). 2012;67 Suppl 1:169-72 pubmed
    ..investigators, but few have investigated this topic in primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Further, as far as we know, only two studies have consistently evaluated bone mineral density ..
  31. Berna M, Annibale B, Marignani M, Luong T, Corleto V, Pace A, et al. A prospective study of gastric carcinoids and enterochromaffin-like cell changes in multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: identification of risk factors. J Clin Endocrinol Metab. 2008;93:1582-91 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) patients frequently develop Zollinger-Ellison syndrome (ZES)...
  32. Shih R, Fackler S, Maturo S, True M, Brennan J, Wells D. Parathyroid carcinoma in multiple endocrine neoplasia type 1 with a classic germline mutation. Endocr Pract. 2009;15:567-72 pubmed publisher
    To report the case of a patient with multiple endocrine neoplasia type 1 (MEN 1) syndrome with con-comitant parathyroid carcinoma and a classic MEN1 germline mutation.
  33. Pieterman C, Vriens M, Dreijerink K, Van der Luijt R, Valk G. Care for patients with multiple endocrine neoplasia type 1: the current evidence base. Fam Cancer. 2011;10:157-71 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11...
  34. Lourenco D, Toledo R, Mackowiak I, Coutinho F, Cavalcanti M, Correia Deur J, et al. Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile. Eur J Endocrinol. 2008;159:259-74 pubmed publisher
    Only few large families with multiple endocrine neoplasia type 1 (MEN1) have been documented. Here, we aimed to investigate the clinical features of a seven-generation Brazilian pedigree, which included 715 at-risk family members.
  35. Fukuuchi A, Nagamura Y, Yaguchi H, Ohkura N, Obara T, Tsukada T. A whole MEN1 gene deletion flanked by Alu repeats in a family with multiple endocrine neoplasia type 1. Jpn J Clin Oncol. 2006;36:739-44 pubmed
    b>Multiple endocrine neoplasia type 1 is an autosomal dominant cancer syndrome characterized by pituitary, parathyroid and enteropancreatic endocrine tumors, which is caused by germline mutations of the tumor suppressor gene MEN1...
  36. Jiang X, Lu J, Cui B, Zhao Y, Wang W, Liu J, et al. MEN1 mutation analysis in Chinese patients with multiple endocrine neoplasia type 1. Endocr Relat Cancer. 2007;14:1073-9 pubmed
    b>Multiple endocrine neoplasia type 1 (MEN1) is an inherited tumour syndrome characterized by the development of tumours of the parathyroid, anterior pituitary and pancreatic islets, etc...
  37. Milanesi A, Yu R, Geller S, Burton D, Deftos L, Wolin E. Concurrent primary hyperparathyroidism and humoral hypercalcemia of malignancy in a patient with multiple endocrine neoplasia type 1. Pancreas. 2011;40:634-7 pubmed publisher
    We report a patient with multiple endocrine neoplasia type 1 presenting with elevation of parathyroid hormone-related protein (PTHrP) from a metastatic pancreatic neuroendocrine tumor (PNET), and parathyroid hormone (PTH) from primary ..
  38. Beckers A, Daly A. The clinical, pathological, and genetic features of familial isolated pituitary adenomas. Eur J Endocrinol. 2007;157:371-82 pubmed
    Pituitary adenomas occur in a familial setting in multiple endocrine neoplasia type 1 (MEN1) and Carney's complex (CNC), which occur due to mutations in the genes MEN1 and PRKAR1A respectively...
  39. Anlauf M, Perren A, Kloppel G. Endocrine precursor lesions and microadenomas of the duodenum and pancreas with and without MEN1: criteria, molecular concepts and clinical significance. Pathobiology. 2007;74:279-84 pubmed
    ..This review focuses on precursor lesions of duodenal and pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) and also discusses 2 new disease entities of pancreatic microadenomatosis...
  40. Perren A, Anlauf M, Henopp T, Rudolph T, Schmitt A, Raffel A, et al. Multiple endocrine neoplasia type 1 (MEN1): loss of one MEN1 allele in tumors and monohormonal endocrine cell clusters but not in islet hyperplasia of the pancreas. J Clin Endocrinol Metab. 2007;92:1118-28 pubmed
    The occurrence of multiple small pancreatic endocrine tumors in patients suffering from multiple endocrine neoplasia type 1 (MEN1) represents a unique possibility to study early neoplasms and their potential precursor lesions...
  41. Lemos M, Harding B, Shalet S, Thakker R. A novel MEN1 intronic mutation associated with multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf). 2007;66:709-13 pubmed
    ..To investigate a family with an unusual combination of multiple endocrine neoplasia (MEN1) and the McCune-Albright syndrome for MEN1 mutations and activating GNAS1 mutations at codons Arg201 and Gln227...
  42. Machens A, Schaaf L, Karges W, Frank Raue K, Bartsch D, Rothmund M, et al. Age-related penetrance of endocrine tumours in multiple endocrine neoplasia type 1 (MEN1): a multicentre study of 258 gene carriers. Clin Endocrinol (Oxf). 2007;67:613-22 pubmed
    In multiple endocrine neoplasia type 1 (MEN1), age-related tumour penetrance according to the type of MEN1 germline mutation has not been investigated in-depth...
  43. La P, Yang Y, Karnik S, Silva A, Schnepp R, Kim S, et al. Menin-mediated caspase 8 expression in suppressing multiple endocrine neoplasia type 1. J Biol Chem. 2007;282:31332-40 pubmed
    b>Multiple endocrine neoplasia type 1 (MEN1) is a familial tumor syndrome linked to mutation of the MEN1 gene, which encodes a tumor suppressor, menin...
  44. Schaaf L, Pickel J, Zinner K, Hering U, Hofler M, Goretzki P, et al. Developing effective screening strategies in multiple endocrine neoplasia type 1 (MEN 1) on the basis of clinical and sequencing data of German patients with MEN 1. Exp Clin Endocrinol Diabetes. 2007;115:509-17 pubmed
    ..As the tumors appear in an unpredictable schedule, uncertainty about screening programs is persisting...
  45. Scheithauer B, Kovacs K, Nose V, Lombardero M, Osamura Y, Lloyd R, et al. Multiple endocrine neoplasia type 1-associated thyrotropin-producing pituitary carcinoma: report of a probable de novo example. Hum Pathol. 2009;40:270-8 pubmed publisher
    ..and morphologically well-documented example of pituitary thyrotropin cell carcinoma in a man with multiple endocrine neoplasia type 1 syndrome...
  46. Berna M, Hoffmann K, Long S, Serrano J, Gibril F, Jensen R. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of. Medicine (Baltimore). 2006;85:331-64 pubmed
    ..For secretin testing, the criterion with the highest sensitivity and specificity is an increase of > or =120 pg/mL, which should replace other criteria commonly used today...
  47. Igreja S, Chahal H, Akker S, Gueorguiev M, Popovic V, Damjanovic S, et al. Assessment of p27 (cyclin-dependent kinase inhibitor 1B) and aryl hydrocarbon receptor-interacting protein (AIP) genes in multiple endocrine neoplasia (MEN1) syndrome patients without any detectable MEN1 gene mutations. Clin Endocrinol (Oxf). 2009;70:259-64 pubmed publisher
    ..The objective of this study was to evaluate the possible contribution of CDKN1B and AIP germline mutations in a cohort of MEN1 mutation-negative MEN1 syndrome patients...
  48. Fendrich V, Langer P, Waldmann J, Bartsch D, Rothmund M. Management of sporadic and multiple endocrine neoplasia type 1 gastrinomas. Br J Surg. 2007;94:1331-41 pubmed
    ..Gastrinomas are functional endocrine duodenopancreatic tumours and are responsible for Zollinger-Ellison syndrome (ZES). Clinical presentation, localization techniques and operative management were reviewed...
  49. Shen H, He M, Powell A, Adem A, Lorang D, Heller C, et al. Recapitulation of pancreatic neuroendocrine tumors in human multiple endocrine neoplasia type I syndrome via Pdx1-directed inactivation of Men1. Cancer Res. 2009;69:1858-66 pubmed publisher
    b>Multiple endocrine neoplasia type 1 (MEN1) is an autosomal syndrome caused by mutations in the MEN1 tumor suppressor gene...
  50. Piecha G, Chudek J, Wiecek A. Multiple Endocrine Neoplasia type 1. Eur J Intern Med. 2008;19:99-103 pubmed publisher
    ..parathyroid hyperplasia with pancreatic endocrine tumours and/or pituitary adenoma is classified as Multiple Endocrine Neoplasia type 1 (MEN-1) and is caused by a germ-line mutation in MEN-1 gene encoding a tumour suppressor protein, ..
  51. Georgitsi M, Raitila A, Karhu A, van der Luijt R, Aalfs C, Sane T, et al. Germline CDKN1B/p27Kip1 mutation in multiple endocrine neoplasia. J Clin Endocrinol Metab. 2007;92:3321-5 pubmed
    Germline mutations in the MEN1 gene predispose to multiple endocrine neoplasia type 1 (MEN1) syndrome, but in up to 20-25% of clinical MEN1 cases, no MEN1 mutations can be found...
  52. Imamura M. Recent standardization of treatment strategy for pancreatic neuroendocrine tumors. World J Gastroenterol. 2010;16:4519-25 pubmed
    ..For curative resection of functioning PNET associated with multiple endocrine neoplasia type 1 (MEN 1) which are usually multiple and sometimes numerous, resection surgery of the pancreas and/or ..
  53. Oberg K. Genetics and molecular pathology of neuroendocrine gastrointestinal and pancreatic tumors (gastroenteropancreatic neuroendocrine tumors). Curr Opin Endocrinol Diabetes Obes. 2009;16:72-8 pubmed
    ..However, the WHO classification introduced in clinical practice will give more insight into genetic and molecular changes related to tumor subtypes...