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1. Montero C, Sanjuán P, Fernández Mdel M, Vidal I, Verea H, Cordido F: [Bronchial carcinoid and type 1 multiple endocrine neoplasia syndrome. A case report]. Arch Bronconeumol; 2010 Oct;46(10):559-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Bronchial carcinoid and type 1 multiple endocrine neoplasia syndrome. A case report].
  • [Transliterated title] Carcinoide bronquial y síndrome de neoplasias endocrinas múltiples TIPO 1. Aportación de un caso.
  • Carcinoid tumours of bronchial origin are rare in type 1 multiple endocrine neoplasia (MEN1) syndrome.
  • We report a patient with a type 1 MEN syndrome, with no respiratory symptoms, with hypergastrinaemia, and in whom a 5mm diameter nodule was detected in the wall of the left main bronchus by computed tomography.
  • [MeSH-major] Bronchial Neoplasms. Carcinoid Tumor. Multiple Endocrine Neoplasia Type 1. Neoplasms, Multiple Primary


2. Dreijerink KM, Varier RA, van Beekum O, Jeninga EH, Höppener JW, Lips CJ, Kummer JA, Kalkhoven E, Timmers HT: The multiple endocrine neoplasia type 1 (MEN1) tumor suppressor regulates peroxisome proliferator-activated receptor gamma-dependent adipocyte differentiation. Mol Cell Biol; 2009 Sep;29(18):5060-9
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  • [Title] The multiple endocrine neoplasia type 1 (MEN1) tumor suppressor regulates peroxisome proliferator-activated receptor gamma-dependent adipocyte differentiation.
  • Menin, the product of the MEN1 (multiple endocrine neoplasia type 1) tumor suppressor gene, is involved in activation of gene transcription as part of an MLL1 (mixed-lineage leukemia 1)/MLL2 (KMT2A/B)-containing protein complex which harbors methyltransferase activity for lysine 4 of histone H3 (H3K4).
  • As MEN1 patients frequently develop lipomas and peroxisome proliferator-activated receptor gamma (PPARgamma) is expressed in several MEN1-related tumor types, we investigated regulation of PPARgamma activity by menin.
  • We propose that menin is an important factor in PPARgamma-mediated adipogenesis and that loss of PPARgamma function may contribute to lipoma development in MEN1 patients.


3. Dreijerink KM, Lips CJ: Diagnosis and Management of Multiple Endocrine Neoplasia Type 1 (MEN1). Hered Cancer Clin Pract; 2005;3(1):1-6
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  • [Title] Diagnosis and Management of Multiple Endocrine Neoplasia Type 1 (MEN1).
  • Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominantly inherited disorder, characterised by the occurrence of tumours of the parathyroid glands, the pancreatic islets, the pituitary gland, the adrenal glands and neuroendocrine carcinoid tumours.
  • Carcinoid tumours of the thymus and pancreatic-duodenal gastrinomas are the most harmful tumour types, since these tumours have malignant potential and curative treatment is difficult to achieve.MEN1 is caused by germline mutations of the MEN1 tumour suppressor gene.
  • MEN1 patients and their family members, family members of mutation carriers and patients who are clinically suspected to be carriers of a MEN1 gene mutation are eligible for mutation analysis.
  • MEN1-associated tumours can be detected and treated at an early stage through periodical clinical monitoring of mutation carriers.

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  • (PMID = 20223025.001).
  • [ISSN] 1897-4287
  • [Journal-full-title] Hereditary cancer in clinical practice
  • [ISO-abbreviation] Hered Cancer Clin Pract
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Poland
  • [Other-IDs] NLM/ PMC2837063
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4. Agha A, Carpenter R, Bhattacharya S, Edmonson SJ, Carlsen E, Monson JP: Parathyroid carcinoma in multiple endocrine neoplasia type 1 (MEN1) syndrome: two case reports of an unrecognised entity. J Endocrinol Invest; 2007 Feb;30(2):145-9
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  • [Title] Parathyroid carcinoma in multiple endocrine neoplasia type 1 (MEN1) syndrome: two case reports of an unrecognised entity.
  • CONTEXT: Primary hyperparathyroidism occurs in almost all patients with the syndrome of multiple endocrine neoplasia type 1 (MEN1), but the association of MEN1 with parathyroid carcinoma has only been described previously in a single patient.
  • In this report, we describe two further cases of parathyroid carcinoma presenting in MEN1 syndrome.
  • Remarkably, she also reported primary amenorrhea and was found to have an invasive pituitary lactotroph adenoma, which was treated with cabergoline and external beam radiotherapy.
  • CONCLUSIONS: This case report describes two further patients in whom parathyroid carcinomas occurred in the context of MEN1, which gives a new insight to the possible presenting phenotype of this condition.
  • Both patients had negative genetic screening for classic MEN1 gene mutation, which may suggest that one or more novel occult mutations may be responsible for this aggressive phenotype.
  • [MeSH-major] Carcinoma / diagnosis. Multiple Endocrine Neoplasia Type 1 / diagnosis. Parathyroid Neoplasms / diagnosis
  • [MeSH-minor] Adult. Aged. Diagnosis, Differential. Female. Humans. Male. Syndrome


5. Thakker RV: Multiple endocrine neoplasia type 1 (MEN1). Best Pract Res Clin Endocrinol Metab; 2010 Jun;24(3):355-70
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  • [Title] Multiple endocrine neoplasia type 1 (MEN1).
  • Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal-dominant disorder characterised by the occurrence of tumours of the parathyroids, pancreas and anterior pituitary.
  • The MEN1 gene, consists of 10 exons that encode a 610-amino acid protein referred to as Menin.
  • Germ-line mutations usually result in MEN1 or occasionally in an allelic variant referred to as Familial Isolated Hyperparathyroidism (FIHP).
  • MEN1 tumours frequently have loss of heterozygosity (LOH) of the MEN1 locus, which is consistent with a tumour suppressor role of MEN1.
  • Furthermore, somatic abnormalities of MEN1 have been reported in MEN1 and non-MEN1 endocrine tumours.
  • The mutations are scattered throughout the >9 kb genomic sequence of the MEN1 gene.
  • The majority of MEN1 mutations are likely to disrupt the interactions of Menin with other proteins and thereby alter critical events in cell cycle regulation and proliferation.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Parathyroid Neoplasms / genetics. Pituitary Neoplasms / genetics
  • [MeSH-minor] Animals. Disease Models, Animal. Germ-Line Mutation. Humans. Mice. Neuroendocrine Tumors / genetics. Neuroendocrine Tumors / pathology. Proto-Oncogene Proteins / genetics

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  • [Copyright] Copyright 2010 Elsevier Ltd. All rights reserved.
  • (PMID = 20833329.001).
  • [ISSN] 1878-1594
  • [Journal-full-title] Best practice & research. Clinical endocrinology & metabolism
  • [ISO-abbreviation] Best Pract. Res. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Grant] United Kingdom / Medical Research Council / / MRC/ G9825289; United Kingdom / Medical Research Council / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; Pancreatic islet cell tumors
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6. Anlauf M, Perren A, Klöppel G: [Gastrin cell hyperplasia associated with duodenal MEN1-related gastrinomas: histopathology and genetics]. Verh Dtsch Ges Pathol; 2007;91:320-9
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  • [Title] [Gastrin cell hyperplasia associated with duodenal MEN1-related gastrinomas: histopathology and genetics].
  • [Transliterated title] Gastrinzellhyperplasie bei duodenalen MEN1-assoziierten Gastrinomen: Histopathologie und Genetik.
  • In this study we investigated whether preneoplastic lesions can be identified in sporadic gastrinomas and in gastrinomas in multiple endocrine neoplasia type 1 (MEN1) patients.
  • These lesions were tested for loss of heterozygosity (LOH) of the MEN1 gene locus on chromosome 11q13.
  • MATERIAL AND METHODS: Tissue specimens from 25 patients with Zollinger-Ellison syndrome (ZES) were analyzed.
  • The MEN1 status was assessed clinically and by mutational analysis.
  • RESULTS: Hyperplastic gastrin cell lesions were present in the nontumorous mucosa of all MEN1 patients, but not in 12 patients with sporadic duodenal gastrinomas.
  • 11q13 LOH was, however, detected in duodenal gastrinomas, some as small as 300 microm in diameter, in 13 patients with MEN1.
  • CONCLUSIONS: MEN1-associated duodenal gastrinomas, but not sporadic gastrinomas, are associated with gastrin cell hyperplasia.
  • It is therefore likely that hyperplastic gastrin cell lesions precede the development of MEN1-associated duodenal gastrinomas.
  • Allelic deletion of the MEN1 gene locus may reflect a decisive initial event in the development of multifocal MEN1-associated gastrinomas from hyperplastic gastrin cell lesions.
  • [MeSH-major] Duodenal Neoplasms / genetics. Duodenal Neoplasms / pathology. Multiple Endocrine Neoplasia Type 1 / genetics. Multiple Endocrine Neoplasia Type 1 / pathology. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adult. Chromosome Mapping. Female. Humans. Hyperplasia. Immunohistochemistry. Loss of Heterozygosity. Male. Microscopy, Fluorescence. Middle Aged. Sequence Deletion. Stomach Neoplasms / classification. Stomach Neoplasms / genetics. Stomach Neoplasms / pathology

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  • (PMID = 18314630.001).
  • [ISSN] 0070-4113
  • [Journal-full-title] Verhandlungen der Deutschen Gesellschaft für Pathologie
  • [ISO-abbreviation] Verh Dtsch Ges Pathol
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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7. Lourenço DM Jr, Toledo RA, Mackowiak II, Coutinho FL, Cavalcanti MG, Correia-Deur JE, Montenegro F, Siqueira SA, Margarido LC, Machado MC, Toledo SP: Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile. Eur J Endocrinol; 2008 Sep;159(3):259-74
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  • [Title] Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile.
  • OBJECTIVE: Only few large families with multiple endocrine neoplasia type 1 (MEN1) have been documented.
  • DESIGN: Genealogical and geographic analysis was used to identify the MEN1 pedigree.
  • RESULTS: Our genetic data indicated that a founding mutation in the MEN1 gene has occurred in this extended Brazilian family.
  • Fifty family members were diagnosed with MEN1.
  • Very high frequencies of functioning and non-functioning MEN1-related tumors were documented and the prevalence of prolactinoma (29.6%) was similar to that previously described in prolactinoma-variant Burin (32%).
  • In addition, bone mineral density analysis revealed severe osteoporosis (T, -2.87+/-0.32) of compact bone (distal radius) in hyperparathyroidism (HPT)/MEN1 patients, while marked bone mineral loss in the lumbar spine (T, -1.95+/-0.39), with most cancellous bone, and femoral neck (mixed composition; T, -1.48+/-0.27) were also present.
  • CONCLUSIONS: In this study, we described clinically and genetically the fifth largest MEN1 family in the literature.
  • Our data confirm previous findings suggesting that prevalence of MEN1-related tumors in large families may differ from reports combining cumulative data of small families.
  • Furthermore, we were able to evaluate the bone status in HPT/MEN1 cases, a subject that has been incompletely approached in the literature.
  • We discussed the bone loss pattern found in our MEN1 patients comparing with that of patients with sporadic primary HPT.


8. Seigne C, Fontanière S, Carreira C, Lu J, Tong WM, Fontanière B, Wang ZQ, Zhang CX, Frappart L: Characterisation of prostate cancer lesions in heterozygous Men1 mutant mice. BMC Cancer; 2010;10:395
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  • [Title] Characterisation of prostate cancer lesions in heterozygous Men1 mutant mice.
  • BACKGROUND: Mutations of the MEN1 gene predispose to multiple endocrine neoplasia type 1 (MEN1) syndrome.
  • Our group and others have shown that Men1 disruption in mice recapitulates MEN1 pathology.
  • Intriguingly, rare lesions in hormone-dependent tissues, such as prostate and mammary glands, were also observed in the Men1 mutant mice.
  • METHODS: To study the occurrence of prostate lesions, we followed a male mouse cohort of 47 Men1+/- mice and 23 age-matched control littermates, starting at 18 months of age, and analysed the prostate glands from the cohort.
  • RESULTS: Six Men1+/- mice (12.8%) developed prostate cancer, including two adenocarcinomas and four in situ carcinomas, while none of the control mice developed cancerous lesions.
  • The expression of menin encoded by the Men1 gene was found to be drastically reduced in all carcinomas, and partial LOH of the wild-type Men1 allele was detected in three of the five analysed lesions.
  • Furthermore, our data showed that the expression of the cyclin-dependent kinase inhibitor CDKN1B (p27), a Men1 target gene known to be inactivated during prostate cell tumorigenesis, was notably decreased in the prostate cancers that developed in the mutant mice.
  • CONCLUSION: Our work suggests the possible involvement of Men1 inactivation in the tumorigenesis of the prostate gland.
  • [MeSH-major] Adenocarcinoma / genetics. Adenocarcinoma / pathology. Loss of Heterozygosity. Prostatic Neoplasms / genetics. Prostatic Neoplasms / pathology. Proto-Oncogene Proteins / physiology


9. Faggiano A, Tavares LB, Tauchmanova L, Milone F, Mansueto G, Ramundo V, De Caro ML, Lombardi G, De Rosa G, Colao A: Effect of treatment with depot somatostatin analogue octreotide on primary hyperparathyroidism (PHP) in multiple endocrine neoplasia type 1 (MEN1) patients. Clin Endocrinol (Oxf); 2008 Nov;69(5):756-62
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  • [Title] Effect of treatment with depot somatostatin analogue octreotide on primary hyperparathyroidism (PHP) in multiple endocrine neoplasia type 1 (MEN1) patients.
  • BACKGROUND: In patients with multiple endocrine neoplasia type 1 (MEN1), expression of somatostatin receptor (SST) in parathyroid adenomas and effectiveness of therapy with somatostatin analogues on primary hyperparathyroidism (PHP) have been scarcely investigated.
  • OBJECTIVE: To evaluate the effects of depot long acting octreotide (OCT-LAR) in patients with MEN1-related PHP.
  • PATIENTS: Eight patients with a genetically confirmed MEN1, presenting both PHP and duodeno-pancreatic neuroendocrine tumours (NET), were enrolled.
  • CONCLUSION: Six months of OCT-LAR therapy controlled hypercalcaemia and hypercalciuria in two-thirds of patients with MEN1-related PHP.
  • Direct OCT-LAR effects mediated by binding to SST expression on parathyroid tumour cells are likely the main mechanism to explain the activity of this compound on calcium and phosphorus abnormalities in MEN1 PHP.
  • [MeSH-major] Duodenal Neoplasms / drug therapy. Hyperparathyroidism, Primary / complications. Multiple Endocrine Neoplasia Type 1 / drug therapy. Neuroendocrine Tumors / drug therapy. Octreotide / administration & dosage. Pancreatic Neoplasms / drug therapy


10. Machens A, Schaaf L, Karges W, Frank-Raue K, Bartsch DK, Rothmund M, Schneyer U, Goretzki P, Raue F, Dralle H: Age-related penetrance of endocrine tumours in multiple endocrine neoplasia type 1 (MEN1): a multicentre study of 258 gene carriers. Clin Endocrinol (Oxf); 2007 Oct;67(4):613-22
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  • [Title] Age-related penetrance of endocrine tumours in multiple endocrine neoplasia type 1 (MEN1): a multicentre study of 258 gene carriers.
  • OBJECTIVE: 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.
  • This study was conducted to examine whether carriers of out-of-frame/truncating and in-frame MEN1 mutations differ in age-related tumour penetrance.
  • DESIGN: A multicentre study with biochemical, hormonal and radiological screening for MEN1-associated tumours.
  • PATIENTS: A total of 258 MEN1 carriers from six major German tertiary referral centres averaging 43 years of age at last follow-up.
  • MEASUREMENTS: Main outcome measure was time to first diagnosis of MEN1-associated tumours.
  • RESULTS: Independent of the year of birth and observation period, time to first tumour diagnosis did not vary much by the type of MEN1 germline mutation or endocrine organ system, and perhaps not even by the type of endocrine tumour when the amount of time was considered by which the diagnosis probably has been advanced through the manifestation of hormonal symptoms.
  • CONCLUSIONS: Because of our inability of predicting tumour penetrance and malignant transformation individually, life-long follow-up of MEN1 carriers is warranted to prevent tumour morbidity.
  • [MeSH-major] Aging / genetics. Germ-Line Mutation. Multiple Endocrine Neoplasia Type 1 / genetics. Penetrance


11. Igaz P: MEN1 clinical background. Adv Exp Med Biol; 2009;668:1-15
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  • [Title] MEN1 clinical background.
  • Multiple endocrine neoplasia Type 1 (MEN1) is a rare hereditary tumor syndrome predisposing to tumor development in several endocrine organs.
  • Its major manifestations include hyperparathyroidism, tumors of endocrine pancreas and pituitary.
  • Beside these three, several other endocrine (adrenocortical, foregut carcinoid) and nonendocrine (lipoma, angiofibroma, collagenoma, ependymoma, meningioma) tumors have been described to be associated with this syndrome.
  • Both familial and sporadic forms of the disease are known.
  • The diagnosis of MEN1 can be established if two of the three major manifestations are found in the same patient, whereas the diagnosis of familial MEN1 requires one MEN1 patient and a first degree relative with at least one MEN1 manifestation.
  • MEN1 is transmitted as an autosomal dominant trait with high penetrance, approaching 95-100% by the age of 60.
  • Both benign (parathyroid, anterior pituitary) and malignant (gastrinoma, glucagonoma) lesions may develop in MEN1 patients.
  • Regular surveillance of MEN1 gene mutation carriers is necessary to reveal disease manifestations.
  • Several diagnostic modalities can be used to screen for and to examine MEN1-related tumors.
  • The therapy of MEN1-associated tumors requires specific approach in some cases, as multiple tumors and recurrence is frequently observed.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Multiple Endocrine Neoplasia Type 1 / pathology. Multiple Endocrine Neoplasia Type 1 / physiopathology
  • [MeSH-minor] Adult. Child. Child, Preschool. Diagnosis, Differential. Genetic Predisposition to Disease. Genetic Testing. Humans. Middle Aged. Multiple Endocrine Neoplasia Type 2a / genetics. Multiple Endocrine Neoplasia Type 2a / pathology. Multiple Endocrine Neoplasia Type 2a / physiopathology. Mutation. Young Adult

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  • (PMID = 20175448.001).
  • [ISSN] 0065-2598
  • [Journal-full-title] Advances in experimental medicine and biology
  • [ISO-abbreviation] Adv. Exp. Med. Biol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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12. Pieterman CR, Schreinemakers JM, Koppeschaar HP, Vriens MR, Rinkes IH, Zonnenberg BA, van der Luijt RB, Valk GD: Multiple endocrine neoplasia type 1 (MEN1): its manifestations and effect of genetic screening on clinical outcome. Clin Endocrinol (Oxf); 2009 Apr;70(4):575-81
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  • [Title] Multiple endocrine neoplasia type 1 (MEN1): its manifestations and effect of genetic screening on clinical outcome.
  • OBJECTIVE: 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.
  • Patients are divided into two groups: patients with a (i) clinical MEN1 diagnosis and (ii) MEN1 diagnosis by genetic screening.
  • PATIENTS AND MEASUREMENTS: Demographic and clinical data were collected on MEN1 patients treated in the UMCU up to 1 January 2008.
  • RESULTS: A total of 74 patients was included (median follow-up 5.5 year); 78% had hyperparathyroidism, 46% a pancreatic neuro-endocrine tumour (NET), 38% a pituitary abnormality, 8% a NET of other origin and 16% an adrenal adenoma at the end of follow-up.
  • All five MEN1-related tumours were seen as first manifestation.
  • Compared with patients identified by genetic screening, patients with a clinical MEN1 diagnosis had significantly more manifestations at diagnosis (P < 0.001) and at end of follow-up (P = 0.002).
  • Eleven of 30 patients with a genetic MEN1 diagnosis (mean age at diagnosis 30.0 years) already had manifestations at diagnosis.
  • CONCLUSIONS: MEN1 is a syndrome with high morbidity.
  • Genetic diagnosis is associated with less morbidity at diagnosis and at follow-up.
  • Early genetic diagnosis might therefore lead to improvement of long-term outcome.
  • [MeSH-major] Genetic Testing. Multiple Endocrine Neoplasia Type 1 / diagnosis. Multiple Endocrine Neoplasia Type 1 / genetics
  • [MeSH-minor] Adenoma / diagnosis. Adenoma / genetics. Adolescent. Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / genetics. Adult. Aged. Child. Cohort Studies. Female. Follow-Up Studies. Humans. Hyperparathyroidism / diagnosis. Hyperparathyroidism / genetics. Male. Middle Aged. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / genetics. Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / genetics. Prognosis. Retrospective Studies. Young Adult


13. Lubaroff DM, Vaena DA, Williams RD, Joudi FN, Smith MC, Zehr PS, Eastman J, Griffith K, Madsen TM, Johnson K: A phase II trial of an adenovirus/PSA vaccine for prostate cancer. J Clin Oncol; 2009 May 20;27(15_suppl):3065

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  • METHODS: In Protocol 1 men with recurrent prostate cancer after definitive initial treatment were randomized to arm A (vaccine injection alone at days 0, 30, and 60) or arm B (vaccine injection 14 days after initiation of androgen deprivation therapy).
  • In Protocol 2 men with hormone-refractory disease receive the vaccine alone at days 0, 30, and 60.
  • Each injection consists of 108 pfu of Ad/PSA vaccine suspended in a collagen matrix.

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  • (PMID = 27962013.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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14. Agarwal SK, Ozawa A, Mateo CM, Marx SJ: The MEN1 gene and pituitary tumours. Horm Res; 2009 Apr;71 Suppl 2:131-8
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  • [Title] The MEN1 gene and pituitary tumours.
  • Sporadic multiple endocrine neoplasia type 1 (MEN1) is defined as the occurrence of tumours in two of three main endocrine tissue types: parathyroid, pituitary and pancreaticoduodenal.
  • A prolactinoma variant or Burin variant of MEN1 was found to occur in three large kindreds, with more prolactinomas and fewer gastrinomas than typical MEN1.
  • MEN1 tumours differ from common tumours by showing features from the MEN1 gene (e.g. larger pituitary tumours).
  • They also show various expressions of tumour multiplicity; however, pituitary tumour in MEN1 is usually solitary.
  • Diagnosis in MEN1 carriers during childhood is not directed at cancers but at benign morbid tumours.
  • Morbid prolactinoma occurred at the age of 5 years in one MEN1 individual; hence, this is the earliest age at which to recommend tumour surveillance in carriers.
  • The MEN1 gene shows biallelic inactivation in 30% of some types of common variety endocrine tumours (e.g. parathyroid adenoma, gastrinoma, insulinoma and bronchial carcinoid), but in only 1-5% of common pituitary tumours.
  • Heterozygous knockout of MEN1 in mice provides a robust model of MEN1 and has been found to support further research on anti-angiogenesis therapy for pituitary tumours.
  • The rarity of MEN1 mutations in some MEN1-like states aids the identification of other mutated genes, such as AIP, HRPT2 and p27(Kip1).
  • We present recent clinical and basic findings about the MEN1 gene, particularly concerning hereditary vs. common variety pituitary tumours.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / metabolism. Prolactinoma / metabolism. Proto-Oncogene Proteins / metabolism

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  • [Copyright] Copyright 2009 S. Karger AG, Basel.
  • (PMID = 19407509.001).
  • [ISSN] 1423-0046
  • [Journal-full-title] Hormone research
  • [ISO-abbreviation] Horm. Res.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / CDC73 protein, human; 0 / CDKN1B protein, human; 0 / Cdkn1b protein, mouse; 0 / HRPT2 protein, mouse; 0 / Intracellular Signaling Peptides and Proteins; 0 / MEN1 protein, human; 0 / Men1 protein, mouse; 0 / Proto-Oncogene Proteins; 0 / Tumor Suppressor Proteins; 0 / aryl hydrocarbon receptor-interacting protein; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27
  • [Number-of-references] 54
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15. Lemos MC, Thakker RV: Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene. Hum Mutat; 2008 Jan;29(1):22-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene.
  • Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of tumors of the parathyroids, pancreas, and anterior pituitary.
  • The MEN1 gene, which was identified in 1997, consists of 10 exons that encode a 610-amino acid protein referred to as menin.
  • Germline mutations usually result in MEN1 or occasionally in an allelic variant referred to as familial isolated hyperparathyroidism (FIHP).
  • MEN1 tumors frequently have loss of heterozygosity (LOH) of the MEN1 locus, which is consistent with a tumor suppressor role of MEN1.
  • Furthermore, somatic abnormalities of MEN1 have been reported in MEN1 and non-MEN1 endocrine tumors.
  • The clinical aspects and molecular genetics of MEN1 are reviewed together with the reported 1,336 mutations.
  • The mutations are scattered throughout the>9-kb genomic sequence of the MEN1 gene.
  • The majority of MEN1 mutations are likely to disrupt the interactions of menin with other proteins and thereby alter critical events in cell cycle regulation and proliferation.
  • [MeSH-major] Germ-Line Mutation. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Animals. Endocrine Gland Neoplasms / genetics. Gene Expression Regulation, Neoplastic. Humans. Mice. Models, Animal. Pancreatic Neoplasms / genetics. Parathyroid Neoplasms / genetics. Pituitary Neoplasms / genetics


16. Stålberg P, Santesson M, Ekeblad S, Lejonklou MH, Skogseid B: Recognizing genes differentially regulated in vitro by the multiple endocrine neoplasia type 1 (MEN1) gene, using RNA interference and oligonucleotide microarrays. Surgery; 2006 Dec;140(6):921-9; discussion 929-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recognizing genes differentially regulated in vitro by the multiple endocrine neoplasia type 1 (MEN1) gene, using RNA interference and oligonucleotide microarrays.
  • BACKGROUND: Data on downstream effects of MEN1 gene inactivation is scarce.
  • In an effort to identify genes regulated by MEN1, we designed a silencing experiment in a human endocrine pancreatic tumor cell line (BON1).
  • METHODS: By using RNA interference, MEN1 mRNA expression was knocked-down by >85%.
  • We also investigated if genes were differentially expressed in 6 malignant endocrine pancreatic tumors (EPTs) with homozygous MEN1 inactivation compared to 2 without MEN1 gene alterations.
  • RESULTS: Using a cut-off of > or =2 times, 66 genes were found to be upregulated, and 22 were downregulated in the MEN1-silenced clones.
  • Genes involved in endocrine cell fate determination, as well as genes known to be involved in NFkappaB, Notch, and Wnt signaling pathways, were among genes verified as differentially regulated in vitro.
  • CONCLUSIONS: The demonstration of pathways affected by silencing of MEN1 in vitro provides novel insight into neoplastic processes of potential importance in vivo, which warrants further study.
  • [MeSH-minor] Cell Line, Tumor. DNA, Neoplasm / genetics. Gene Expression Profiling. Humans. NF-kappa B / genetics. NF-kappa B / metabolism. Pancreatic Neoplasms / genetics. Pancreatic Neoplasms / metabolism. Pancreatic Neoplasms / pathology. RNA, Messenger / genetics. RNA, Messenger / metabolism. RNA, Neoplasm / genetics. Receptors, Notch / genetics. Receptors, Notch / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Transfection. Wnt Proteins / genetics. Wnt Proteins / metabolism

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  • [CommentIn] Surgery. 2007 Jun;141(6):830-1 [17560264.001]
  • (PMID = 17188139.001).
  • [ISSN] 0039-6060
  • [Journal-full-title] Surgery
  • [ISO-abbreviation] Surgery
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / MEN1 protein, human; 0 / NF-kappa B; 0 / Proto-Oncogene Proteins; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / Receptors, Notch; 0 / Wnt Proteins
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17. Shah SR, Raghavan R, Desai DC, Chauhan PH, Lala M, Dherai AJ, Ashavaid TF: An Indian family of multiple endocrine neoplasia type 1 (MEN1): molecular diagnosis, treatment and follow up. Indian J Gastroenterol; 2008 Nov-Dec;27(6):242-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] An Indian family of multiple endocrine neoplasia type 1 (MEN1): molecular diagnosis, treatment and follow up.
  • BACKGROUND/OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal, dominant syndrome, characterized mainly by the combination of tumors involving the parathyroid, pancreatic and pituitary glands.
  • METHODS: We performed molecular analysis of the MEN1 gene to identify mutations in an Indian family with MEN1 syndrome.
  • The proband was identified with multiple peptic ulcers because of multifocal recurrent gastrinomas, as well as parathyroid and pituitary adenomas.
  • All the 10 exons of the MEN1 gene were amplified using the polymerase chain reaction (PCR).
  • The MEN1 gene was then screened by direct DNA sequencing.
  • DNA sequencing revealed the presence of a heterozygous Y227X mutation in exon 4 of the MEN1 gene in the proband.
  • Following screening, one asymptomatic child has been identified with and treated for insulinoma and parathyroid adenoma.
  • CONCLUSION: Detection of the MEN1 gene mutation enables selection of family members for screening and long-term follow up.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / diagnosis. Multiple Endocrine Neoplasia Type 1 / genetics

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  • [CommentIn] Indian J Gastroenterol. 2008 Nov-Dec;27(6):225-6 [19405254.001]
  • (PMID = 19405259.001).
  • [ISSN] 0254-8860
  • [Journal-full-title] Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
  • [ISO-abbreviation] Indian J Gastroenterol
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] India
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18. Perren A, Anlauf M, Henopp T, Rudolph T, Schmitt A, Raffel A, Gimm O, Weihe E, Knoefel WT, Dralle H, Heitz PU, Komminoth P, Klöppel G: 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 Mar;92(3):1118-28
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] 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.
  • CONTEXT: 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.
  • To date, it is unknown whether small islet-like endocrine cell clusters found in MEN1 patients are neoplastic or rather hyperplastic.
  • DESIGN: We hypothesized that monohormonal endocrine cell clusters observed in MEN1 patients are small neoplasms with loss of heterozygosity of the MEN1 locus.
  • Using a technique combining fluorescence in situ hybridization of the MEN1 locus and the centromeric region of chromosome 11q with hormone immunostaining, we examined resection specimens from four MEN1 patients.
  • 2) monohormonal endocrine cell clusters;.
  • 3) endocrine and exocrine structures entrapped in microadenomas; and 4) morphologically normal islets.
  • RESULTS: Loss of one MEN1 allele was found in all 27 microadenomas and 19 of 20 (95%) monohormonal endocrine cell clusters.
  • Our results indicate that monohormonal endocrine cell clusters represent a minute form of microadenomas.
  • Islet hyperplasia does not seem to be an obligatory stage in pancreatic MEN1-associated tumor development.
  • [MeSH-major] Carcinoma, Pancreatic Ductal / genetics. Loss of Heterozygosity. Multiple Endocrine Neoplasia Type 1 / genetics. Pancreas / pathology. Pancreatic Neoplasms / genetics. Proto-Oncogene Proteins / genetics


19. Haven CJ, van Puijenbroek M, Tan MH, Teh BT, Fleuren GJ, van Wezel T, Morreau H: Identification of MEN1 and HRPT2 somatic mutations in paraffin-embedded (sporadic) parathyroid carcinomas. Clin Endocrinol (Oxf); 2007 Sep;67(3):370-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Identification of MEN1 and HRPT2 somatic mutations in paraffin-embedded (sporadic) parathyroid carcinomas.
  • Although MEN1 is not typically thought to be involved in carcinoma formation, parathyroid carcinoma may be an extremely rare feature of the multiple endocrine neoplasia type 1 (MEN1) syndrome.
  • We recently concluded that loss of heterozygosity (LOH) of the MEN1 gene is present in a relatively large number of parathyroid carcinomas, often in combination with LOH at the HRPT2 locus.
  • The aim of this study was to evaluate the role of MEN1 and HRPT2 mutations in sporadic parathyroid tumours fulfilling histological criteria for malignancy.
  • HRPT2 (27/28 cases) and MEN1 (23/28 cases) were analysed by direct sequencing.
  • RESULTS: Somatic MEN1 mutations were found in three of 23 (13%) sporadic parathyroid carcinoma cases; these consisted of one missense and two frameshift mutations.
  • CONCLUSIONS: These results suggest that not only HRPT2 but also MEN1 mutations may play a role in sporadic parathyroid cancer formation.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Parathyroid Neoplasms / genetics. Proto-Oncogene Proteins / genetics. Tumor Suppressor Proteins / genetics

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  • (PMID = 17555500.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / CDC73 protein, human; 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 0 / Tumor Suppressor Proteins
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20. Toledo RA, Lourenço DM Jr, Coutinho FL, Quedas E, Mackowiack I, Machado MC, Montenegro F, Cunha-Neto MB, Liberman B, Pereira MA, Correa PH, Toledo SP: Novel MEN1 germline mutations in Brazilian families with multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf); 2007 Sep;67(3):377-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Novel MEN1 germline mutations in Brazilian families with multiple endocrine neoplasia type 1.
  • OBJECTIVE: To characterize clinical features and identify MEN1 germline mutations in Brazilian families with multiple endocrine neoplasia type 1 (MEN1).
  • PATIENTS: Fourteen Brazilian families with MEN1 and 141 at-risk relatives.
  • RESULTS: We identified 12 different MEN1 disease-causing mutations, seven of them previously unreported: 308delC; 375del21; 549A>T (I147F); 1243delA; 1348T>G (L413R); 1351T>C (L414P) and 1523G>T (W471C).
  • Most of the MEN1 single point mutations involved evolutionarily conserved residues, whereas most of the deletion/frameshift changes occurred in GC-rich repetitive regions.
  • Genetic screening of 141 at-risk family members identified 38 MEN1 mutation carriers, 37 (97.4%) of whom had at least one major MEN1-related tumour upon clinical investigation.
  • CONCLUSIONS: High frequencies of MEN1 gene mutations were detected in Brazilian families with MEN1, including seven new genetic mutations that are predicted to cause inactivation of the MEN1 tumour suppressor gene.
  • Our data underscore the need to implement a systematic MEN1 screening programme in Brazil.
  • [MeSH-major] Germ-Line Mutation. Multiple Endocrine Neoplasia Type 1 / epidemiology. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / genetics

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  • (PMID = 17555499.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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21. Moyes VJ, Monson JP, Chew SL, Akker SA: Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism. Int J Endocrinol; 2010;2010:906163
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical Use of Cinacalcet in MEN1 Hyperparathyroidism.
  • Background. Management of multiple-endocrine neoplasia type 1- (MEN1-) associated hyperparathyroidism is associated with high recurrence rates and high surgical morbidity due to multiple neck explorations.
  • Cinacalcet, a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism and parathyroid carcinoma, may provide a medical alternative for the management of these complex patients.
  • Methods. A prospective audit was performed of eight patients; three males and five females, aged 20-38 at diagnosis.
  • All were commenced on cinacalcet 30 mg bd for MEN1 associated hyperparathyroidism; doses were subsequently reduced to 30 mg od in four patients.
  • Conclusion. Cinacalcet is an effective and well-tolerated medical treatment for the management of complex primary hyperparathyroidism.

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  • [Cites] Arch Surg. 1999 Oct;134(10):1119-23 [10522858.001]
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  • [Cites] J Clin Endocrinol Metab. 2007 Oct;92(10):3803-8 [17666472.001]
  • (PMID = 20585352.001).
  • [ISSN] 1687-8345
  • [Journal-full-title] International journal of endocrinology
  • [ISO-abbreviation] Int J Endocrinol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2877200
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22. Falchetti A, Marini F, Luzi E, Giusti F, Cavalli L, Cavalli T, Brandi ML: Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors. Genet Med; 2009 Dec;11(12):825-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors.
  • MEN 1 is a rare hereditary cancer syndrome which manifests a variety of endocrine and non-endocrine neoplasms and lesions.
  • Growing knowledge of this condition in both its molecular genetic underpinnings and its clinical implications have affected the entire spectrum of the clinical management of MEN patients.
  • The MEN1 gene is a tumor suppressor gene, and mutations in it account for the development of the MEN1 clinical syndrome through impairment of several cell functions, such as cell proliferations, cell growth control, apoptosis, DNA replication and repair, gene expression, transcriptional machinery control, and hormone secretion.
  • The ever increrasing combination of genetic and clinical tools will allow early detection of MEN1-associated neoplasms, potentially improving clinical outcomes and quality of life for both affected patients and their relatives.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Multiple Endocrine Neoplasia Type 1 / pathology. Mutation. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Gastrinoma / complications. Genotype. Humans. Insulinoma / complications. Parathyroid Neoplasms / complications. Phenotype. Pituitary Neoplasms / complications

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  • (PMID = 19904212.001).
  • [ISSN] 1530-0366
  • [Journal-full-title] Genetics in medicine : official journal of the American College of Medical Genetics
  • [ISO-abbreviation] Genet. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
  • [Number-of-references] 150
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23. Dreijerink KM, van Beek AP, Lentjes EG, Post JG, van der Luijt RB, Canninga-van Dijk MR, Lips CJ: Acromegaly in a multiple endocrine neoplasia type 1 (MEN1) family with low penetrance of the disease. Eur J Endocrinol; 2005 Dec;153(6):741-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Acromegaly in a multiple endocrine neoplasia type 1 (MEN1) family with low penetrance of the disease.
  • Multiple endocrine neoplasia type 1 (MEN1) is an inherited syndrome that is characterised by the occurrence of tumours in the parathyroid glands, the endocrine pancreas, the pituitary gland and the adrenal glands and by neuroendocrine carcinoid tumours, often at a young age.
  • The penetrance of MEN1 among gene carriers is reported to be high; 82-99% at age 50.
  • He turned out to be a carrier of a MEN1 germ-line mutation in intron 3 (IVS3-6C > G).
  • However, none of these relatives have developed any MEN1-related lesion yet, although several are older than 60 years.
  • To our knowledge, a MEN1 family with as few clinical features as this family has not been reported to date.
  • Because MEN1 patients have an increased risk of developing acromegaly, insulin-like growth factor (IGF-I) levels are monitored periodically.
  • We investigated whether IGF-I levels might serve as a presymptomatic marker for acromegaly; 9% (3/33) of MEN1 patients showed temporary IGF-I elevations.
  • Possibly, acromegaly in MEN1 is preceded by a transient preacromegalic state.


24. Turner JJ, Christie PT, Pearce SH, Turnpenny PD, Thakker RV: Diagnostic challenges due to phenocopies: lessons from Multiple Endocrine Neoplasia type1 (MEN1). Hum Mutat; 2010 Jan;31(1):E1089-101
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Diagnostic challenges due to phenocopies: lessons from Multiple Endocrine Neoplasia type1 (MEN1).
  • We report phenocopies in Multiple Endocrine Neoplasia type 1 (MEN1), an autosomal dominant disorder, characterised by the combined occurrence of parathyroid, pituitary and pancreatic tumours.
  • We studied 261 affected individuals from 74 families referred with a clinical diagnosis of MEN1 and sought inconsistencies between the mutational and clinical data.
  • Patients 1 and 2 from families with MEN1, developed prolactinomas as the sole endocrinopathy but they did not harbour the germline MEN1 mutation present in their affected relatives.
  • Patients 3 and 4 and their relatives did not have MEN1 mutations, but instead had familial hypocalciuric hypercalcaemia (FHH) due to a calcium-sensing receptor mutation (p.Arg680Cys), and the hyperparathyroidism-jaw tumour (HPT-JT) syndrome due to a hyperparathyroidism type 2 deletional-frameshift mutation (c.1239delA), respectively.
  • Phenocopies may mimic MEN1 either by occurrence of a single sporadic endocrine tumour in a patient with familial MEN1, or occurrence of two endocrine abnormalities associated with different aetiologies.
  • Phenocopies arose in >5% of MEN1 families, and awareness of them is important in the clinical management of MEN1 and other hereditary disorders.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1
  • [MeSH-minor] Adult. Female. Humans. Hypercalcemia / diagnosis. Hypercalcemia / genetics. Hyperparathyroidism / diagnosis. Hyperparathyroidism / genetics. Male. Middle Aged. Mutation. Pancreatic Neoplasms / diagnosis. Pancreatic Neoplasms / genetics. Parathyroid Neoplasms / diagnosis. Parathyroid Neoplasms / genetics. Phenotype. Proto-Oncogene Proteins / genetics. Receptors, Calcium-Sensing / genetics. Sequence Analysis, DNA. Tumor Suppressor Proteins / genetics

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  • (PMID = 19953642.001).
  • [ISSN] 1098-1004
  • [Journal-full-title] Human mutation
  • [ISO-abbreviation] Hum. Mutat.
  • [Language] eng
  • [Grant] United Kingdom / Medical Research Council / / G9825289
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CDC73 protein, human; 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 0 / Receptors, Calcium-Sensing; 0 / Tumor Suppressor Proteins
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25. Lemos MC, Harding B, Reed AA, Jeyabalan J, Walls GV, Bowl MR, Sharpe J, Wedden S, Moss JE, Ross A, Davidson D, Thakker RV: Genetic background influences embryonic lethality and the occurrence of neural tube defects in Men1 null mice: relevance to genetic modifiers. J Endocrinol; 2009 Oct;203(1):133-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Genetic background influences embryonic lethality and the occurrence of neural tube defects in Men1 null mice: relevance to genetic modifiers.
  • Germline mutations of the multiple endocrine neoplasia type 1 (MEN1) gene cause parathyroid, pancreatic and pituitary tumours in man.
  • MEN1 mutations also cause familial isolated primary hyperparathyroidism (FIHP) and the same MEN1 mutations, in different families, can cause either FIHP or MEN1.
  • We investigated the effects of genetic background on the phenotype of embryonic lethality that occurs in a mouse model for MEN1.
  • Men1(+/-) mice were backcrossed to generate C57BL/6 and 129S6/SvEv incipient congenic strains, and used to obtain homozygous Men1(-/-) mice.
  • No viable Men1(-/-) mice were obtained.
  • Moreover, live Men1(-/-) embryos were absent by 13.5 and 15.5 dpc in the 129S6/SvEv and C57BL/6 strains respectively thereby indicating an earlier lethality by 2 days in the 129S6/SvEv strain (P<0.01).
  • Men1(-/-) embryos had macroscopic haemorrhages, and histology and optical projection tomography revealed them to have internal haemorrhages, myocardial hypotrophy, pericardial effusion, hepatic abnormalities and neural tube defects.
  • Thus, our findings demonstrate the importance of genetic background in influencing the phenotypes of embryonic lethality and neural tube defects in Men1(-/-) mice, and implicate a role for genetic modifiers.

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  • (PMID = 19587266.001).
  • [ISSN] 1479-6805
  • [Journal-full-title] The Journal of endocrinology
  • [ISO-abbreviation] J. Endocrinol.
  • [Language] eng
  • [Grant] United Kingdom / Department of Health / / DH/ 913; United Kingdom / Medical Research Council / / G9825289, 2004; United Kingdom / Medical Research Council / / MRC/ G9825289; United Kingdom / Medical Research Council / / MRC/ MC/ U127527203; United Kingdom / Multiple Sclerosis Society / / MSS/ 913; United Kingdom / Medical Research Council / / MRC/ G0501780; United Kingdom / Medical Research Council / / MRC/ MC/ U127574434
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Men1 protein, mouse; 0 / Proto-Oncogene Proteins
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26. Yang Y, Wang H, Hua X: Deletion of the Men1 gene prevents streptozotocin-induced hyperglycemia in mice. Exp Diabetes Res; 2010;2010:876701
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Deletion of the Men1 gene prevents streptozotocin-induced hyperglycemia in mice.
  • Here, we report that excision of the Men1 gene, whose loss-of-function mutation leads to inherited multiple endocrine neoplasia type 1 (MEN1), rendered resistant to streptozotocin-induced hyperglycemia in a tamoxifen-inducible and temporally controlled Men1 excision mouse model as well as in a tissue-specific Men1 excision mouse model.
  • Men1 excision prevented mice from streptozotocin-induced hyperglycemia mainly through increasing the number of functional beta cells.
  • BrdU incorporation by beta cells, islet size, and circulating insulin levels were significantly increased in Men1-excised mice.
  • Membrane localization of glucose transporter 2 was largely preserved in Men1-excised beta cells, but not in Men1-expressing beta cells.
  • Our findings suggest that repression of menin, a protein encoded by the Men1 gene, might be a valuable means to maintain or increase the number of functional endogenous beta cells to prevent or ameliorate diabetes.

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  • (PMID = 21318185.001).
  • [ISSN] 1687-5303
  • [Journal-full-title] Experimental diabetes research
  • [ISO-abbreviation] Exp Diabetes Res
  • [Language] ENG
  • [Grant] United States / NIDDK NIH HHS / DK / R01 DK085121; United States / NIDDK NIH HHS / DK / R01-DK085121
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Glucose Transporter Type 2; 0 / Men1 protein, mouse; 0 / Proto-Oncogene Proteins; 0 / Slc2a2 protein, mouse; 5W494URQ81 / Streptozocin
  • [Other-IDs] NLM/ PMC3034935
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27. Tsukada T, Nagamura Y, Ohkura N: MEN1 gene and its mutations: basic and clinical implications. Cancer Sci; 2009 Feb;100(2):209-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MEN1 gene and its mutations: basic and clinical implications.
  • Heterozygous germline mutations of the tumor-suppressor gene MEN1 are responsible for multiple endocrine neoplasia type 1 (MEN1), a dominantly inherited familial cancer syndrome characterized by pituitary, parathyroid, and enteropancreatic tumors.
  • Various mutations have been identified throughout the entire gene region in patients with MEN1 and related disorders.
  • Neither mutation hot spot nor phenotype–genotype correlation has been established in MEN1 although some missense mutations may be specifically associated with a phenotype of familial isolated hyperparathyroidism.
  • The gene product menin has been implicated in multiple roles, including gene transcription, maintenance of genomic integrity, and control of cell division and differentiation.
  • These multiple functions are likely to be conferred by association with multiple protein factors.
  • Occurrence of MEN1-causing missense mutations throughout menin also suggests the requirement of multiple binding factors for its full tumor-suppressive activity.
  • A DNA test for MEN1 germline mutations is a useful tool for diagnosis of MEN1 although it needs further improvements
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Mutation / genetics. Proto-Oncogene Proteins / genetics

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  • (PMID = 19068082.001).
  • [ISSN] 1349-7006
  • [Journal-full-title] Cancer science
  • [ISO-abbreviation] Cancer Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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28. Owens M, Ellard S, Vaidya B: Analysis of gross deletions in the MEN1 gene in patients with multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf); 2008 Mar;68(3):350-4
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  • [Title] Analysis of gross deletions in the MEN1 gene in patients with multiple endocrine neoplasia type 1.
  • BACKGROUND: Mutation analysis with direct DNA sequencing is commonly used for the molecular diagnosis of multiple endocrine neoplasia type 1 (MEN1).
  • However, a significant number of patients, despite clinical features of MEN1, do not show MEN1 mutations on direct DNA sequencing.
  • OBJECTIVE: To determine the prevalence of gross deletions in MEN1 in a large cohort of MEN1 patients.
  • PATIENTS AND METHODS: During 1997-2006, we screened MEN1 mutations by direct DNA sequencing in 368 probands referred to our diagnostic molecular genetic laboratory.
  • Of these, 101 probands (23 familial, 78 sporadic) fulfilled the clinical criteria for MEN1 (presence of at least two of the parathyroid, pancreatic or pituitary tumours) but were negative for mutations on DNA sequencing.
  • Their DNA samples were examined for gross deletions of one or more exons of MEN1 by using multiple ligation-dependent probe amplification (MLPA) and long-range polymerase chain reaction (PCR) amplification.
  • We also sequenced the minimal promoter region of MEN1 for mutations in the familial cases.
  • RESULTS: We identified a gross deletion involving exons 5 and 6 of MEN1 in one proband (prevalence rate 1%).
  • CONCLUSION: Gross deletion in the MEN1 gene is an uncommon cause of MEN1 and should be tested for in patients with a high clinical suspicion but without mutations on direct DNA sequencing.
  • [MeSH-major] Gene Deletion. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / genetics

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  • (PMID = 17854391.001).
  • [ISSN] 1365-2265
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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29. Hannan FM, Nesbit MA, Christie PT, Fratter C, Dudley NE, Sadler GP, Thakker RV: Familial isolated primary hyperparathyroidism caused by mutations of the MEN1 gene. Nat Clin Pract Endocrinol Metab; 2008 Jan;4(1):53-8
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  • [Title] Familial isolated primary hyperparathyroidism caused by mutations of the MEN1 gene.
  • BACKGROUND: Familial isolated primary hyperparathyroidism (FIHP) is an autosomal dominant disorder that can represent an early stage of either the multiple endocrine neoplasia type 1 (MEN1) or hyperparathyroidism-jaw tumor (HPT-JT) syndromes; alternatively, the condition can be caused by an allelic variant of MEN1 or HRPT2 (hyperparathyroidism 2 gene), or caused by a distinct entity involving another locus.
  • INVESTIGATIONS: Serum biochemistry and radiological investigations for primary hyperparathyroidism, MEN1 and HPT-JT, and genetic testing for MEN1 and HRPT2 mutations were undertaken.
  • DIAGNOSIS: FIHP with primary hyperparathyroidism as the sole endocrinopathy due to a previously unreported heterozygous missense germline MEN1 mutation, Tyr351Asn.
  • In addition, another unreported heterozygous missense germline MEN1 mutation, Trp220Leu, was identified in an unrelated male patient with FIHP, whose mother and sister also had primary hyperparathyroidism.
  • This is consistent with Knudson's 'two-hit' model of hereditary cancer and a tumor suppressor role for MEN1 in FIHP.
  • None has developed abnormalities of the MEN1 syndrome, providing further support that FIHP is a distinct genetic variant of the MEN1 syndrome.


30. Jiang XH, Lu JL, Cui B, Zhao YJ, Wang WQ, Liu JM, Fang WQ, Cao YN, Ge Y, Zhang CX, Casse H, Li XY, Ning G: MEN1 mutation analysis in Chinese patients with multiple endocrine neoplasia type 1. Endocr Relat Cancer; 2007 Dec;14(4):1073-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MEN1 mutation analysis in Chinese patients with multiple endocrine neoplasia type 1.
  • 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.
  • Heterozygous germ line mutations of MEN1 gene are responsible for the onset of MEN1.
  • We investigated the probands and 31 family members from eight unrelated Chinese families associated with MEN1 and identified four novel mutations, namely 373_374ins18, 822delT, 259delT and 1092delC, as well as three previously reported mutations, such as 357_360delCTGT, 427_428delTA and R108X (CGA>TGA) of MEN1 gene.
  • Furthermore, we detected a loss of heterozygosity (LOH) at chromosome 11q in the removed tumours, including gastrinoma, insulinoma and parathyroid adenoma from two probands of MEN1 families.
  • RT-PCR and direct sequencing showed that mutant MEN1 transcripts remained in the MEN1-associated endocrine tumours, whereas normal menin proteins could not be detected in those tumours by either immunohistochemistry or immunoblotting.
  • In conclusion, MEN1 heterozygous mutations are associated with LOH and menin absence, which are present in MEN1-associated endocrine tumours.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Mutation. Proto-Oncogene Proteins / genetics


31. Eller-Vainicher C, Chiodini I, Battista C, Viti R, Mascia ML, Massironi S, Peracchi M, D'Agruma L, Minisola S, Corbetta S, Cole DE, Spada A, Scillitani A: Sporadic and MEN1-related primary hyperparathyroidism: differences in clinical expression and severity. J Bone Miner Res; 2009 Aug;24(8):1404-10
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  • [Title] Sporadic and MEN1-related primary hyperparathyroidism: differences in clinical expression and severity.
  • Primary hyperparathyroidism (PHPT) is a common endocrine disease that is associated with multiple endocrine neoplasia type 1 (MEN1) in approximately 2% of PHPT cases.
  • Lack of a family history and other specific expressions may lead to underestimated MEN1 prevalence in PHPT.
  • The aim of this study was to identify clinical or biochemical features predictive of MEN1 and to compare the severity of the disease in MEN1-related versus sporadic PHPT (sPHPT).
  • We performed a 36-mo cross-sectional observational study in three tertiary referral centers on an outpatient basis on 469 consecutive patients with sporadic PHPT and 64 with MEN1-related PHPT.
  • MEN1 patients showed lower BMD Z-scores at the LS (-1.33 +/- 1.23 versus -0.74 +/- 1.4, p = 0.008) and FN (-1.13 +/- 0.96 versus -0.6 +/- 1.07, p = 0.002) and lower phosphate (2.38 +/- 0.52 versus 2.56 +/- 0.45 mg/dl, p = 0.003) and PTH (113.8 +/- 69.5 versus 173.7 +/- 135 pg/ml, p = 0.001) levels than sPHPT patients.
  • Considering probands only, the presence of MEN1 was more frequently associated with PTH values in the normal range (OR, 3.01; 95% CI, 1.07-8.50; p = 0.037) and younger age (OR, 1.61; 95% CI, 1.28-2.02; p = 0.0001).
  • A combination of PTH values in the normal range plus age <50 yr was strongly associated with MEN1 presence (OR, 13.51; 95% CI, 3.62-50.00; p = 0.0001).
  • In conclusion, MEN1-related PHPT patients show more severe bone but similar kidney involvement despite a milder biochemical presentation compared with their sPHPT counterparts.
  • Normal PTH levels and young age are associated with MEN1 presence.

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  • (PMID = 19309299.001).
  • [ISSN] 1523-4681
  • [Journal-full-title] Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
  • [ISO-abbreviation] J. Bone Miner. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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32. Schnepp RW, Chen YX, Wang H, Cash T, Silva A, Diehl JA, Brown E, Hua X: Mutation of tumor suppressor gene Men1 acutely enhances proliferation of pancreatic islet cells. Cancer Res; 2006 Jun 1;66(11):5707-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mutation of tumor suppressor gene Men1 acutely enhances proliferation of pancreatic islet cells.
  • Multiple endocrine neoplasia type 1 (MEN1), an inherited tumor syndrome affecting endocrine organs including pancreatic islets, results from mutation of the tumor suppressor gene Men1 that encodes protein menin.
  • Although menin is known to be involved in regulating cell proliferation in vitro, it is not clear how menin regulates cell cycle and whether mutation of Men1 acutely promotes pancreatic islet cell proliferation in vivo.
  • Here we show that excision of the floxed Men1 in mouse embryonic fibroblasts (MEF) accelerates G(0)/G(1) to S phase entry.
  • Moreover, Men1 excision results in decreased expression of p18(Ink4c) and p27(Kip1) in the pancreas.
  • Furthermore, complementation of menin-null cells with wild-type menin represses S-phase entry.
  • To extend the role of menin in repressing cell cycle in cultured cells to in vivo pancreatic islets, we generated a system in which floxed Men1 alleles can be excised in a temporally controllable manner.
  • As early as 7 days following Men1 excision, pancreatic islet cells display increased proliferation, leading to detectable enlargement of pancreatic islets 14 days after Men1 excision.
  • These observations are consistent with the notion that an acute effect of Men1 mutation is accelerated S-phase entry and enhanced cell proliferation in pancreatic islets.
  • Together, these results suggest a molecular mechanism whereby menin suppresses MEN1 tumorigenesis at least partly through repression of G(0)/G(1) to S transition.

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  • (PMID = 16740708.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA113962-02; United States / NCI NIH HHS / CA / CA100912-04; United States / NCI NIH HHS / CA / R01 CA113962; United States / NCI NIH HHS / CA / R01 CA100912; United States / NCI NIH HHS / CA / R01 CA100912-04; United States / NCI NIH HHS / CA / CA113962-02; United States / NCI NIH HHS / CA / CA100912
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cdkn1b protein, mouse; 0 / Cyclin-Dependent Kinase Inhibitor p18; 0 / Men1 protein, mouse; 0 / Proto-Oncogene Proteins; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27; EC 2.7.11.22 / Cyclin-Dependent Kinase 2
  • [Other-IDs] NLM/ NIHMS183850; NLM/ PMC2839933
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33. Choi H, Kim S, Moon JH, Lee YH, Rhee Y, Kang ES, Ahn CW, Cha BS, Lee EJ, Kim KR, Lee HC, Jeong SY, Kim HJ, Lim SK: Multiple endocrine neoplasia type 1 with multiple leiomyomas linked to a novel mutation in the MEN1 gene. Yonsei Med J; 2008 Aug 30;49(4):655-61
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  • [Title] Multiple endocrine neoplasia type 1 with multiple leiomyomas linked to a novel mutation in the MEN1 gene.
  • Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominantly inherited syndrome.
  • MEN1 is characterized by the presence of functioning and nonfunctioning tumors or hyperplasia of the pituitary gland, parathyroid glands, and pancreatic islet cells.
  • In addition, MEN1 carriers can have adrenal or thyroid tumors and non-endocrine tumors, such as lipomas, angiofibromas, and leiomyomas.
  • Although leiomyoma is not a major component of MEN1, it is thought to occur more frequently than expected.
  • However, there has been no report of a case of MEN1 with leiomyoma in Korea so far.
  • This report describes a patient with multiple leiomyomas in MEN1.
  • Sequencing of the MEN1 gene in this patient revealed a novel missense mutation (D350V, exon 7).
  • This is the first case of MEN1 accompanied with multiple leiomyomas, parathyroid adenoma, pituitary adenoma, pancreatic tumor, and adrenal tumor.
  • [MeSH-major] Leiomyomatosis / metabolism. Leiomyomatosis / pathology. Multiple Endocrine Neoplasia Type 1 / metabolism


34. Anlauf M, Perren A, Klöppel G: Endocrine precursor lesions and microadenomas of the duodenum and pancreas with and without MEN1: criteria, molecular concepts and clinical significance. Pathobiology; 2007;74(5):279-84
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  • [Title] Endocrine precursor lesions and microadenomas of the duodenum and pancreas with and without MEN1: criteria, molecular concepts and clinical significance.
  • Proliferative changes in the neuroendocrine cells that precede neoplasia are of interest for the understanding of tumorigenesis and the early recognition of neuroendocrine tumors.
  • 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.
  • The gastrinomas observed in MEN1 are almost exclusively localized in the duodenum and are multicentric.
  • In the pancreas, microadenomatosis (multiple tumors up to 5 mm in diameter) is a feature of MEN1.
  • These microadenomas predominantly express glucagon and pancreatic polypeptide, but do not cause a hormonal syndrome.
  • Approximately 50% of MEN1 minigastrinomas in the duodenum and almost all microadenomas in the pancreas show allelic deletion of the MEN1 gene and therefore may represent 'initial' neoplasms.
  • In contrast, endocrine cell precursor lesions retain heterozygosity.
  • Whether these types of microadenomatosis represent novel disease entities and how to diagnose and treat these patients remains to be clarified by further studies.
  • [MeSH-major] Adenoma / pathology. Duodenal Neoplasms / pathology. Multiple Endocrine Neoplasia Type 1 / genetics. Multiple Endocrine Neoplasia Type 1 / pathology. Pancreatic Neoplasms / pathology. Precancerous Conditions / pathology

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  • [Copyright] Copyright 2007 S. Karger AG, Basel.
  • [CommentIn] Pathobiology. 2007;74(5):277-8 [17890893.001]
  • (PMID = 17890894.001).
  • [ISSN] 1423-0291
  • [Journal-full-title] Pathobiology : journal of immunopathology, molecular and cellular biology
  • [ISO-abbreviation] Pathobiology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 24
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35. Bazzi W, Renon M, Vercherat C, Hamze Z, Lacheretz-Bernigaud A, Wang H, Blanc M, Roche C, Calender A, Chayvialle JA, Scoazec JY, Cordier-Bussat M: MEN1 missense mutations impair sensitization to apoptosis induced by wild-type menin in endocrine pancreatic tumor cells. Gastroenterology; 2008 Nov;135(5):1698-1709.e2
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  • [Title] MEN1 missense mutations impair sensitization to apoptosis induced by wild-type menin in endocrine pancreatic tumor cells.
  • BACKGROUND & AIMS: Missense mutations account for 30% of mutations identified in patients with the multiple endocrine neoplasia type 1 (MEN1) syndrome.
  • We aimed to evaluate the functional consequences of missense MEN1 mutations in an appropriate endocrine cellular context.
  • METHODS: From the INS-1 insulinoma cell line, we established clones conditionally over expressing wild-type (WT) menin or its A160T, H317Y, and A541T variants.
  • CONCLUSIONS: Taking advantage of a new endocrine cellular model, we show a loss of function for 2 missense disease-related menin mutants and for a controversial variant as well.
  • Furthermore, our results suggest the existence of functional interactions between p53 and menin for the control of apoptosis, which may cast new light on the mechanisms of endocrine tumorigenesis.
  • [MeSH-major] Apoptosis / drug effects. DNA, Neoplasm / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Mutation, Missense. Pancreatic Neoplasms / genetics. Proto-Oncogene Proteins / genetics


36. Fontanière S, Casse H, Bertolino P, Zhang CX: Analysis of p27(Kip1) expression in insulinomas developed in pancreatic beta-cell specific Men1 mutant mice. Fam Cancer; 2006;5(1):49-54
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  • [Title] Analysis of p27(Kip1) expression in insulinomas developed in pancreatic beta-cell specific Men1 mutant mice.
  • Multiple Endocrine Neoplasia type 1 (MEN1) is a hereditary disease characterised by the occurrence of multiple endocrine tumours.
  • The biological functions of the responsible gene, MEN1, and its encoded protein, menin, remain so far largely elusive.
  • The recent generation of Men1 mutant mice by our group and other laboratories provides powerful tools allowing for the identification of cellular and molecular events that occur after gene disruption.
  • Interestingly, it has been recently reported that p27(Kip1) expression is regulated by menin and that decreased p27(Kip1) expression can be found in MEN1 insulinomas and parathyroid adenomas.
  • In order to address whether and when p27(Kip1) expression alters during insulinoma development in pancreatic beta-cell-specific Men1 mutant mice, we analysed p27(Kip1) expression in islet lesions from mutant mice at different ages.
  • Our work confirms the altered p27(Kip1) expression reported in tumours from MEN1 patients, whereas it suggests that other molecular events may also participate in the tumorigenesis process initiated by the Men1 gene inactivation.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p27 / genetics. Gene Expression Regulation, Neoplastic. Insulinoma / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Pancreatic Neoplasms / genetics
  • [MeSH-minor] Animals. Blotting, Western. Cell Survival. Disease Models, Animal. Down-Regulation. Gene Deletion. Immunohistochemistry. Islets of Langerhans / cytology. Mice. Mice, Mutant Strains. Probability. Proto-Oncogene Proteins / genetics. Sensitivity and Specificity. Tumor Cells, Cultured


37. Shen HC, He M, Powell A, Adem A, Lorang D, Heller C, Grover AC, Ylaya K, Hewitt SM, Marx SJ, Spiegel AM, Libutti SK: Recapitulation of pancreatic neuroendocrine tumors in human multiple endocrine neoplasia type I syndrome via Pdx1-directed inactivation of Men1. Cancer Res; 2009 Mar 1;69(5):1858-66
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  • [Title] Recapitulation of pancreatic neuroendocrine tumors in human multiple endocrine neoplasia type I syndrome via Pdx1-directed inactivation of Men1.
  • Multiple endocrine neoplasia type 1 (MEN1) is an autosomal syndrome caused by mutations in the MEN1 tumor suppressor gene.
  • Whereas the protein product of MEN1, menin, is ubiquitously expressed, somatic loss of the remaining wild-type MEN1 allele results in tumors primarily in parathyroid, pituitary, and endocrine pancreas.
  • To understand the endocrine specificity of the MEN1 syndrome, we evaluated biallelic loss of Men1 by inactivating Men1 in pancreatic progenitor cells using the Cre-lox system.
  • Men1 deletion in progenitor cells that differentiate into exocrine and endocrine pancreas did not affect normal pancreas morphogenesis and development.
  • However, mice having homozygous inactivation of the Men1 in pancreas developed endocrine tumors with no exocrine tumor manifestation, recapitulating phenotypes seen in the MEN1 patients.
  • In the absence of menin, the endocrine pancreas showed increase in cell proliferation, vascularity, and abnormal vascular structures; such changes were lacking in exocrine pancreas.
  • Further analysis revealed that these endocrine manifestations were associated with up-regulation in vascular endothelial growth factor expression in both human and mouse MEN1 pancreatic endocrine tumors.
  • Together, these data suggest the presence of cell-specific factors for menin and a permissive endocrine environment for MEN1 tumorigenesis in endocrine pancreas.
  • Based on our analysis, we propose that menin's ability to maintain cellular and microenvironment integrity might explain the endocrine- restrictive nature of the MEN1 syndrome.
  • [MeSH-major] Homeodomain Proteins / physiology. Multiple Endocrine Neoplasia Type 1 / etiology. Neuroendocrine Tumors / etiology. Pancreatic Neoplasms / etiology. Proto-Oncogene Proteins / physiology. Trans-Activators / physiology


38. Peppa M, Boutati E, Kamakari S, Pikounis V, Peros G, Koutsodontis G, Metaxa-Mariatou V, Economopoulos T, Raptis SA, Hadjidakis D: Novel germline mutations of the MEN1 gene in Greek families with multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf); 2009 Jan;70(1):75-81
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  • [Title] Novel germline mutations of the MEN1 gene in Greek families with multiple endocrine neoplasia type 1.
  • INTRODUCTION: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary disorder associated with mutations of the MEN1 gene and characterized by the combined occurrence of tumours of the parathyroid glands, the pancreatic islet cells and the anterior pituitary.
  • AIM: To identify MEN1 gene mutations and characterize clinical manifestations in Greek patients with MEN1.
  • PATIENTS AND METHODS: We studied four unrelated index patients with MEN1, 17 relatives and 100 control subjects.
  • DNA extraction, polymerase chain reaction (PCR) and direct sequencing of the MEN1 exons 2-10 and exon/intron boundaries were performed according to standard procedures.
  • RESULTS: We identified novel MEN1 gene mutations in three out of four index patients (75%) and in all affected (100%) relatives.
  • CONCLUSIONS: This is the first report to reveal a high prevalence of novel MEN1 gene mutations among Greek MEN1 patients with apparent absence of genotype-phenotype correlation.
  • [MeSH-major] Germ-Line Mutation. Multiple Endocrine Neoplasia Type 1 / genetics


39. Balogh K, Hunyady L, Patocs A, Gergics P, Valkusz Z, Toth M, Racz K: MEN1 gene mutations in Hungarian patients with multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf); 2007 Nov;67(5):727-34
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  • [Title] MEN1 gene mutations in Hungarian patients with multiple endocrine neoplasia type 1.
  • OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary disorder associated with mutations of the MEN1 gene.
  • MEN1 may present as a familial or a sporadic disorder, with multiple endocrine tumours including parathyroid adenomas or hyperplasias, and pancreatic endocrine and pituitary gland tumours.
  • The aim of this study was to examine the prevalence and spectrum of MEN1 gene mutations in Hungarian patients with familial and sporadic MEN1 and in those with a MEN1-related state.
  • DESIGN: Mutation analysis, using temporal temperature gradient gel electrophoresis and direct sequencing of all coding exons and the corresponding exon-intron boundaries of the MEN1 gene, was performed.
  • PATIENTS AND MEASUREMENTS: Peripheral blood DNA was obtained from 32 patients (19 index patients with familial or sporadic MEN1 and 13 index patients with familial or sporadic MEN1-related state).
  • RESULTS: Ten different MEN1 gene mutations were identified in 10 index patients, including four novel mutations (A91V, G28A and E26X all in exon 2, and L301R in exon 6).
  • All but one mutation occurred in index patients with familial or sporadic MEN1; the prevalence of mutation was considerably higher in index patients with familial MEN1 (6/6 patients, 100%) than in those with sporadic MEN1 (3/13 patients, 23%).
  • Of the 13 index patients with a MEN1-related state, only one patient with recurrent isolated primary hyperparathyroidism had a MEN1 gene mutation.
  • CONCLUSION: These results confirm previous reports on the high prevalence of novel MEN1 gene mutations among patients with MEN1, and support the questionable efficacy of mutation screening in patients with sporadic MEN1-related states.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Mutation, Missense


40. Sun HY, Cui B, Su DW, Jin XL, Sun FK, Zu Y, Jiang L, Wang WQ, Ning G: LOH on chromosome 11q, but not SDHD and Men1 mutations was frequently detectable in Chinese patients with pheochromocytoma and paraganglioma. Endocrine; 2006 Dec;30(3):307-12
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  • [Title] LOH on chromosome 11q, but not SDHD and Men1 mutations was frequently detectable in Chinese patients with pheochromocytoma and paraganglioma.
  • The tumor-suppressor gene Men1 located in 11q13 is responsible for multiple endocrine neoplasia type 1 (Men1).
  • However, the involvement of the Men1 gene in tumorigenesis of sporadic PCC/PGL is yet to be determined.
  • To understand the roles of the two tumor-suppressor genes and LOH on chromosome 11q in Chinese patients with sporadic PCC or PGL, we performed mutation detection of the SDHD and Men1 genes in tumors from 35 Chinese patients with PCC/PGL; we also did LOH analysis at chromosome 11q for 25 patients out of the 35.
  • Our results suggested that mutation in SDHD or Men1 gene was not found in Chinese patients with sporadic PCC/PGL.

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  • (PMID = 17526943.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 0 / SDHD protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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41. Odou MF, Cardot-Bauters C, Vantyghem MC, Carnaille B, Leteurtre E, Pigny P, Verier-Mine O, Desailloud R, Porchet N: Contribution of genetic analysis in screening for MEN1 among patients with sporadic disease and one or more typical manifestation. Ann Endocrinol (Paris); 2006 Dec;67(6):581-7
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  • [Title] Contribution of genetic analysis in screening for MEN1 among patients with sporadic disease and one or more typical manifestation.
  • Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal dominant hereditary syndrome (OMIM 131100) due to MEN1 gene mutations, predisposing to the development of hyperplasic and tumoral lesions of neuroendocrine tissues.
  • Since the identification of the gene in 1997, more than 400 different mutations of MEN1 have been registered.
  • Genotypic analysis of MEN1 remains fastidious and must be reserved to targeted situations.
  • If the lesions appear in a familial assessed context, there is a strong argument to search for MEN1 mutation.
  • With experience acquired in our laboratory, we evaluated the frequency of MEN1 mutations in patients with sporadic presentations.
  • Our aim was to better define criteria for MEN1 genotypic analysis.
  • These patients exhibited 1 to 4 manifestations of MEN1 without any familial context.
  • After DNA extraction, the analysis was undertaken by PCR-sequencing of all the MEN1 coding exons and exon/intron boundaries or by PCR of the pre-screened fragments alone, a technique made possible by indirect screening mutation methods.
  • Mutations were identified by comparing the sequences to the reference MEN1 sequence available from GENBANK (U93237.1).
  • We showed a relationship between clinical presentation and MEN1 mutation identification, especially with the number of clinical manifestations but also with the type of manifestation.
  • In a sporadic context with at least two established manifestations of MEN1, the overall probability of identifying a mutation was 26%, warranting MEN1 genotypic analysis.
  • [MeSH-major] Chromosomes, Human, Pair 11. Genetic Testing. Multiple Endocrine Neoplasia Type 1 / genetics
  • [MeSH-minor] Adult. DNA / blood. DNA / genetics. DNA / isolation & purification. Diagnosis, Differential. Gene Frequency. Humans. Middle Aged. Molecular Sequence Data. Mutation

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  • (PMID = 17194968.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] eng
  • [Databank-accession-numbers] GENBANK/ U93237; OMIM/ 131100
  • [Publication-type] Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 9007-49-2 / DNA
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42. Dilley WG, Kalyanaraman S, Verma S, Cobb JP, Laramie JM, Lairmore TC: Global gene expression in neuroendocrine tumors from patients with the MEN1 syndrome. Mol Cancer; 2005 Feb 03;4(1):9
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  • [Title] Global gene expression in neuroendocrine tumors from patients with the MEN1 syndrome.
  • BACKGROUND: Multiple Endocrine Neoplasia type 1 (MEN1, OMIM 131100) is an autosomal dominant disorder characterized by endocrine tumors of the parathyroids, pancreatic islets and pituitary.
  • The disease is caused by the functional loss of the tumor suppressor protein menin, coded by the MEN1 gene.
  • However, none of these binding studies have led to a convincing theory of how loss-of-menin leads to neoplasia.
  • RESULTS: Global gene expression studies on eight neuroendocrine tumors from MEN1 patients and 4 normal islet controls was performed utilizing Affymetrix U95Av2 chips.
  • Within the group of tumors, those of the same type were mostly clustered together.
  • CONCLUSION: This is the first analysis of global gene expression in MEN1-associated neuroendocrine tumors.
  • Many genes were identified which were differentially expressed in neuroendocrine tumors arising in patients with the MEN1 syndrome, as compared with normal human islet cells.
  • The expression of a group of apoptosis-related genes was significantly suppressed, suggesting that these genes may play crucial roles in tumorigenesis in this syndrome.

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  • (PMID = 15691381.001).
  • [ISSN] 1476-4598
  • [Journal-full-title] Molecular cancer
  • [ISO-abbreviation] Mol. Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P30 CA091842; United States / NCI NIH HHS / CA / P30 CA91842
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Hormones
  • [Other-IDs] NLM/ PMC549185
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43. Ellard S, Hattersley AT, Brewer CM, Vaidya B: Detection of an MEN1 gene mutation depends on clinical features and supports current referral criteria for diagnostic molecular genetic testing. Clin Endocrinol (Oxf); 2005 Feb;62(2):169-75
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  • [Title] Detection of an MEN1 gene mutation depends on clinical features and supports current referral criteria for diagnostic molecular genetic testing.
  • OBJECTIVE: Diagnostic molecular genetic testing for multiple endocrine neoplasia type 1 (MEN1) has been available since the identification of the MEN1 gene in 1997.
  • Mutation screening of the MEN1 gene has been recommended for patients who meet clinical criteria for MEN1 (at least two of the following: parathyroid hyperplasia, pancreatic endocrine tumour or pituitary adenoma) and those in whom a diagnosis of MEN1 is suspected.
  • The coding region of the MEN1 gene was sequenced in 186 index cases and mutation testing was requested for 106 subjects, including 83 asymptomatic relatives.
  • RESULTS: MEN1 gene mutations were identified in 68/186 index cases (37%).
  • Twenty-nine of the 60 MEN1 mutations reported are novel.
  • The likelihood of finding a mutation was correlated with the number of MEN1-related tumours (mutation detection rate of 79%, 37% and 15% in patients with three, two and one main MEN1-related tumours; P < or = 0.00001) and increased in the presence of a family history (mutation detection rate of 91%, 69% and 29%vs. 69%, 23% and 0% in sporadic cases with three, two or one main MEN1-related tumours, respectively; P < or = 0.00001).
  • CONCLUSIONS: The likelihood of finding an MEN1 mutation depends on the clinical features of the patient and their family.
  • This large series supports present referral criteria for diagnostic mutation screening, but suggests that patients with sporadic isolated tumours rarely have MEN1 mutations.
  • [MeSH-major] Genetic Testing. Multiple Endocrine Neoplasia Type 1 / genetics. Patient Selection. Proto-Oncogene Proteins / genetics

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  • (PMID = 15670192.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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44. Hussein N, Casse H, Fontanière S, Morera AM, Asensio MJ, Bakeli S, Lu JL, Coste I, Di Clemente N, Bertolino P, Zhang CX: Reconstituted expression of menin in Men1-deficient mouse Leydig tumour cells induces cell cycle arrest and apoptosis. Eur J Cancer; 2007 Jan;43(2):402-14
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  • [Title] Reconstituted expression of menin in Men1-deficient mouse Leydig tumour cells induces cell cycle arrest and apoptosis.
  • Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome caused by the inactivation of the responsible gene, MEN1.
  • To date, the lack of MEN1-deficient cell lines derived directly from MEN1 tumours has hampered the detailed study of the MEN1 gene.
  • We have established several stable Men1-deficient Leydig cell tumour (LCT) lines derived from a Leydig cell tumour developed in a male heterozygous Men1 mutant mouse.
  • Interestingly, reconstituted menin expression in one of Men1-deficient LCT cell lines resulted in cell growth inhibition, suggesting that the function of cell growth suppression of the menin pathway, apart from menin itself, is essentially preserved in these cells.
  • Furthermore, we show that menin re-expression in these Men1-deficient cells leads to a block in the transition from G0/G1 to S phase of the cell cycle and an increase in apoptosis, accompanied by a marked increase of p18INK4C and p27Kip1 expression.
  • The current study therefore highlights the importance of menin expression in cell cycle and cell survival control in endocrine cells, and may provide insights into the mechanisms of tumour suppression by menin in related endocrine tumours.
  • [MeSH-major] Apoptosis / genetics. Cell Cycle / genetics. Leydig Cell Tumor / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Mutation / genetics. Proto-Oncogene Proteins / genetics


45. Snabboon T, Plengpanich W, Siriwong S, Wisedopas N, Suwanwalaikorn S, Khovidhunkit W, Shotelersuk V: A novel germline mutation, 1793delG, of the MEN1 gene underlying multiple endocrine neoplasia type 1. Jpn J Clin Oncol; 2005 May;35(5):280-2
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  • [Title] A novel germline mutation, 1793delG, of the MEN1 gene underlying multiple endocrine neoplasia type 1.
  • They usually occur sporadically; however, their association with multiple endocrine neoplasia type 1 (MEN1) syndrome has been documented.
  • Her family history was unremarkable for any MEN-related lesions.
  • Genetic testing revealed a novel deletion mutation at exon 10 (1793delG) of the MEN1 gene, resulting in a stop codon 26 amino acids downstream.
  • [MeSH-major] Carcinoid Tumor / genetics. Germ-Line Mutation. Lung Neoplasms / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Female. Genes, Tumor Suppressor. Humans. Lymphocytes, Null. Pedigree. Pituitary Neoplasms / genetics


46. Xu L, Li X, Feng B, Ni Y, Wang H, Wang L: A novel mutation of the MEN1 gene in a Chinese kindred with multiple endocrine neoplasia type 1. Endocr J; 2010;57(9):839-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A novel mutation of the MEN1 gene in a Chinese kindred with multiple endocrine neoplasia type 1.
  • Germline mutations in the MEN1 gene are well documented as the genetic cause of multiple endocrine neoplasia type 1 (MEN1).
  • In this study, we performed genetic analysis by direct MEN1 gene mutation analysis on a Chinese MEN1 family.
  • The two patients in this family were diagnosed as MEN1 by the typical clinical findings of parathyroidoma, insulinoma and pituitary adenoma.
  • The coding sequences, including 9 coding exons and exon/intron boundaries of the MEN1 gene were amplified by polymerase chain reaction (PCR) and subjected to direct sequencing.
  • In conclusion, we add a new mutation of MEN1 gene in Chinese patients with MEN1, and it would be useful for the diagnosis of the disease.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics
  • [MeSH-minor] Adenoma / genetics. Adolescent. Asian Continental Ancestry Group / genetics. Base Sequence. China. Codon, Nonsense. Female. Germ-Line Mutation. Humans. Male. Middle Aged. Mutagenesis, Insertional. Mutation. Pancreatic Neoplasms / surgery. Parathyroid Neoplasms / surgery. Pedigree. Pituitary Neoplasms / genetics

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  • (PMID = 20710116.001).
  • [ISSN] 1348-4540
  • [Journal-full-title] Endocrine journal
  • [ISO-abbreviation] Endocr. J.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Codon, Nonsense
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47. Dreijerink KM, Varier RA, van Nuland R, Broekhuizen R, Valk GD, van der Wal JE, Lips CJ, Kummer JA, Timmers HT: Regulation of vitamin D receptor function in MEN1-related parathyroid adenomas. Mol Cell Endocrinol; 2009 Dec 10;313(1-2):1-8
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  • [Title] Regulation of vitamin D receptor function in MEN1-related parathyroid adenomas.
  • Multiple endocrine neoplasia type 1 (MEN1) is a heriditary syndrome characterised by the occurrence of parathyroid, gastroenteropancreatic and pituitary tumours.
  • The MEN1 gene product, menin, co-activates gene transcription by recruiting histone methyltransferases for lysine 4 of histone H3 (H3K4).
  • We investigated whether in MEN1 tumours global changes in H3K4 trimethylation (H3K4me3) occur or whether alterations in gene expression can be observed.
  • By immunohistochemistry we found that global levels of H3K4me3 are not affected in MEN1-related parathyroid adenomas.
  • Messenger RNA levels of VDR target genes CYP24 and KLK6 were significantly lower in MEN1 parathyroid adenomas compared to normal tissue.
  • Thus, aberrant gene expression in MEN1 tumours is not caused by lower global H3K4me3, but rather by specific effects on genes that are regulated by menin-interacting proteins, such as VDR.
  • [MeSH-major] Histones. Lysine / metabolism. Multiple Endocrine Neoplasia Type 1. Parathyroid Neoplasms. Receptors, Calcitriol / metabolism

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  • (PMID = 19729047.001).
  • [ISSN] 1872-8057
  • [Journal-full-title] Molecular and cellular endocrinology
  • [ISO-abbreviation] Mol. Cell. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Histones; 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 0 / Receptors, Calcitriol; K3Z4F929H6 / Lysine
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48. Raghavan R, Shah S, Kondkar AA, Dherai AJ, Desai D, Chauhan P, Lala M, Ashavaid TF: MEN1 935-1G&gt;C splicing mutation in an Indian patient with multiple endocrine neoplasia type 1. Mol Diagn Ther; 2007;11(2):129-31
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  • [Title] MEN1 935-1G>C splicing mutation in an Indian patient with multiple endocrine neoplasia type 1.
  • BACKGROUND AND OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome characterized mainly by multiple tumors involving parathyroid, pancreatic, and pituitary glands.
  • To date, there have been no genetic studies reported on MEN1 in the Indian population.
  • In order to begin to establish molecular diagnosis to improve the management of MEN1 in India, we performed a molecular analysis of the MEN1 gene in a patient of Indian origin.
  • METHODS: Molecular analysis of the MEN1 gene was performed to identify mutations in an Indian patient previously diagnosed with sporadic MEN1.
  • All the 10 exons of the MEN1 gene were amplified using the polymerase chain reaction and screened by direct DNA sequencing.
  • RESULTS: The DNA sequencing results revealed the presence of an intronic, heterozygous, splicing mutation 935-1G>C in intron 5 of the MEN1 gene.
  • CONCLUSION: This study provides the first data on genetic analysis of MEN1 in Indian patients.
  • [MeSH-major] Alternative Splicing / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Point Mutation. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adenoma / complications. Adenoma / genetics. Adult. Base Sequence. DNA Mutational Analysis. Gastrinoma / complications. Gastrinoma / genetics. Humans. India. Pancreatic Neoplasms / complications. Pancreatic Neoplasms / genetics. Pituitary Neoplasms / complications. Pituitary Neoplasms / genetics

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  • (PMID = 17397250.001).
  • [ISSN] 1177-1062
  • [Journal-full-title] Molecular diagnosis & therapy
  • [ISO-abbreviation] Mol Diagn Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] New Zealand
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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49. Zhou H, Schweikert HU, Wolff M, Fischer HP: Primary peripancreatic lymph node gastrinoma in a woman with MEN1. J Hepatobiliary Pancreat Surg; 2006;13(5):477-81
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  • [Title] Primary peripancreatic lymph node gastrinoma in a woman with MEN1.
  • A 39-year-old woman was admitted to hospital due to perforated relapsing duodenal ulcer.
  • Clinical, laboratory, and surgical examinations revealed a peripancreatic lymph node gastrinoma as the cause of Zollinger-Ellison syndrome.
  • Further examinations established multiple endocrine neoplasia type 1 (MEN1) with a germline mutation at codon 1153 (T->A) in exon 7, causing an amino-acid change, from isoleucine to asparagine (Ile348Asn), in the MEN1 gene.
  • The following findings strongly supported a diagnosis of primary lymph node gastrinoma: a rapid fall of the serum gastrin level after operation, the continuous normalization of the serum gastrin level before and after secretin stimulation, the lack of any symptoms, and the absence of another tumor for 13 years after surgical resection of the tumor-bearing lymph node.
  • A long-term symptom-free follow-up after the excision of a lymphnode gastrinoma is the only reliable criterion for the diagnosis of a primary lymph node tumor.
  • To our knowledge, this is the only well-documented case of a primary lymph node gastrinoma in a patient with MEN1.
  • Our case supports the idea that any gastrinoma in patients with MEN1 should be surgically resected for cure if possible.
  • [MeSH-major] Lymphoma / pathology. Multiple Endocrine Neoplasia Type 1 / pathology
  • [MeSH-minor] Adult. Female. Gastrinoma. Humans. Pancreas. Zollinger-Ellison Syndrome / pathology

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  • (PMID = 17013727.001).
  • [ISSN] 0944-1166
  • [Journal-full-title] Journal of hepato-biliary-pancreatic surgery
  • [ISO-abbreviation] J Hepatobiliary Pancreat Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 39
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50. Naito J, Kaji H, Sowa H, Kitazawa R, Kitazawa S, Tsukada T, Hendy GN, Sugimoto T, Chihara K: Expression and functional analysis of menin in a multiple endocrine neoplasia type 1 (MEN1) patient with somatic loss of heterozygosity in chromosome 11q13 and unidentified germline mutation of the MEN1 gene. Endocrine; 2006 Jun;29(3):485-90
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  • [Title] Expression and functional analysis of menin in a multiple endocrine neoplasia type 1 (MEN1) patient with somatic loss of heterozygosity in chromosome 11q13 and unidentified germline mutation of the MEN1 gene.
  • In some patients with multiple endocrine neoplasia type 1 (MEN1) it is not possible to identify a germline mutation in the MEN1 gene.
  • We sought to document the loss of expression and function of the MEN1 gene product, menin, in the tumors of such a patient.
  • No germline MEN1 mutation was identified in the proband or her son.
  • However, loss of heterozygosity at the MEN1 locus and complete lack of menin expression were demonstrated in the proband's tumor tissue.
  • [MeSH-major] Chromosomes, Human, Pair 11. Germ-Line Mutation / physiology. Loss of Heterozygosity. Multiple Endocrine Neoplasia Type 1 / genetics. Multiple Endocrine Neoplasia Type 1 / metabolism. Proto-Oncogene Proteins / genetics. Proto-Oncogene Proteins / metabolism
  • [MeSH-minor] Aged. Female. Humans. Hypercalcemia / etiology. Hyperthyroidism / diagnosis. Hyperthyroidism / genetics. Liver Neoplasms / diagnosis. Microsatellite Repeats. Parathyroid Hormone / secretion. Parathyroid Neoplasms / pathology. Parathyroid Neoplasms / secretion. Polymorphism, Genetic. Transforming Growth Factor beta / metabolism. Tumor Cells, Cultured

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  • (PMID = 16943588.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Parathyroid Hormone; 0 / Proto-Oncogene Proteins; 0 / Transforming Growth Factor beta
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51. Kihara M, Miyauchi A, Ito Y, Yoshida H, Miya A, Kobayashi K, Takamura Y, Fukushima M, Inoue H, Higashiyama T, Tomoda C: MEN1 gene analysis in patients with primary hyperparathyroidism: 10-year experience of a single institution for thyroid and parathyroid care in Japan. Endocr J; 2009;56(5):649-56
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  • [Title] MEN1 gene analysis in patients with primary hyperparathyroidism: 10-year experience of a single institution for thyroid and parathyroid care in Japan.
  • Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary disease.
  • Primary hyperparathyroidism is known to occur at an early age in MEN1 patients.
  • In MEN1 patients, special care regarding not only surgery for hyperparathyroidism but also other MEN1-related tumors is required.
  • Between 1998 and 2007, 482 patients, including 16 whose hyperparathyroidism was discovered by family screening for MEN1, underwent surgical therapy for primary hyperparathyroidism at our institution.
  • We recommended MEN1 gene analysis for patients having one of the following clinicopathological features:.
  • 2) enlargement of multiple glands;.
  • 3) coexistence or presence of past history of MEN1-related tumors; or 4) family history of hyperparathyroidism or MEN1-related tumors.
  • Thirty-nine of these patients consented to undergo MEN1 genetic analysis and 16 (41%) showed MEN1 mutation.
  • Subject to this strategy, MEN1 index patients in Japan could be detected efficiently and selected for appropriate therapies for hyperparathyroidism and MEN1-related tumors.
  • [MeSH-major] Hyperparathyroidism, Primary / genetics. Multiple Endocrine Neoplasia Type 1 / genetics


52. Libé R, Chanson P: [Endocrine tumors of the pancreas (EPTs) in multiple endocrine neoplasia (MEN1): up-date on prognostic factors, diagnostic procedures and treatment]. Ann Endocrinol (Paris); 2007 Jun;68 Suppl 1:1-8
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  • [Title] [Endocrine tumors of the pancreas (EPTs) in multiple endocrine neoplasia (MEN1): up-date on prognostic factors, diagnostic procedures and treatment].
  • [Transliterated title] Les tumeurs endocrines du pancréas lors de la néoplasie endocrinienne multiple type 1 (NEM1): actualités sur les facteurs pronostiques, l'imagerie et le traitement.
  • Endocrine pancreatic tumors (EPTs) are uncommon tumors, representing 1-2% of all pancreatic neoplasms.
  • EPTs may be part of the multiple endocrine neoplasia type 1 (MEN 1), an autosomal dominant syndrome due to inactivating germline mutation of the menin gene.
  • Somatic mutations of menin are present in about 20% of sporadic neoplasms, particularly gastrinomas and insulinomas.
  • 30-75% of patients with MEN1 have EPTs.
  • The choice of treatment is still debated and is different when the tumor occurs as a part of the MEN syndrome.
  • [MeSH-major] Carcinoma, Islet Cell. Multiple Endocrine Neoplasia Type 1

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  • [ErratumIn] Ann Endocrinol (Paris). 2008 Nov;69(5):459-60
  • (PMID = 17961653.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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53. Fontanière S, Tost J, Wierinckx A, Lachuer J, Lu J, Hussein N, Busato F, Gut I, Wang ZQ, Zhang CX: Gene expression profiling in insulinomas of Men1 beta-cell mutant mice reveals early genetic and epigenetic events involved in pancreatic beta-cell tumorigenesis. Endocr Relat Cancer; 2006 Dec;13(4):1223-36
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  • [Title] Gene expression profiling in insulinomas of Men1 beta-cell mutant mice reveals early genetic and epigenetic events involved in pancreatic beta-cell tumorigenesis.
  • Mutations of the MEN1 gene lead to the occurrence of multiple endocrine neoplasia type 1 (MEN1).
  • To gain insights into the mechanisms of the tumorigenesis related to MEN1 inactivation, we have used mice in which the Men1 gene was specifically disrupted in pancreatic beta-cells.
  • Moreover, an increased proportion of cyclin A2- and D2-expressing cells and the overexpression of insulin-like growth factor 2 (IGF2) protein are detected in mouse Men1 insulinomas by immunostaining.
  • Interestingly, the analysis of DNA methylation patterns by quantitative serial pyrosequencing reveals that four specific CpGs in the intragenic differentially methylated region 2 (DMR2) region of the Igf2 gene known to augment transcription through methylation are significantly hypermethylated in insulinomas of Men1 beta-cell mutant mice at 6 and 10 months of age, even before IGF2 overexpression can be detected.
  • Thus, our data indicate the involvement of both genetic and epigenetic mechanisms in early tumorigenesis of beta-cells related to MEN1 inactivation.
  • [MeSH-major] DNA Methylation. Epigenesis, Genetic. Gene Expression Profiling. Insulin-Secreting Cells / metabolism. Insulinoma / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Pancreatic Neoplasms / genetics
  • [MeSH-minor] Animals. Disease Models, Animal. Gene Expression Regulation, Neoplastic. Integrases / metabolism. Islets of Langerhans / metabolism. Islets of Langerhans / pathology. Mice. Mice, Mutant Strains. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism. Oligonucleotide Array Sequence Analysis. Promoter Regions, Genetic. RNA, Messenger / genetics. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 17158767.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Neoplasm Proteins; 0 / RNA, Messenger; EC 2.7.7.- / Cre recombinase; EC 2.7.7.- / Integrases
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54. Falchetti A, Cilotti A, Vaggelli L, Masi L, Amedei A, Cioppi F, Tonelli F, Brandi ML: A patient with MEN1-associated hyperparathyroidism, responsive to cinacalcet. Nat Clin Pract Endocrinol Metab; 2008 Jun;4(6):351-7
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  • [Title] A patient with MEN1-associated hyperparathyroidism, responsive to cinacalcet.
  • BACKGROUND: A 30-year-old woman with suspected multiple endocrine neoplasia type 1 (MEN1) was referred to our center in 2001 with primary hyperparathyroidism caused by a multiglandular parathyroid adenoma.
  • MEN1 was confirmed by analysis of the MEN1 gene, which revealed a 1555insG frameshift mutation.
  • DIAGNOSIS: Local recurrence of a parathyroid adenoma associated with MEN1.
  • Cinacalcet could represent an important pharmacological intervention in MEN1-associated primary hyperparathyroidism before surgery and in postsurgical recurrences.
  • [MeSH-major] Hyperparathyroidism, Primary / drug therapy. Multiple Endocrine Neoplasia Type 1 / complications. Naphthalenes / therapeutic use

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  • [ErratumIn] Nat Clin Pract Endocrinol Metab. 2008 Jul;4(7):420. Vagelli, Luca [corrected to Vaggelli, Luca]
  • (PMID = 18414463.001).
  • [ISSN] 1745-8374
  • [Journal-full-title] Nature clinical practice. Endocrinology & metabolism
  • [ISO-abbreviation] Nat Clin Pract Endocrinol Metab
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Naphthalenes; 0 / Parathyroid Hormone; 1K860WSG25 / Cinacalcet Hydrochloride; SY7Q814VUP / Calcium
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55. Tham E, Grandell U, Lindgren E, Toss G, Skogseid B, Nordenskjöld M: Clinical testing for mutations in the MEN1 gene in Sweden: a report on 200 unrelated cases. J Clin Endocrinol Metab; 2007 Sep;92(9):3389-95
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  • [Title] Clinical testing for mutations in the MEN1 gene in Sweden: a report on 200 unrelated cases.
  • CONTEXT: Multiple endocrine neoplasia type 1 (MEN1) is a tumor syndrome of the parathyroid, endocrine pancreas, and anterior pituitary caused by mutations in the MEN1 gene on 11q13.
  • OBJECTIVE: The goal of this study was to determine the MEN1 mutation spectrum and detection rate among Swedish patients and identify which patient categories should be tested for MEN1 mutations.
  • DESIGN/SETTING/PATIENTS: DNA sequences and referral forms from patients referred to the Department of Clinical Genetics at Karolinska University Hospital, Sweden, for clinical MEN1 mutation screening were analyzed.
  • The mutation status of 371 patients (including 200 probands) was ascertained, and the multiplex ligation-dependent probe amplification (MLPA) assay was evaluated for the detection of large deletions.
  • MAIN OUTCOME MEASURE: The main outcome measure was MEN1 genotypes.
  • A total of 94% of all MEN1 families had a mutation in the coding region of the MEN1 gene.
  • A total of 6% of sporadic cases had MEN1 mutations.
  • There was no correlation between severe disease and mutation type or location.
  • CONCLUSIONS: A total of 4% of all mutations were large deletions, and MLPA is now included in our standard MEN1 mutation screening.
  • Individuals with at least one typical endocrine tumour and at least one of the following:.
  • 1) a first-degree relative with a major endocrine tumor;.
  • 2) an age of onset less than 30 yr; and/or 3) multiple pancreatic tumors/parathyroid hyperplasia were most likely to harbor a mutation; thus these patients should be screened for MEN1 mutations.
  • [MeSH-major] DNA Mutational Analysis. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Female. Genetic Testing. Genotype. Humans. Male. Middle Aged. Mutation. Pancreatic Neoplasms / genetics. Parathyroid Neoplasms / genetics. Sweden

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  • (PMID = 17623761.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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56. Lindberg D, Akerström G, Westin G: Evaluation of CDKN2C/p18, CDKN1B/p27 and CDKN2B/p15 mRNA expression, and CpG methylation status in sporadic and MEN1-associated pancreatic endocrine tumours. Clin Endocrinol (Oxf); 2008 Feb;68(2):271-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Evaluation of CDKN2C/p18, CDKN1B/p27 and CDKN2B/p15 mRNA expression, and CpG methylation status in sporadic and MEN1-associated pancreatic endocrine tumours.
  • OBJECTIVE: Menin, encoded by the multiple endocrine neoplasia type 1 (MEN1) gene at 11q13, enhances transcription of the cyclin-dependent kinase inhibitors (CDIs), CDKN2C (p18) and CDKN1B (p27) in mouse pancreatic islets, and inactivation of menin reduced CDKN2B (p15) expression in this mouse model.
  • Here, we have compared the relative mRNA expression level and CpG methylation status of p18, p27 and p15 in 18 pancreatic endocrine tumours (PETs) with or without MEN1 gene mutations.
  • Of the three remaining tumours with no expression, two displayed loss of heterozygocity (LOH) at 11q13, one derived from a MEN1 patient.
  • Promoter methylation was not detected for p18 and p27. p15 expression was undetectable in 8/18 (44%) of the PETs, and no general relations to tumour syndrome, malignancy or MEN1 gene mutations were evident.
  • CONCLUSIONS: Expression of p15, p18 and p27 was not generally related to the MEN1 gene mutational status of the investigated 18 PETs.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p15 / genetics. Cyclin-Dependent Kinase Inhibitor p18 / genetics. Cyclin-Dependent Kinase Inhibitor p27 / genetics. Pancreatic Neoplasms / genetics


57. Katai M, Sakurai A, Uchino S, Minemura K, Hashizume K, Fukushima Y: Novel 14 base-pair deletion of the MEN1 gene in a patient with recurrent primary hyperparathyroidism. Jpn J Clin Oncol; 2006 Jun;36(6):395-7
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  • [Title] Novel 14 base-pair deletion of the MEN1 gene in a patient with recurrent primary hyperparathyroidism.
  • MEN1 is the causative gene for multiple endocrine neoplasia type 1 (MEN1), a hereditary syndrome characterized by hyperplastic and neoplastic disorder of endocrine organs such as parathyroid, anterior pituitary and gastroenteropancreatic endocrine tissues.
  • More than 300 germline mutations have already been reported in patients with MEN1.
  • Genetic testing revealed a heterozygous deletion involving 14 bp in exon 6 (starting at amino acid codon 293) of MEN1, which results in early termination of the protein.
  • [MeSH-major] Germ-Line Mutation. Hyperparathyroidism, Primary / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Parathyroid Glands / pathology. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Base Pairing. Female. Frameshift Mutation. Gene Deletion. Humans. Hypercalcemia / diagnosis. Hyperplasia. Middle Aged. Parathyroidectomy. Pedigree. Polymerase Chain Reaction. Recurrence

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  • (PMID = 16714299.001).
  • [ISSN] 0368-2811
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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58. Tala HP, Carvajal CA, González AA, Garrido JL, Tobar J, Solar A, Campino C, Arteaga E, Fardella CE: New splicing mutation of MEN1 gene affecting the translocation of menin to the nucleus. J Endocrinol Invest; 2006 Nov;29(10):888-93
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  • [Title] New splicing mutation of MEN1 gene affecting the translocation of menin to the nucleus.
  • Multiple endocrine neoplasia type 1 (MEN1) is a syndrome inherited in an autosomal dominant trait caused by the inactivation of the tumor suppressor gene MEN1.
  • OBJECTIVE: To communicate a family with a new heterozygous germ line mutation in the intronic region of MEN1 gene and to study its influence in the menin expression.
  • One of them had also a neuroendocrine pancreatic tumor, and 2 had non-functional multinodular cortical adrenal hyperplasia compatible with the diagnosis of MEN1.
  • In vitro studies were done in Chinese hamster ovary (CHO) cells transfected with reporter minigenes carrying the coding regions spanning exon (EX)-intron 9 and EX10 with the mutant and the wild type sequences.
  • RESULTS: We identified a heterozygous G-to-T substitution in the intron-EX junction (IVS9-1 G>T) of MEN1 gene in the index case and the family members.
  • Immunoblot analysis demonstrated that wild type (67 kDa) and two additional mutant proteins (approximately 55 and approximately 90 kDa) were expressed in the pancreatic tissue.
  • The in vitro study showed a 45% nuclear localization of the mutated menin signal and a 95% in the wild type protein.
  • CONCLUSIONS: We identified a new intronic heterozygous germ line mutation (IVS9-1G>T) of MEN1 gene in a family affected by MEN1 syndrome.
  • [MeSH-major] Cell Nucleus / metabolism. Germ-Line Mutation / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / metabolism

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  • (PMID = 17185897.001).
  • [ISSN] 1720-8386
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 9007-49-2 / DNA
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59. Kann PH, Kann B, Fassbender WJ, Forst T, Bartsch DK, Langer P: Small neuroendocrine pancreatic tumors in multiple endocrine neoplasia type 1 (MEN1): least significant change of tumor diameter as determined by endoscopic ultrasound (EUS) imaging. Exp Clin Endocrinol Diabetes; 2006 Jul;114(7):361-5
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  • [Title] Small neuroendocrine pancreatic tumors in multiple endocrine neoplasia type 1 (MEN1): least significant change of tumor diameter as determined by endoscopic ultrasound (EUS) imaging.
  • Knowledge about the prognosis of very small PETs in MEN1 is limited, and if there are no clinical symptoms, endocrine activity or mechanical problems and thus no clear indication for surgical therapy, an appropriate decision for the management of such patients might be to control their follow-up by endosonographic imaging.
  • We included 33 PETs smaller than 15 mm in their largest diameter detected by endosonographic imaging (Pentax FG 32 UA) in ten patients with genetically confirmed MEN1-disease.
  • [MeSH-major] Carcinoma, Neuroendocrine / ultrasonography. Multiple Endocrine Neoplasia Type 1 / ultrasonography. Pancreatic Neoplasms / ultrasonography


60. Triponez F, Goudet P, Dosseh D, Cougard P, Bauters C, Murat A, Cadiot G, Niccoli-Sire P, Calender A, Proye CA, French Endocrine Tumor Study Group: Is surgery beneficial for MEN1 patients with small (&lt; or = 2 cm), nonfunctioning pancreaticoduodenal endocrine tumor? An analysis of 65 patients from the GTE. World J Surg; 2006 May;30(5):654-62; discussion 663-4
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  • [Title] Is surgery beneficial for MEN1 patients with small (< or = 2 cm), nonfunctioning pancreaticoduodenal endocrine tumor? An analysis of 65 patients from the GTE.
  • BACKGROUND: The management of small, nonfunctioning pancreaticoduodenal endocrine tumors (NFPET) in multiple endocrine neoplasia type 1 (MEN1) patients is still controversial.
  • We therefore investigated the effect of surgery on survival and tumor progression in MEN1 patients with NFPET < or = 2 cm by analyzing data from the Groupe des Tumeurs Endocrines (GTE) registry.
  • MATERIALS AND METHODS: Among 579 MEN1 patients in the registry, 65 had NFPET < or = 2 cm.
  • Age at MEN1 and NFPET diagnosis was similar in both groups, as was size of the primary tumor.
  • The average follow-up time after NFPET diagnosis was 6.7 years in the surgery group and 3.3 years in the no surgery group.
  • The 2 patients who died of NFPET had undergone pancreatic surgery at the time of NFPET diagnosis.
  • Overall life expectancy of patients with NFPET < or = 2 cm was not different than that of the 229 MEN1 patients in the registry without any pancreaticoduodenal tumor (P = 0.33).
  • CONCLUSIONS: This study suggests that surgery may not be beneficial for MEN1 patients with NFPET < or = 2 cm.
  • [MeSH-major] Duodenal Neoplasms / surgery. Multiple Endocrine Neoplasia Type 1. Pancreatic Neoplasms / surgery
  • [MeSH-minor] Adult. Disease Progression. Humans. Middle Aged. Neoplasm Staging. Neoplasms, Multiple Primary. Neoplasms, Second Primary. Registries. Retrospective Studies. Survival Analysis

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  • (PMID = 16680582.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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61. Goudet P, Murat A, Cardot-Bauters C, Emy P, Baudin E, du Boullay Choplin H, Chapuis Y, Kraimps JL, Sadoul JL, Tabarin A, Vergès B, Carnaille B, Niccoli-Sire P, Costa A, Calender A, GTE network (Groupe des Tumeurs Endocrines): Thymic neuroendocrine tumors in multiple endocrine neoplasia type 1: a comparative study on 21 cases among a series of 761 MEN1 from the GTE (Groupe des Tumeurs Endocrines). World J Surg; 2009 Jun;33(6):1197-207
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  • [Title] Thymic neuroendocrine tumors in multiple endocrine neoplasia type 1: a comparative study on 21 cases among a series of 761 MEN1 from the GTE (Groupe des Tumeurs Endocrines).
  • BACKGROUND: Thymic neuroendocrine tumors (Th-NET) present a poor prognosis for patients with multiple endocrine neoplasia type 1 (MEN1).
  • The purpose of this article was to study the clinical, biological, and pathological features of Th-NET in a large cohort of patients with MEN1.
  • METHODS: The 761-patient MEN1 cohort from the GTE registry was used (Groupe des Tumeurs Endocrines).
  • All, except one, Th-NET patients were men.
  • Mean age at the time of diagnosis was 42.7 (range, 16.1-67.5) years.
  • Seven patients (33%) belonged to clustered MEN1 families.
  • Various endocrine markers were high, but none were sensitive or specific enough to be useful for Th-NET detection.
  • CT-scan and MRI were always positive at the time of diagnosis.
  • CONCLUSIONS: Several end points may be helpful for future guidelines:.
  • (1) earlier detection of Th-NET in MEN1 patients is required;.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1. Thymus Neoplasms


62. Ozturk M, Chiu CY, Akdeniz N, Jenq SF, Chang SC, Hsa CY, Jap TS: Two novel mutations in the MEN1 gene in subjects with multiple endocrine neoplasia-1. J Endocrinol Invest; 2006 Jun;29(6):523-7
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  • [Title] Two novel mutations in the MEN1 gene in subjects with multiple endocrine neoplasia-1.
  • Multiple endocrine neoplasia type 1 (MEN1) is characterized by parathyroid, enteropancreatic endocrine and pituitary adenomas as well as germline mutation of the MEN1 gene.
  • We describe 2 families with MEN1 with novel mutations in the MEN1 gene.
  • Sequence analysis of the MEN1 gene from leukocyte genomic DNA revealed heterozygous mutations in both probands.
  • The Turkish patient and her affected relatives all had a heterozygous A to G transition at codon 557 (AAG-->GAG) of exon 10 of MEN1 that results in a replacement of lysine by glutamic acid.
  • In conclusion, we have identified 2 novel missense mutations in the MEN1 gene.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics. Mutation, Missense. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adenoma / genetics. Adolescent. Adult. Aged. Amino Acid Sequence. Child. Child, Preschool. DNA Mutational Analysis. Female. Humans. Hyperparathyroidism, Primary / genetics. Male. Middle Aged. Parathyroid Neoplasms / genetics. Pituitary Neoplasms / genetics. Prolactinoma / genetics. Taiwan. Turkey

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  • (PMID = 16840830.001).
  • [ISSN] 0391-4097
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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63. Vierimaa O, Villablanca A, Alimov A, Georgitsi M, Raitila A, Vahteristo P, Larsson C, Ruokonen A, Eloranta E, Ebeling TM, Ignatius J, Aaltonen LA, Leisti J, Salmela PI: Mutation analysis of MEN1, HRPT2, CASR, CDKN1B, and AIP genes in primary hyperparathyroidism patients with features of genetic predisposition. J Endocrinol Invest; 2009 Jun;32(6):512-8
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  • [Title] Mutation analysis of MEN1, HRPT2, CASR, CDKN1B, and AIP genes in primary hyperparathyroidism patients with features of genetic predisposition.
  • OBJECTIVE: Primary hyperparathyroidism (PHPT), a common endocrine condition, is usually caused by sporadically occurring parathyroid adenoma.
  • A subset of patients carry germline mutations in genes such as MEN1 (multiple endocrine neoplasia type 1), HRPT2 (hyperparathyroidism 2), and CASR (calcium-sensing receptor) predisposing to syndromic forms of PHPT or familial isolated hyperparathyroidism (FIHP).
  • Recently, germline mutations in two novel genes AIP (aryl hydrocarbon receptor-interacting protein) and CDKN1B (cyclin-dependent kinase inhibitor 1B) have been found to be associated with endocrine tumors.
  • The purpose of this study was to evaluate the role of MEN1, HRPT2, CASR, AIP, and CDKN1B genes in PHPT patients with clinical features suggestive of genetic predisposition.
  • Patients with multiglandular or recurrent/persistent disease, other MEN1- related manifestations, aged 40 yr or younger at onset or with a family history of PHPT/MEN1-related tumor were invited to the study.
  • Twenty patients with previously diagnosed MEN1 were excluded.
  • Participants were interviewed and blood samples obtained for biochemical screening and mutation analysis of MEN1, HRPT2, CASR, AIP, and CDKN1B.
  • One patient was found to carry the c. 1356_1367del12 MEN1 founder mutation.
  • CONCLUSIONS: Apart from MEN1, mutations in other genes predisposing to PHPT seem to be rare or non-existing in Northern Finnish PHPT patients.
  • [MeSH-major] Hyperparathyroidism, Primary / genetics. Parathyroid Neoplasms / genetics
  • [MeSH-minor] Adult. Cyclin-Dependent Kinase Inhibitor p27. DNA / chemistry. DNA / genetics. Female. Finland. Genetic Predisposition to Disease. Genetic Variation. Humans. Intracellular Signaling Peptides and Proteins / chemistry. Intracellular Signaling Peptides and Proteins / genetics. Male. Middle Aged. Polymerase Chain Reaction. Proto-Oncogene Proteins / chemistry. Proto-Oncogene Proteins / genetics. Receptors, Calcium-Sensing / chemistry. Receptors, Calcium-Sensing / genetics. Retrospective Studies. Sequence Analysis, DNA. Tumor Suppressor Proteins / chemistry. Tumor Suppressor Proteins / genetics. Young Adult

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  • (PMID = 19474519.001).
  • [ISSN] 1720-8386
  • [Journal-full-title] Journal of endocrinological investigation
  • [ISO-abbreviation] J. Endocrinol. Invest.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / CASR protein, human; 0 / CDC73 protein, human; 0 / CDKN1B protein, human; 0 / Intracellular Signaling Peptides and Proteins; 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 0 / Receptors, Calcium-Sensing; 0 / Tumor Suppressor Proteins; 0 / aryl hydrocarbon receptor-interacting protein; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27; 9007-49-2 / DNA
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64. Goudet P, Murat A, Binquet C, Cardot-Bauters C, Costa A, Ruszniewski P, Niccoli P, Ménégaux F, Chabrier G, Borson-Chazot F, Tabarin A, Bouchard P, Delemer B, Beckers A, Bonithon-Kopp C: Risk factors and causes of death in MEN1 disease. A GTE (Groupe d'Etude des Tumeurs Endocrines) cohort study among 758 patients. World J Surg; 2010 Feb;34(2):249-55
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  • [Title] Risk factors and causes of death in MEN1 disease. A GTE (Groupe d'Etude des Tumeurs Endocrines) cohort study among 758 patients.
  • BACKGROUND: The natural history of multiple endocrine neoplasia type 1 (MEN1) is known through single-institution or single-family studies.
  • We aimed to analyze the risk factors and causes of death in a large cohort of MEN1 patients.
  • METHODS: Overall, 758 symptomatic MEN1 patients were identified through the GTE network (Groupe d'étude des Tumeurs Endocrines), which involves French and Belgian genetics laboratories responsible for MEN1 diagnosis and 80 clinical reference centers.
  • Female gender, family history of MEN1, and recent diagnosis were associated with a lower risk of death.
  • Compared with nonaffected patients, those with thymic tumors (hazard ratio [HR] = 4.64, 95% CI = 1.73-12.41), glucagonomas-vipomas-somatostatinomas (HR = 4.29, 95% CI = 1.54-11.93), nonfunctioning pancreatic tumors (HR = 3.43, 95% CI = 1.71-6.88), and gastrinoma (HR = 1.89, 95% CI = 1.09-3.25) had a higher risk of death after adjustment for age, gender, and diagnosis period.
  • The proportion of MEN1-related deaths decreased from 76.8 to 71.4% after 1990.
  • CONCLUSIONS: The prognosis of MEN1 disease has improved since 1980.
  • Most deaths were related to MEN1.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / mortality

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  • (PMID = 19949948.001).
  • [ISSN] 1432-2323
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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65. Jap TS, Chiu CY, Won JG, Wu YC, Chen HS: Novel mutations in the MEN1 gene in subjects with multiple endocrine neoplasia-1. Clin Endocrinol (Oxf); 2005 Mar;62(3):336-42
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  • [Title] Novel mutations in the MEN1 gene in subjects with multiple endocrine neoplasia-1.
  • OBJECTIVE: To identify MEN1 gene mutations and characterize clinical manifestations in Chinese kindred with multiple endocrine neoplasia type 1 (MEN1) in Taiwan.
  • PATIENTS AND METHODS: Eight unrelated subjects (one male and seven females, age range 26-70 years) with clinical manifestations of MEN1 were analysed.
  • RESULTS: We identified heterozygous MEN1 gene mutations in all eight probands and 10 affected subjects as well as in 13 clinically asymptomatic relatives.
  • Affected subjects in families 5-7 shared the same C insertion at nucleotide 1650 of exon 10, similar to that previously described as a hotspot for mutation, and proband 8 had a previously described mutation in intron 4 of the MEN1 gene (IVS4-9 G --> A).
  • We also found that 18 (58%) of our 31 MEN1 mutant carriers had clinical symptoms, whereas four (13%) had biochemical abnormalities without clinical symptoms, and nine (29%) were unaffected both clinically and biochemically.
  • CONCLUSIONS: We have identified four novel mutations in the MEN1 gene in patients with MEN1 in Taiwan.
  • [MeSH-major] Germ-Line Mutation. Multiple Endocrine Neoplasia Type 1 / genetics. Proto-Oncogene Proteins / genetics

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  • (PMID = 15730416.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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66. Anlauf M, Enosawa T, Henopp T, Schmitt A, Gimm O, Brauckhoff M, Dralle H, Musil A, Hauptmann S, Perren A, Klöppel G: Primary lymph node gastrinoma or occult duodenal microgastrinoma with lymph node metastases in a MEN1 patient: the need for a systematic search for the primary tumor. Am J Surg Pathol; 2008 Jul;32(7):1101-5
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  • [Title] Primary lymph node gastrinoma or occult duodenal microgastrinoma with lymph node metastases in a MEN1 patient: the need for a systematic search for the primary tumor.
  • We report on a 38-year-old woman suffering from multiple endocrine neoplasia type 1 (MEN1) confirmed by menin gene mutation analysis.
  • MEN1 disease started with primary hyperparathyroidism followed by Cushing disease, the detection of tumors of the pituitary, adrenal cortex, and the pancreas and also an elevated serum gastrin level.
  • In addition, 3 suprapancreatic lymph nodes with gastrin-positive endocrine tissue were found.
  • None of the pancreatic microadenomas expressed gastrin and no duodenal endocrine tumor was found despite careful macroscopic examination.
  • [MeSH-major] Duodenal Neoplasms / secondary. Gastrinoma / secondary. Lymph Nodes / pathology. Multiple Endocrine Neoplasia Type 1 / pathology. Neoplasms, Unknown Primary


67. Veldman MW, Reading CC, Farrell MA, Mullan BP, Wermers RA, Grant CS, Thompson GB: Percutaneous parathyroid ethanol ablation in patients with multiple endocrine neoplasia type 1. AJR Am J Roentgenol; 2008 Dec;191(6):1740-4
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  • [Title] Percutaneous parathyroid ethanol ablation in patients with multiple endocrine neoplasia type 1.
  • OBJECTIVE: 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.
  • CONCLUSION: Ethanol ablation is a viable alternative to reoperation for the management of recurrent primary hyperparathyroidism in patients with MEN1.
  • [MeSH-major] Ethanol / administration & dosage. Hyperparathyroidism / therapy. Multiple Endocrine Neoplasia Type 1 / therapy. Neoplasm Recurrence, Local / therapy. Parathyroid Neoplasms / therapy


68. Goudet P, Bonithon C, Costa A, Cadiot G, Baudin E, Murat A, Delemer B, Tabarin A, Lecomte P, Calender A, Endocrine Tumor Study Group (GTE): [A Multiple endocrine neoplasia type-1 observatory in a French-speaking area. A tool from the Endocrine Tumor study Group (GTE)]. Ann Endocrinol (Paris); 2007 Jun;68(2-3):154-9
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  • [Title] [A Multiple endocrine neoplasia type-1 observatory in a French-speaking area. A tool from the Endocrine Tumor study Group (GTE)].
  • [Transliterated title] Observatoire francophone des néoplasies endocriniennes multiples de type 1. Un outil du Groupe d'étude des Tumeurs Endocrines (GTE).
  • Wermer's syndrome or Multiple Endocrine Neoplasia Type-1 (MEN1) is an autosomal dominant inherited disease, related to mutations in MEN1, an approximately 10-kb gene encoding menin, localized on chromosome 11q13.
  • The Endocrine Tumor Group (GTE) has set up a MEN1 observatory of 1001 regularly followed MEN1 cases.
  • This observatory aims at registering and evaluating MEN1 cases in a large cohort.
  • Any new study on a particular unexplored aspect of the disease may be proposed by a physician to the GTE.
  • This article describes the way to diagnose a new MEN1 case and to register it.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / epidemiology. Multiple Endocrine Neoplasia Type 1 / genetics


69. Bartsch DK, Langer P, Rothmund M: Surgical aspects of gastrinoma in multiple endocrine neoplasia type 1. Wien Klin Wochenschr; 2007;119(19-20):602-8
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  • [Title] Surgical aspects of gastrinoma in multiple endocrine neoplasia type 1.
  • Gastrinoma is the most frequent functional pancreaticoduodenal endocrine tumor in patients with multiple endocrine neoplasia type 1 (MEN1) and one major determinant of mortality in this syndrome.
  • Whether routine surgical exploration should be performed in a patient with MEN1 associated Zollinger-Ellison syndrome (ZES) to possibly reduce the malignant spread and eventually increase survival still remains controversial.
  • There is not only disagreement about the indication for surgical exploration, but also what type of procedure should be performed, since sufficient evidence-based data are not available.
  • The article discusses the available data on treatment strategies of MEN1 associated ZES.
  • [MeSH-major] Duodenal Neoplasms / surgery. Gastrinoma / surgery. Gastrins / blood. Multiple Endocrine Neoplasia Type 1 / surgery. Pancreatic Neoplasms / surgery
  • [MeSH-minor] Disease Progression. Humans. Laparoscopy. Pancreatectomy. Pancreaticoduodenectomy. Prognosis. Reoperation. Survival Rate. Zollinger-Ellison Syndrome / blood. Zollinger-Ellison Syndrome / mortality. Zollinger-Ellison Syndrome / surgery


70. Tanabe T, Yasuo M, Tsushima K, Urushihata K, Yamamoto H, Hanaoka M, Koizumi T, Fujimoto K, Yamazaki Y, Hirose Y, Hamano H, Sakurai A, Kubo K: Mediastinal seminoma in a patient with multiple endocrine neoplasia type 1. Intern Med; 2008;47(18):1615-9
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  • [Title] Mediastinal seminoma in a patient with multiple endocrine neoplasia type 1.
  • A patient with multiple endocrine neoplasia type 1 (MEN1) developed a mediastinal seminoma.
  • A diagnosis of mediastinal seminoma was pathologically confirmed and a complete remission was achieved by chemotherapy.
  • During his hospital stay, hyperparathyroidism and multiple pancreatic tumors associated with hypergastrinemia were found.
  • A diagnosis of MEN1 was made genetically.
  • Although patients with MEN1 manifest a variety of neoplastic disorders, no cases of concurrent seminoma and MEN1 have previously been reported.
  • In addition, no etiological relationship between seminoma and MEN1 has yet been reported.
  • [MeSH-major] Mediastinal Neoplasms / diagnosis. Multiple Endocrine Neoplasia Type 1 / diagnosis. Neoplasms, Multiple Primary / diagnosis. Seminoma / diagnosis


71. Papaconstantinou M, Maslikowski BM, Pepper AN, Bédard PA: Menin: the protein behind the MEN1 syndrome. Adv Exp Med Biol; 2009;668:27-36
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  • [Title] Menin: the protein behind the MEN1 syndrome.
  • The cloning of the MEN1 gene in 1997 led to the characterization of menin, the protein behind the multiple endocrine neoplasia Type 1 syndrome.
  • MEN1 missense mutations are dispersed along the coding region of the gene but are more common in the most conserved regions.
  • These protein interactions are generally facilitated by multiple domains or encompass a large portion of menin.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1. Proto-Oncogene Proteins

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  • (PMID = 20175450.001).
  • [ISSN] 0065-2598
  • [Journal-full-title] Advances in experimental medicine and biology
  • [ISO-abbreviation] Adv. Exp. Med. Biol.
  • [Language] eng
  • [Grant] Canada / Canadian Institutes of Health Research / / MOP-10272; Canada / Canadian Institutes of Health Research / / MOP-84359
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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72. Ferolla P, Falchetti A, Filosso P, Tomassetti P, Tamburrano G, Avenia N, Daddi G, Puma F, Ribacchi R, Santeusanio F, Angeletti G, Brandi ML: Thymic neuroendocrine carcinoma (carcinoid) in multiple endocrine neoplasia type 1 syndrome: the Italian series. J Clin Endocrinol Metab; 2005 May;90(5):2603-9
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  • [Title] Thymic neuroendocrine carcinoma (carcinoid) in multiple endocrine neoplasia type 1 syndrome: the Italian series.
  • Neuroendocrine tumors may occur in the setting of multiple endocrine neoplasia type 1 (MEN1) syndrome.
  • We report characterization of the seven Italian cases in which this association occurred among a series of 221 MEN1 patients (41 sporadic and 180 familial cases; prevalence, 3.1%).
  • The first diagnosis was between the second and fifth decades.
  • Therefore, prophylactic thymectomy should be considered at neck surgery for primary hyperparathyroidism in MEN1 male patients, especially for smokers, and, due to the frequent familial clusters distribution of this pathology, in subjects with affected relatives presenting this feature.
  • Thus, we recommend screening every patient affected with a neuroendocrine thymic neoplasm for MEN1 syndrome.
  • [MeSH-major] Carcinoid Tumor / genetics. Multiple Endocrine Neoplasia Type 1 / genetics. Thymus Neoplasms / genetics


73. Rigabert J, De Clermont H: [Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1]. Ann Endocrinol (Paris); 2007 Jun;68(2-3):199-203
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  • [Title] [Diagnostic procedures and more particularly, place of scintigraphy in neuroendocrine tumors, example of vipoma in MEN 1].
  • [Transliterated title] Outils diagnostiques et plus particulièrement, place de la scintigraphie dans les tumeurs neuroendocrines: l'exemple d'un vipome dans une NEM de type 1.
  • Functioning endocrine pancreatic tumors in multiple endocrine neoplasia type 1 (MEN1) are rare.
  • This case is interesting in many ways: this is an exceptional illustration of MEN 1 with vipoma associated with calcitonin secretion and it is also a good example of the benefits and limitations of each diagnostic procedure in the heterogeneous group of neuroendocrine tumors.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / diagnosis. Neuroendocrine Tumors / diagnosis. Vipoma / diagnosis

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  • (PMID = 17292846.001).
  • [ISSN] 0003-4266
  • [Journal-full-title] Annales d'endocrinologie
  • [ISO-abbreviation] Ann. Endocrinol. (Paris)
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers; 9007-12-9 / Calcitonin; 971Z4W1S09 / Technetium Tc 99m Sestamibi
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74. Kim HJ, Park JS, Kim CS, Kang ES, Cha BS, Lim SK, Kim KR, Lee HC, Ahn CW: A case of multiple endocrine neoplasia type 1 combined with papillary thyroid carcinoma. Yonsei Med J; 2008 Jun 30;49(3):503-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of multiple endocrine neoplasia type 1 combined with papillary thyroid carcinoma.
  • This is the first report of papillary thyroid carcinoma combined with multiple endocrine neoplasia type 1 (MEN1) in Korea.
  • MEN1 is a hereditary disease comprising neoplastic disorders such as pituitary, parathyroid and pancreatic neuroendocrine tumor, such as gastrinoma.
  • Herein we present a 39-year-old woman who manifested typical features of MEN1 with a coincidental papillary thyroid carcinoma.
  • Although the family history of MEN1 was definite, her genetic analysis of DNA had revealed no germline mutation in MEN1 gene locus.
  • Unidentified culprit gene unable us further genetic study to find LOH (loss of heterogeneity) in 11q13, the possible explanation of papillary thyroid carcinoma as a new component of MEN1.
  • As we have first experienced a case of MEN1 combined with papillary thyroid carcinoma in Korea, we report it with the review of literature.
  • [MeSH-major] Carcinoma, Papillary / pathology. Multiple Endocrine Neoplasia Type 1 / pathology. Thyroid Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans. Mutation. Proto-Oncogene Proteins / genetics


75. Gaudray P, Weber G: Genetic background of MEN1: from genetic homogeneity to functional diversity. Adv Exp Med Biol; 2009;668:17-26
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Genetic background of MEN1: from genetic homogeneity to functional diversity.
  • Multiple Endocrine Neoplasia Type 1 corresponds to a monogenic predisposition syndrome inherited as a dominant trait that affects a variety of endocrine tissues, in particular parathyroids, endocrine pancreas and anterior pituitary.
  • It is caused by mutations in the MEN1 tumor suppressor gene that inactivate menin, the MEN1 encoded protein.
  • However, the role of menin does not--by far--end here.
  • It plays (too) many roles in the control of cell life and normality, far beyond endocrine oncogenesis, making it unlikely that the function of menin can be deciphered only by genetic investigation.
  • In this context, writing a chapter on the genetic background of MEN1 appears at the same time as a challenge and a paradox.
  • A paradox since genetics is simultaneously at the background and at the forefront of MEN1.
  • Our attempts are thus more investigating new--as well as already open issues than delivering a catalog of MEN1 gene mutations.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / genetics
  • [MeSH-minor] Animals. Genetic Predisposition to Disease. Humans. Mutation. Phylogeny. Proto-Oncogene Proteins / classification. Proto-Oncogene Proteins / genetics

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  • (PMID = 20175449.001).
  • [ISSN] 0065-2598
  • [Journal-full-title] Advances in experimental medicine and biology
  • [ISO-abbreviation] Adv. Exp. Med. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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76. Benito M, Asa SL, Livolsi VA, West VA, Snyder PJ: Gonadotroph tumor associated with multiple endocrine neoplasia type 1. J Clin Endocrinol Metab; 2005 Jan;90(1):570-4
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  • [Title] Gonadotroph tumor associated with multiple endocrine neoplasia type 1.
  • Although anterior pituitary tumors constitute a main clinical feature of multiple endocrine neoplasia type 1 (MEN1), and most types of pituitary tumors have been associated with MEN1, gonadotroph tumors have not previously been recognized clinically as part of this syndrome.
  • The diagnosis of MEN1 was confirmed by finding a deletion mutation (c.307delC) on the second exon of the MEN1 gene that predicts truncation of the resulting menin protein 15 codons downstream from the deletion (p.Leu103fsX15).
  • This case illustrates that gonadotroph tumors, like other pituitary tumors, can be part of MEN1.
  • The clinical implications of this case are that the clinical and biochemical features of gonadotroph tumors should be considered when evaluating patients for MEN1, and MEN1 should be considered in patients who have gonadotroph tumors.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / complications. Pituitary Neoplasms / etiology


77. Fendrich V, Langer P, Waldmann J, Bartsch DK, Rothmund M: Management of sporadic and multiple endocrine neoplasia type 1 gastrinomas. Br J Surg; 2007 Nov;94(11):1331-41
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  • [Title] Management of sporadic and multiple endocrine neoplasia type 1 gastrinomas.
  • BACKGROUND: Gastrinomas are functional endocrine duodenopancreatic tumours and are responsible for Zollinger-Ellison syndrome (ZES).
  • Some 20 per cent of gastrinomas occur in association with multiple endocrine neoplasia type 1 (MEN1) and 50-60 per cent of tumours are malignant at the time of diagnosis.
  • Pylorus-preserving pancreaticoduodenectomy may be the procedure of choice for MEN1-ZES.
  • [MeSH-major] Duodenal Neoplasms / therapy. Gastrinoma / surgery. Multiple Endocrine Neoplasia Type 1 / therapy. Pancreatic Neoplasms / therapy


78. Luzi E, Marini F, Tognarini I, Carbonell Sala S, Galli G, Falchetti A, Brandi ML: Ribozyme-mediated compensatory induction of menin-oncosuppressor function in primary fibroblasts from MEN1 patients. Cancer Gene Ther; 2010 Nov;17(11):814-25
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  • [Title] Ribozyme-mediated compensatory induction of menin-oncosuppressor function in primary fibroblasts from MEN1 patients.
  • Multiple endocrine neoplasia type 1 (MEN1) syndrome is characterized by the occurrence of tumors of parathyroids, neuroendocrine cells of the gastro-enteropancreatic tract and anterior pituitary.
  • MEN1 gene encodes menin-oncosuppressor protein.
  • Loss of heterozygosity at 11q13 is typical of MEN1 tumors.
  • We have analyzed the MEN1 mRNA and menin expression in fibroblasts from normal skin biopsies and from MEN1 patients (two with a frameshift 738del4 (exon 3) mutation, introducing a premature stop codon, and an individual with an R460X (exon 10) nonsense mutation).
  • The expression of full-length menin protein did not differ between MEN1 and normal fibroblasts.
  • Wild-type alleles mRNAs were expressed in MEN1 patients, whereas mutant alleles were partially degraded by nonsense-mediated mRNA decay pathway, suggesting a mechanism of compensation for allelic loss by the up-regulation of wild-type menin expression at a post-transcriptional level.
  • Small-interfering RNA silencing of the wild-type mRNA allele abolished menin compensation, whereas the ribozyme silencing of the MEN1-mutated mRNA allele resulted in strongly enhanced wild-type menin expression.
  • Gel-retardation analysis showed that in vitro-specific RNA-protein complexes bound to MEN1 mRNA.
  • These findings contribute to the understanding of tumorigenesis in MEN1, offering the basis for the development of RNA-based therapies in MEN1 gene mutation carriers.
  • [MeSH-major] Fibroblasts / metabolism. Multiple Endocrine Neoplasia Type 1 / metabolism. Proto-Oncogene Proteins / genetics. RNA, Catalytic / metabolism. RNA, Small Interfering / metabolism

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  • (PMID = 20706287.001).
  • [ISSN] 1476-5500
  • [Journal-full-title] Cancer gene therapy
  • [ISO-abbreviation] Cancer Gene Ther.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins; 0 / RNA, Catalytic; 0 / RNA, Messenger; 0 / RNA, Small Interfering; EC 3.4.22.- / CASP8 protein, human; EC 3.4.22.- / Caspase 8
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79. Sakurai A, Katai M, Yamashita K, Mori J, Fukushima Y, Hashizume K: Long-term follow-up of patients with multiple endocrine neoplasia type 1. Endocr J; 2007 Apr;54(2):295-302
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  • [Title] Long-term follow-up of patients with multiple endocrine neoplasia type 1.
  • Whether early surgical treatment of non-functioning pancreas islet cell tumor (NFPT) provides a favorable quality of life and life expectancy in patients with multiple endocrine neoplasia type 1 (MEN1) remains controversial.
  • We analyzed the long-term clinical courses and surgical outcomes of 14 Japanese patients with MEN1-associated NFPTs.
  • [MeSH-major] Adenoma, Islet Cell / etiology. Adenoma, Islet Cell / surgery. Multiple Endocrine Neoplasia Type 1 / complications. Pancreatic Neoplasms / etiology. Pancreatic Neoplasms / surgery
  • [MeSH-minor] Adult. Asian Continental Ancestry Group. Diabetes Mellitus / etiology. Disease Progression. Female. Follow-Up Studies. Glucose Intolerance / etiology. Humans. Male. Middle Aged. Neoplasm Metastasis. Neoplasms, Second Primary / radiography. Pancreas / physiopathology. Pancreatectomy / adverse effects. Pancreatectomy / methods. Tomography, X-Ray Computed


80. Krysiak R, Kajdaniuk D, Marek B, Okopień B: Atypical clinical manifestations of multiple endocrine neoplasia type 1 syndrome. Pol Arch Med Wewn; 2009 Mar;119(3):175-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical clinical manifestations of multiple endocrine neoplasia type 1 syndrome.
  • Multiple endocrine neoplasia type 1 (MEN1) is a hereditary tumor syndrome characterized by a genetic predisposition to develop a variety of neuroendocrine tumors and hormone excess syndromes.
  • The major components of MEN1 are hyperparathyroidism due to multiple parathyroid adenomas or hyperplasia, duodenopancreatic neuroendocrine tumors and pituitary adenomas, most often producing prolactin.
  • Physicians' inadequate knowledge of this clinical entity and sometimes its atypical presentation result in a probable significant underdiagnosis of MEN1.
  • This case shows that MEN1 manifests itself even in older groups and hyperparathyroidism may not be the first symptom of this syndrome.
  • Therefore, we believe that all subjects who, regardless of age, gender and initial manifestation present with whichever the major symptom should be followed up regularly for the early detection of MEN1.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / diagnosis


81. Nunes VS, Chang CV, Mazeto GM, Marques ME, Castro AV, Nogueira CR: Possible association between Carney complex and multiple endocrine neoplasia type 1 phenotypes. Arq Bras Endocrinol Metabol; 2008 Nov;52(8):1356-61
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  • [Title] Possible association between Carney complex and multiple endocrine neoplasia type 1 phenotypes.
  • Carney Complex (CNC) and Multiple Endocrine Neoplasia type 1 (MEN1) are forms of multiple endocrine neoplasia of dominant autosomal inheritance.
  • Diagnosis of CNC occurs when two major criteria (lentiginoses, primary pigmented nodular adrenocortical disease, cardiac and cutaneous myxomas, acromegaly, testicular neoplasias, thyroid cancer) are observed and/or a major criterion associated with a supplementary criterion (affected relative, PRKAR1A gene mutation) occurs.
  • On the other hand, diagnosis for MEN1 occurs through detection of two or more tumors located at the pituitary gland, parathyroid and/or pancreatic cells.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / pathology
  • [MeSH-minor] Acromegaly / diagnosis. Carcinoma, Papillary / diagnosis. Humans. Hyperparathyroidism, Primary / diagnosis. Male. Middle Aged. Mutation. Pedigree. Phenotype. Proto-Oncogene Proteins / genetics. Thyroid Neoplasms / diagnosis


82. Hellman P, Hennings J, Akerström G, Skogseid B: Endoscopic ultrasonography for evaluation of pancreatic tumours in multiple endocrine neoplasia type 1. Br J Surg; 2005 Dec;92(12):1508-12
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  • [Title] Endoscopic ultrasonography for evaluation of pancreatic tumours in multiple endocrine neoplasia type 1.
  • BACKGROUND: Pancreatic tumours are common in patients with multiple endocrine neoplasia type 1 (MEN1), and close surveillance is needed to detect pancreatic lesions at an early stage.
  • METHODS: EUS was evaluated in 25 patients with MEN1, two of whom had symptoms due to hormonal secretion.
  • CONCLUSION: EUS is a more sensitive technique for the detection and localization of potentially malignant lesions in patients with MEN1 than computed tomography or transabdominal ultrasonography.
  • [MeSH-major] Endosonography / instrumentation. Multiple Endocrine Neoplasia Type 1 / ultrasonography. Pancreatic Neoplasms / ultrasonography


83. de Vogelaere K, De Schepper J, Vanhoeij M, De Mey J, Goossens A, Vanbesien J, De Backer A, Delvaux G: Laparoscopic management of insulinoma in a child with multiple endocrine neoplasia type 1. J Laparoendosc Adv Surg Tech A; 2006 Jun;16(3):335-8
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  • [Title] Laparoscopic management of insulinoma in a child with multiple endocrine neoplasia type 1.
  • The diagnosis and surgical management of insulinomas associated with multiple endocrine neoplasia type 1 (MEN1) pose additional problems in children because of the long-term risk of recurrence of other pancreatic and non-pancreatic tumors.
  • MEN1 was genetically proven by direct DNA testing.
  • A pancreatic tumor can arise before the age of 10 in patients with MEN1 and can be surgically treated by a laparoscopical approach.
  • [MeSH-major] Insulinoma / pathology. Insulinoma / surgery. Laparoscopy / methods. Multiple Endocrine Neoplasia Type 1 / pathology. Pancreatic Neoplasms / pathology. Pancreatic Neoplasms / surgery


84. Lejonklou MH, Edfeldt K, Johansson TA, Stålberg P, Skogseid B: Neurogenin 3 and neurogenic differentiation 1 are retained in the cytoplasm of multiple endocrine neoplasia type 1 islet and pancreatic endocrine tumor cells. Pancreas; 2009 Apr;38(3):259-66
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neurogenin 3 and neurogenic differentiation 1 are retained in the cytoplasm of multiple endocrine neoplasia type 1 islet and pancreatic endocrine tumor cells.
  • OBJECTIVES: To investigate if transcription factors involved in pancreatic differentiation and regeneration are present in pancreatic endocrine tumors and if they are differentially expressed in normal pancreas compared with multiple endocrine neoplasia type 1 (MEN1) nontumorous pancreas.
  • METHODS: The expression of neurogenin 3 (NEUROG3), neurogenic differentiation 1 (NEUROD1), POU class 3 homeobox 4 (POU3F4), pancreatic duodenal homeobox factor 1 (PDX1), ribosomal protein L10 (RPL10), delta-like 1 homolog (Drosophila; DLK1), and menin was analyzed by immunohistochemistry in normal pancreas and pancreatic endocrine tumors from 6 patients with MEN1 and 16 patients with sporadic tumors, as well as pancreatic specimens from Men1 heterozygous and wild type mice.
  • RESULTS: Tumors and MEN1 nontumorous endocrine cells showed a prominent cytoplasmatic NEUROG3 and NEUROD1 expression.
  • These factors were significantly more expressed in the cytoplasm of Men1 heterozygous mouse islet cells compared with wild type islets; the latter showed an exclusively nuclear reactivity.
  • The degree of Pou3f4, Rpl10, and Dlk1 immunoreactivities differed significantly between islets of heterozygous and wild type mice.
  • The expressions of RPL10 and NEUROD1 were prominent in the MEN1 human and heterozygous mouse exocrine pancreas.
  • CONCLUSIONS: Transcription factors involved in pancreatic development show altered expression and subcellular localization in MEN1 nontumorous pancreas and pancreatic endocrine tumors.
  • [MeSH-major] Basic Helix-Loop-Helix Transcription Factors / metabolism. Insulinoma / metabolism. Multiple Endocrine Neoplasia Type 1 / metabolism. Nerve Tissue Proteins / metabolism. Pancreatic Neoplasms / metabolism

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  • (PMID = 19307926.001).
  • [ISSN] 1536-4828
  • [Journal-full-title] Pancreas
  • [ISO-abbreviation] Pancreas
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Basic Helix-Loop-Helix Transcription Factors; 0 / DLK1 protein, human; 0 / Dlk1 protein, mouse; 0 / Homeodomain Proteins; 0 / Intercellular Signaling Peptides and Proteins; 0 / Membrane Proteins; 0 / NEUROD1 protein, human; 0 / NEUROG3 protein, human; 0 / Nerve Tissue Proteins; 0 / Neurod1 protein, mouse; 0 / Neurog3 protein, mouse; 0 / POU Domain Factors; 0 / POU3F4 protein, human; 0 / Ribosomal Proteins; 0 / Trans-Activators; 0 / pancreatic and duodenal homeobox 1 protein; 0 / ribosomal protein L10; 147258-09-1 / Pou3f4 protein, mouse
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85. Matoso A, Zhou Z, Hayama R, Flesken-Nikitin A, Nikitin AY: Cell lineage-specific interactions between Men1 and Rb in neuroendocrine neoplasia. Carcinogenesis; 2008 Mar;29(3):620-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cell lineage-specific interactions between Men1 and Rb in neuroendocrine neoplasia.
  • Inactivation of multiple endocrine neoplasia (MEN) type 1 gene (Men1) results in development of multiple endocrine tumors in Men1(+/-) mice and in humans.
  • Intriguingly, loss of the wild-type retinoblastoma 1 (Rb) gene also leads to MEN-like phenotype in Rb(+/-) mice.
  • To evaluate potential genetic interactions between these genes, we prepared and characterized Men1(+/-)Rb(+/-) compound mice in parallel with their parental genotypes.
  • Men1 and Rb did not cooperate in tumor suppression, as demonstrated by comparable survival rates of Rb(+/-) and Men1(+/-)Rb(+/-) mice, absence of tumor growth acceleration and lack of novel neoplasms.
  • Notably, the loss of the remaining copy of the wild-type Men1 and Rb was mutually exclusive in all tumors of Men1(+/-)Rb(+/-) mice, including pituitary anterior lobe and adrenal medulla neoplasms shared by Rb- and Men1-deficient phenotypes.
  • Down-regulation of Men1 targets p18 and p27 and increased presence of phosphorylated-Rb were observed in Men1-deficient pheochromocytomas of Men1(+/-)Rb(+/-) and Men1(+/-) mice.
  • At the same time, the RNA interference (RNAi) knock-down of Men1 mRNA resulted in increased apoptosis of Rb-deficient medullary thyroid carcinoma cells.
  • These results demonstrate that, depending on cell lineage context, combined Men1 and Rb deficiency may be either redundant or detrimental to neoplastic growth.
  • Identification of cell lineage-specific interactions between Men1 and Rb may have important implications for development of rationally designed therapeutic approaches.


86. Müssig K, Wehrmann M, Horger M, Bares R, Häring HU, Gallwitz B, Petersenn S: Lymph node gastrinoma in multiple endocrine neoplasia type 1 - a diagnostic challenge. Exp Clin Endocrinol Diabetes; 2008 Oct;116(9):554-7
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  • [Title] Lymph node gastrinoma in multiple endocrine neoplasia type 1 - a diagnostic challenge.
  • BACKGROUND: Gastrinomas are the most frequent hormonally-active neuroendocrine tu-mours in patients with multiple endocrine neoplasia type 1 (MEN1).
  • CASE REPORT: We report on the diagnostic difficulties in a 62-year-old female patient with MEN1 and lymph node gastrinoma.
  • CONCLUSION: Localization of gastrinomas in patients with MEN1 is challenging due to their small size, frequent duodenal location, and multiplicity.
  • Therefore, while some studies support the existence of primary lymph node gastrinoma in patients with sporadic disease, this diagnosis should not be made in MEN1 patients.
  • [MeSH-major] Gastrinoma / pathology. Lymph Nodes / pathology. Multiple Endocrine Neoplasia Type 1 / pathology
  • [MeSH-minor] Diarrhea / etiology. Duodenal Ulcer / pathology. Esophagitis / pathology. Female. Follow-Up Studies. Gastritis / pathology. Humans. Middle Aged. Treatment Outcome

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  • (PMID = 18523912.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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87. Bartsch DK, Fendrich V, Langer P, Celik I, Kann PH, Rothmund M: Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Ann Surg; 2005 Dec;242(6):757-64, discussion 764-6
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  • [Title] 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 PETs or visualized nonfunctioning PETs larger than 1 cm in size on imaging were operated.
  • RESULTS: Twenty-six genetically confirmed MEN1 patients underwent duodenopancreatic resection for functioning (n = 17) or nonfunctioning (n = 9) PETs.
  • The surgical approach was selected based on the type, location, and size of PETs.
  • Four Zollinger-Ellison syndrome (ZES) patients required pylorus preserving pancreaticoduodenectomy (PPPD) as initial or redo procedure, 20 patients underwent other duodenopancreatic resections, and 2 patients had simple enucleations of PETs.
  • CONCLUSIONS: Early and aggressive surgery of PETs in MEN1 patients prevents the development of liver metastases, which are the most life-threatening determinant.
  • PPPD might be the procedure of choice for MEN1-ZES, which has to be proven in large scale studies.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1 / surgery. Pancreatic Neoplasms / surgery. Pancreaticoduodenectomy / methods

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  • (PMID = 16327485.001).
  • [ISSN] 0003-4932
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC1409888
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88. Amano H, Yamada T, Jujoh T, Kuroda F, Sakao S, Tada Y, Kurosu K, Kasahara Y, Tanabe N, Takiguchi Y, Tatsumi K: [Case of thymic carcinoid associated with multiple endocrine neoplasia type I treated effectively with chemotherapy]. Nihon Kokyuki Gakkai Zasshi; 2010 Nov;48(11):855-9
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  • [Title] [Case of thymic carcinoid associated with multiple endocrine neoplasia type I treated effectively with chemotherapy].
  • He had a past history of hyperparathyroidism, for which he underwent surgery in 1994, and also had a family history in that his sister had multiple endocrine neoplasia type 1 (MEN1).
  • He was given a diagnosis of MEN I on genetic testing.
  • Although there are some cases of MEN-related thymoma treated by surgery, a case which successfully responded to chemotherapy alone is thought to be extremely rare.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoid Tumor / complications. Carcinoid Tumor / drug therapy. Multiple Endocrine Neoplasia Type 1 / complications. Thymus Neoplasms / complications. Thymus Neoplasms / drug therapy


89. Lourenço DM Jr, Toledo RA, Coutinho FL, Margarido LC, Siqueira SA, dos Santos MA, Montenegro FL, Machado MC, Toledo SP: The impact of clinical and genetic screenings on the management of the multiple endocrine neoplasia type 1. Clinics (Sao Paulo); 2007 Aug;62(4):465-76
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The impact of clinical and genetic screenings on the management of the multiple endocrine neoplasia type 1.
  • PURPOSE: To perform clinical and genetic screening for multiple endocrine neoplasia type 1 (MEN1) in patients at the Academic Hospital of the University of São Paulo School of Medicine, and to analyze its impact on clinical management of patients with MEN1.
  • METHODS: The clinical diagnosis of MEN1 was made in accordance with the Consensus on multiple endocrine neoplasias.
  • Mutation analysis of the entire MEN1 tumor suppressor gene and genetic screening of at-risk family members were performed by direct sequencing.
  • To analyze the implementation of genetic diagnosis, the studied patients were separated into 3 groups: MEN1 index cases (group I), clinically diagnosed MEN1 cases (group II), and genetically diagnosed MEN1 cases (group III).
  • We identified 12 different MEN1 mutations.
  • Fifty-two MEN1 cases were identified: 13 in group I, 28 in group II, and 11 in group III.
  • Patients in groups I and II mostly presented 2 or 3 MEN1-related tumors, while 81.8% of those in group III presented 1 or no MEN1-related tumor.
  • Surveillance for MEN1 mutations allowed the exclusion of 102 noncarriers, including a case of MEN1 phenocopy.
  • CONCLUSION: Our data supports the benefits of clinical and genetic screening for multiple endocrine neoplasia type 1 in the management of this syndrome.
  • [MeSH-major] Genetic Testing. Multiple Endocrine Neoplasia Type 1 / genetics. Mutation. Proto-Oncogene Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Follow-Up Studies. Genetic Predisposition to Disease. Germ-Line Mutation. Humans. Middle Aged. Polymerase Chain Reaction. Prevalence. Risk Factors


90. Trouillas J, Labat-Moleur F, Sturm N, Kujas M, Heymann MF, Figarella-Branger D, Patey M, Mazucca M, Decullier E, Vergès B, Chabre O, Calender A, Groupe d'études des Tumeurs Endocrines: 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 Apr;32(4):534-43
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  • [Title] 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.
  • Patients affected by the multiple endocrine neoplasia type I syndrome (MEN1) display a high incidence of pituitary adenomas, though it is still unknown whether these pituitary tumors have specific pathologic features that would distinguish them from sporadic pituitary adenomas.
  • Pituitary tissue specimens of 77 MEN1 patients from the GTE (Groupe d'étude des Tumeurs Endocrines) register were compared with unselected 2509 non-MEN1 sporadic pituitary tumors and also to a control subgroup of 296 cases, where 1 MEN1 tumor was matched with 4 sporadic tumors of the same hormonal immunoprofile.
  • MEN1 tumors were significantly larger and more often invasive by histology.
  • MEN1 patients with large pituitary tumors (grade IV) were younger than non-MEN1 patients.
  • MEN1 tumors had no other characteristic histologic features and no predominance of any one hormone producing subtype.
  • However, plurihormonal adenomas versus monohormonal and nonimmunoreactive adenomas were more frequent in MEN1 tumors (39%) than in the control non-MEN1 group (P = 0.001).
  • Especially, the growth hormone and prolactin plurihormonality with unusual association with follicle-stimulating hormone, luteinizing hormone, or adrenocorticotropic hormone was more frequent in MEN1 tumors.
  • In addition, multiple adenomas were significantly more frequent (4% vs. 0.1%; P < 0.0001), especially prolactin-adrenocorticotropic hormone.
  • Somatotroph hyperplasia, with or without a microadenoma was found in only 3 MEN1 patients, with growth hormone-releasing hormone hypersecretion by a pancreatic tumor in 2 of them.
  • 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.
  • [MeSH-major] Adenoma / pathology. Multiple Endocrine Neoplasia Type 1 / pathology. Pituitary Gland / pathology. Pituitary Neoplasms / pathology


91. Marek LR, Kottemann MC, Glazer PM, Bale AE: MEN1 and FANCD2 mediate distinct mechanisms of DNA crosslink repair. DNA Repair (Amst); 2008 Mar 1;7(3):476-86
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  • [Title] MEN1 and FANCD2 mediate distinct mechanisms of DNA crosslink repair.
  • Cells mutant for multiple endocrine neoplasia type I (MEN1) or any of the Fanconi anemia (FA) genes are hypersensitive to the killing effects of crosslinking agents, but the precise roles of these genes in the response to interstrand crosslinks (ICLs) are unknown.
  • To determine if MEN1 and the FA genes function cooperatively in the same repair process or in distinct repair processes, we exploited Drosophila genetics to compare the mutation frequency and spectra of MEN1 and FANCD2 mutants and to perform genetic interaction studies.
  • We created a novel in vivo reporter system in Drosophila based on the supF gene and showed that MEN1 mutant flies were extremely prone to single base deletions within a homopolymeric tract.
  • FANCD2 mutants, on the other hand, had a mutation frequency and spectrum similar to wild type using this assay.
  • In contrast to the supF results, both MEN1 and FANCD2 mutants were hypermutable using a different assay based on the lats tumor suppressor gene.
  • The lats assay showed that FANCD2 mutants had a high frequency of large deletions, which the supF assay was not able to detect, while large deletions were rare in MEN1 mutants.
  • Genetic interaction studies showed that neither overexpression nor loss of MEN1 modified the ICL sensitivity of FANCD2 mutants.
  • The strikingly different mutation spectra of MEN1 and FANCD2 mutants together with lack of evidence for genetic interaction between these genes indicate MEN1 plays an essential role in ICL repair distinct from the Fanconi anemia genes.

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  • (PMID = 18258493.001).
  • [ISSN] 1568-7864
  • [Journal-full-title] DNA repair
  • [ISO-abbreviation] DNA Repair (Amst.)
  • [Language] ENG
  • [Grant] United States / NIGMS NIH HHS / GM / GM066079-02; United States / NIGMS NIH HHS / GM / R01 GM066079-01; United States / NIGMS NIH HHS / GM / R01 GM066079-02; United States / NIGMS NIH HHS / GM / R01 GM066079; United States / NIGMS NIH HHS / GM / GM066079-03; United States / NIGMS NIH HHS / GM / GM066079-01; United States / NIGMS NIH HHS / GM / R01 GM66079; United States / NIGMS NIH HHS / GM / R01 GM066079-03
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Cross-Linking Reagents; 0 / Drosophila Proteins; 0 / Fancd2 protein, Drosophila; 0 / Fanconi Anemia Complementation Group D2 Protein; 0 / Mnn1 protein, Drosophila; 0 / Mutagens
  • [Other-IDs] NLM/ NIHMS41900; NLM/ PMC2277339
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92. Whitley SA, Moyes VJ, Park KM, Brooke AM, Grossman AB, Chew SL, Rockall AG, Monson JP, Reznek RH: The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1. Eur J Endocrinol; 2008 Dec;159(6):819-24
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  • [Title] The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1.
  • AIMS: To review the morphology of the adrenal glands in multiple endocrine neoplasia type 1 (MEN1) on computed tomography (CT) to compare the results with established normal values for adrenal size and nodularity and to correlate adrenal size with serum cortisol secretory dynamics.
  • MATERIALS AND METHODS: Two observers independently reviewed the adrenal CT in 28 patients with MEN1, measuring the maximum width of the body of the gland and the medial and lateral limbs.
  • Following exclusion of known cases of Cushing's syndrome, adrenal gland size was compared with previously documented normative data.
  • RESULTS: Comparison of mean adrenal size in MEN1 patients with normative data showed that the adrenal limbs were significantly larger in MEN1 than normal (P<0.0001 in all four limbs).
  • CONCLUSIONS: In patients with MEN1, adrenal limb hyperplasia and adrenal nodules are significantly more common than in the normal population, a phenomenon not previously documented in a quantitative manner.


93. Vierimaa O, Ebeling TM, Kytölä S, Bloigu R, Eloranta E, Salmi J, Korpi-Hyövälti E, Niskanen L, Orvola A, Elovaara E, Gynther A, Sane T, Välimäki M, Ignatius J, Leisti J, Salmela PI: Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype phenotype correlation. Eur J Endocrinol; 2007 Sep;157(3):285-94
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype phenotype correlation.
  • OBJECTIVE: The existence of genotype-phenotype correlation in multiple endocrine neoplasia type 1 (MEN1) is controversial.
  • Two founder mutations of the MEN1 gene in Northern Finland gave us an opportunity to compare clinical features among heterozygotes of different mutations.
  • DESIGN AND METHODS: Study cohort included 82 MEN1 heterozygotes who were tested for MEN1 during the years 1982-2001.
  • Medical records were reviewed for manifestations of MEN1, other tumours and cause of death by the end of August 2003.
  • Logistic regression analysis was used in evaluating the impact of age, gender and mutational status of affected heterozygotes on the likelihood of developing manifestations of MEN1.
  • Except for pituitary adenoma and nonfunctional pancreatic tumour (NFPT), age was a risk factor for all the disease manifestations.

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  • (PMID = 17766710.001).
  • [ISSN] 0804-4643
  • [Journal-full-title] European journal of endocrinology
  • [ISO-abbreviation] Eur. J. Endocrinol.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Codon, Nonsense; 0 / MEN1 protein, human; 0 / Proto-Oncogene Proteins
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94. Nishiuchi T, Imachi H, Murao K, Fujiwara M, Muraoka T, Kikuchi F, Nishiuchi Y, Kushida Y, Haba R, Ishida T: Co-existence of glucagonoma with recurrent insulinoma in a patient with multiple endocrine neoplasia-type 1 (MEN-1). Endocrine; 2009 Aug;36(1):20-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Co-existence of glucagonoma with recurrent insulinoma in a patient with multiple endocrine neoplasia-type 1 (MEN-1).
  • Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by tumors of the parathyroid glands, the anterior pituitary, and the endocrine pancreas.
  • Our patient was a 58-year-old man who manifested typical features of MEN-1 including primary hyperparathyroidism, lung carcinoid, and lipomas and insulinoma.
  • We found a germline mutation of the MEN1 gene (E45G, exon 2) in this patient.
  • Although it is uncommon for patients with MEN1 to exhibit insulinoma and glucagonoma, this case suggests the need for careful analysis of pancreatic tumors in patients with MEN1.
  • [MeSH-major] Glucagonoma / pathology. Insulinoma / pathology. Multiple Endocrine Neoplasia Type 1 / pathology. Pancreatic Neoplasms / pathology


95. Scacheri PC, Davis S, Odom DT, Crawford GE, Perkins S, Halawi MJ, Agarwal SK, Marx SJ, Spiegel AM, Meltzer PS, Collins FS: Genome-wide analysis of menin binding provides insights into MEN1 tumorigenesis. PLoS Genet; 2006 Apr;2(4):e51
SciCrunch. ArrayExpress: Data: Microarray .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Genome-wide analysis of menin binding provides insights into MEN1 tumorigenesis.
  • Multiple endocrine neoplasia type I (MEN1) is a familial cancer syndrome characterized primarily by tumors of multiple endocrine glands.
  • The gene for MEN1 encodes a ubiquitously expressed tumor suppressor protein called menin.
  • Menin was recently shown to interact with several components of a trithorax family histone methyltransferase complex including ASH2, Rbbp5, WDR5, and the leukemia proto-oncoprotein MLL.
  • To elucidate menin's role as a tumor suppressor and gain insights into the endocrine-specific tumor phenotype in MEN1, we mapped the genomic binding sites of menin, MLL1, and Rbbp5, to approximately 20,000 promoters in HeLa S3, HepG2, and pancreatic islet cells using the strategy of chromatin-immunoprecipitation coupled with microarray analysis.
  • Our findings expand the realm of menin-targeted genes several hundred-fold beyond that previously described and provide potential insights to the endocrine tumor bias observed in MEN1 patients.

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  • (PMID = 16604156.001).
  • [ISSN] 1553-7404
  • [Journal-full-title] PLoS genetics
  • [ISO-abbreviation] PLoS Genet.
  • [Language] eng
  • [Grant] United States / NIDDK NIH HHS / DK / K25 DK070813; United States / NIDDK NIH HHS / DK / R01 DK068655; United States / Intramural NIH HHS / / ; United States / NIDDK NIH HHS / DK / K25-DK070813; United States / NIDDK NIH HHS / DK / R01-DK068655
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Homeodomain Proteins; 0 / MEN1 protein, human; 0 / MLL protein, human; 0 / MNX1 protein, human; 0 / Nuclear Proteins; 0 / Proto-Oncogene Proteins; 0 / RBBP5 protein, human; 0 / Transcription Factors; 149025-06-9 / Myeloid-Lymphoid Leukemia Protein; EC 2.1.1.43 / Histone-Lysine N-Methyltransferase
  • [Other-IDs] NLM/ PMC1428788
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96. Sakurai A, Murakami A, Sano K, Uchino S, Fukushima Y: Unusual clinical and pathological presentation of a neuroendocrine tumor in a patient with multiple endocrine neoplasia type 1. Endocr J; 2009;56(7):887-95
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Unusual clinical and pathological presentation of a neuroendocrine tumor in a patient with multiple endocrine neoplasia type 1.
  • Neuroendocrine tumors develop in various organs in patients with multiple endocrine neoplasia type 1 (MEN1).
  • We have experienced a patient with MEN1 who have developed an "ectopic" retroperitoneal neuroendocrine tumor.
  • Genetic analysis of the MEN1 gene in tumor cells revealed a somatic mutation in exon 9 as well as a germline mutation in exon 10.
  • Immunohistochemical staining with an anti-menin antibody revealed that wild-type menin is not expressed in tumor cells.
  • These results confirmed the inactivation of the MEN1 gene as a genetic cause of an ectopically developed neuroendocrine tumor in a patient with MEN1.
  • [MeSH-major] Carcinoid Tumor / pathology. Multiple Endocrine Neoplasia Type 1 / pathology. Neuroendocrine Tumors / pathology
  • [MeSH-minor] Cyclin-Dependent Kinase Inhibitor p27 / genetics. Female. Germ-Line Mutation. Humans. Loss of Heterozygosity. Middle Aged. Proto-Oncogene Proteins / genetics. Retroperitoneal Neoplasms / pathology


97. Kouvaraki MA, Shapiro SE, Cote GJ, Lee JE, Yao JC, Waguespack SG, Gagel RF, Evans DB, Perrier ND: Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg; 2006 May;30(5):643-53
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1.
  • INTRODUCTION: Pancreatic endocrine tumors (PETs) occur in at least 50% of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of disease-specific mortality.
  • However, the timing and extent of surgery for MEN1-related PETs is controversial owing to the indolent tumor growth seen in most patients and the desire to avoid complications associated with insulin dependence.
  • To help resolve this controversy, we retrospectively analyzed the clinical characteristics, surgical treatment, and clinical outcome of patients with MEN1-related PETs.
  • METHODS: All patients had histologic or radiographic confirmation of a PET in the setting of MEN1.
  • Disease progression was defined radiographically as the development of new pancreatic tumors or distant metastases.
  • RESULTS: We identified 98 patients with MEN1, 55 (56%) of whom had PETs, including 27 women and 28 men with a median age of 37 years (range 8-69 years) at the time of diagnosis.
  • Recurrent disease developed in the residual pancreas in 7 (20%) of 35 at-risk patients a median of 7.8 years after the first operation, and distant metastases occurred in 5 (14 %) of 36 surgically treated patients without distant metastasis (2 patients had distant metastases when surgery on the primary tumor was performed) at a median of 2.7 years following surgery.
  • At last follow-up, 16 (29%) of 55 patients with PETs had died, 12 (22%) were alive with disease, 26 (47%) were alive without evidence of disease, and 1 (2%) was lost to follow-up.
  • The median OS was 19.5 years (range 13-26 years) and was significantly longer for patients who had functioning PETs versus those with nonfunctioning tumors (P = 0.0007), for patients who underwent surgical resection of their PETs versus those who did not (P = 0.0043), and for patients with localized versus metastatic PETs at the time of diagnosis (P < 0.0001).
  • CONCLUSIONS: Our data suggest that early diagnosis and surgical excision of MEN1-related PETs improves survival.
  • However, translating these data into a surveillance strategy for the early detection of PETs is complex owing to the potential morbidity of pancreatic resection and the risk of long-term insulin dependence.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 1. Pancreatic Neoplasms / surgery


98. Agarwal SK, Mateo CM, Marx SJ: Rare germline mutations in cyclin-dependent kinase inhibitor genes in multiple endocrine neoplasia type 1 and related states. J Clin Endocrinol Metab; 2009 May;94(5):1826-34
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rare germline mutations in cyclin-dependent kinase inhibitor genes in multiple endocrine neoplasia type 1 and related states.
  • CONTEXT: Germline mutation in the MEN1 gene is the usual cause of multiple endocrine neoplasia type 1 (MEN1).
  • However, the prevalence of identifiable germline MEN1 mutations in familial MEN1 cases is only 70%.
  • OBJECTIVE: The aim of the study was to investigate cases of MEN1 or related states for germline mutations in all CDKI genes.
  • METHODS: A total of 196 consecutive index cases were selected with clear or suspected MEN1 and no identifiable germline MEN1 mutation.
  • No characteristic clinical subtype related to MEN1 was identified among the seven index cases and their families.
  • CONCLUSION: Rare germline mutation in any among four (p15, p18, p21, and p27) of the seven CDKIs is a probable cause of MEN1 or of some related states.


99. Lee CH, Tseng LM, Chen JY, Hsiao HY, Yang AH: Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates. Ann Surg Oncol; 2006 Jan;13(1):103-9
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates.
  • BACKGROUND: To evaluate the outcomes in different surgical modalities for primary hyperparathyroidism in multiple endocrine neoplasia type 1 (MEN1) patients, intraoperative findings from a single surgeon were studied to investigate a potentially improved modality of parathyroidectomy (PTx).
  • RESULTS: There were 7 men and 15 women, aged 22 to 67 years (average, 43 years).
  • Sixteen had familial and six had sporadic MEN1.
  • CONCLUSIONS: Primary hyperparathyroidism in our MEN1 patients was less aggressive than that reported in the literature.
  • [MeSH-major] Hyperparathyroidism / surgery. Multiple Endocrine Neoplasia Type 1 / complications


100. Thomas-Marques L, Murat A, Delemer B, Penfornis A, Cardot-Bauters C, Baudin E, Niccoli-Sire P, Levoir D, Choplin Hdu B, Chabre O, Jovenin N, Cadiot G, Groupe des Tumeurs Endocrines (GTE): Prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. Am J Gastroenterol; 2006 Feb;101(2):266-73
NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1.
  • BACKGROUND: The frequency of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1 (MEN1) remains unknown.
  • PATIENTS AND METHODS: MEN1 patients without functioning pancreatic involvement underwent systematic pancreaticoduodenal EUS in nine GTE (Groupe des Tumeurs Endocrines) centers.
  • MEN1 had been diagnosed 3 [0-20] yr earlier, notably by genetic screening for 26 (51%) with asymptomatic disease.
  • CONCLUSION: The frequency of nonfunctioning pancreatic endocrine tumors is higher (54.9%) than previously thought.
  • Pancreatic EUS should be performed once MEN1 is diagnosed to monitor disease progression.
  • [MeSH-major] Duodenal Neoplasms / ultrasonography. Endosonography. Multiple Endocrine Neoplasia Type 1 / ultrasonography. Pancreatic Neoplasms / ultrasonography
  • [MeSH-minor] Adolescent. Adult. Aged. Biomarkers, Tumor / blood. Diagnosis, Differential. Disease Progression. Female. Follow-Up Studies. Gastrins / blood. Glucagon / blood. Humans. Incidence. Male. Middle Aged. Pancreatic Polypeptide / blood. Peptides / blood. Prospective Studies. Severity of Illness Index. Vasoactive Intestinal Peptide / blood






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