Skip to main content

Advertisement

SpringerLink
Log in

Care for patients with multiple endocrine neoplasia type 1: the current evidence base

  • Published:
Familial Cancer Aims and scope Submit manuscript

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is a rare disease caused by mutations in the MEN1 gene on chromosome 11. It is characterized by the occurrence of primary hyperparathyroidism (pHPT), duodenopancreatic neuroendocrine tumours (pNET), pituitary tumours (PIT), adrenal adenomas (ADR) and neuroendocrine tumours (NET) of the stomach, bronchus and thymus. MEN1 is a syndrome with high penetrance and high morbidity. Malignant NETs are the most important cause of MEN1-related death. Since 1997 the diagnosis can be made by genetic screening. MEN1 is a complex syndrome and the endocrine manifestations cannot be viewed upon as coinciding sporadic tumours. Differences in epidemiology and pathology between MEN1-related tumours and their sporadic counterparts show that a unique approach is needed. Therefore the care for MEN1 patients should be provided by a centre of expertise. Early genetic diagnosis and periodic screening are important pillars of care. For primary hyperparathyroidism surgery is the most important treatment modality, with a subtotal parathyroid gland resection as the procedure of choice. In neuroendocrine tumours surgery also is the most important treatment modality. Selective tumour enucleation has no place in the surgical treatment of MEN1-related pNETs; the exact procedure depends on the functionality of the tumour. In MEN1-associated pituitary and adrenal adenomas, watchful waiting and medical therapy play more important roles. In the twenty-first century new developments will impact the care for MEN1 patients. These developments should be critically evaluated in clinical research with the ultimate goal of optimizing the care for MEN1 patients on an evidence base.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Abbreviations

5-HIAA:

5-Hydroxyindolacetic acid

ADR:

Adrenal adenoma

AIP:

Aryl hydrocarbon receptor-interacting protein

CDK:

Cyclin-dependent kinase

CDKN:

Cyclin-dependent kinase inhibitor

CgA:

Chromogranin A

CT:

Computed tomography

DHEAS:

Dehydroepiandrosterone sulphate

DNA:

Deoxyribonucleic acid

DPR:

Distal pancreatic resection

ECL:

Enterochromaffin-like

EUS:

Endoscopic ultrasound

IGF-1:

Insulin-like growth factor 1

LOH:

Loss of heterozygosity

MEN1:

Multiple endocrine neoplasia type 1

MEN2:

Multiple endocrine neoplasia type 2

MLPA:

Multiplex ligation-dependent probe amplification

MRI:

Magnetic resonance imaging

mTOR:

Mammalian target of rapamycin

NET:

Neuroendocrine tumour

NF:

Non-functioning

PET:

Positron emission tomography

pHPT:

Primary hyperparathyroidism

PIT:

Pituitary tumour

pNET:

Duodenopancreatic neuroendocrine tumour

PTH:

Parathyroid hormone

SF-36:

Short form 36

SRS:

Somatostatin receptor scintigraphy

TCT:

Transcervical thymectomy

ZES:

Zollinger–Ellison syndrome

References

  1. Erdheim J (1903) Zur normalen und pathologischen Histologie der Glandula thyreoidea, parathyreoidea und Hypophysis. Beitr z path Anat u z allg Path 33:158–236

    Google Scholar 

  2. Underdahl LO, Woolner LB, Black BM (1953) Multiple endocrine adenomas; report of 8 cases in which the parathyroids, pituitary and pancreatic islets were involved. J Clin Endocrinol Metab 13(1):20–47

    CAS  PubMed  Google Scholar 

  3. Castleman B, Towne VW (1953) Case records of the Massachusetts General Hospital weekly clinicopathological exercises: case 39501. N Engl J Med 249(24):990–993

    Google Scholar 

  4. Wermer P (1954) Genetic aspects of adenomatosis of endocrine glands. Am J Med 16(3):363–371

    CAS  PubMed  Google Scholar 

  5. Chandrasekharappa SC, Guru SC, Manickam P et al (1997) Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science 276(5311):404–407

    CAS  PubMed  Google Scholar 

  6. Kouvaraki MA, Lee JE, Shapiro SE et al (2002) Genotype-phenotype analysis in multiple endocrine neoplasia type 1. Arch Surg 137(6):641–647

    PubMed  Google Scholar 

  7. Bassett JH, Forbes SA, Pannett AA et al (1998) Characterization of mutations in patients with multiple endocrine neoplasia type 1. Am J Hum Genet 62(2):232–244

    CAS  PubMed  Google Scholar 

  8. Carty SE, Helm AK, Amico JA et al (1998) The variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1. Surgery 124(6):1106–1113; discussion 13–14

    Google Scholar 

  9. Trump D, Farren B, Wooding C et al (1996) Clinical studies of multiple endocrine neoplasia type 1 (MEN1). QJM 89(9):653–669

    CAS  PubMed  Google Scholar 

  10. Dreijerink KM, van Beek AP, Lentjes EG et al (2005) Acromegaly in a multiple endocrine neoplasia type 1 (MEN1) family with low penetrance of the disease. Eur J Endocrinol 153(6):741–746

    CAS  PubMed  Google Scholar 

  11. Drori-Herishanu L, Horvath A, Nesterova M et al (2009) An Intronic mutation is associated with prolactinoma in a young boy, decreased penetrance in his large family, and variable effects on MEN1 mRNA and protein. Horm Metab Res 41(8):630–634

    CAS  PubMed  Google Scholar 

  12. Dean PG, van Heerden JA, Farley DR et al (2000) Are patients with multiple endocrine neoplasia type I prone to premature death? World J Surg 24(11):1437–1441

    CAS  PubMed  Google Scholar 

  13. Doherty GM, Olson JA, Frisella MM, Lairmore TC, Wells SA Jr, Norton JA (1998) Lethality of multiple endocrine neoplasia type I. World J Surg 22(6):581–586; discussion 6–7

    Google Scholar 

  14. Geerdink EA, Van der Luijt RB, Lips CJ (2003) Do patients with multiple endocrine neoplasia syndrome type 1 benefit from periodical screening? Eur J Endocrinol 149(6):577–582

    CAS  PubMed  Google Scholar 

  15. Goudet P, Murat A, Binquet C et al (2010) 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 34(2):249–255

    PubMed  Google Scholar 

  16. Lemmens I, Van de Ven WJ, Kas K et al (1997) Identification of the multiple endocrine neoplasia type 1 (MEN1) gene. The European consortium on MEN1. Hum Mol Genet 6(7):1177–1183

    CAS  PubMed  Google Scholar 

  17. Larsson C, Skogseid B, Oberg K, Nakamura Y, Nordenskjold M (1988) Multiple endocrine neoplasia type 1 gene maps to chromosome 11 and is lost in insulinoma. Nature 332(6159):85–87

    CAS  PubMed  Google Scholar 

  18. Lemos MC, Thakker RV (2008) Multiple endocrine neoplasia type 1 (MEN1): analysis of 1336 mutations reported in the first decade following identification of the gene. Hum Mutat 29(1):22–32

    CAS  PubMed  Google Scholar 

  19. Turner JJ, Leotlela PD, Pannett AA et al (2002) Frequent occurrence of an intron 4 mutation in multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 87(6):2688–2693

    CAS  PubMed  Google Scholar 

  20. Giraud S, Zhang CX, Serova-Sinilnikova O et al (1998) Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders. Am J Hum Genet 63(2):455–467

    CAS  PubMed  Google Scholar 

  21. Kouvaraki MA, Shapiro SE, Cote GJ et al (2006) Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg 30(5):643

    PubMed  Google Scholar 

  22. Vierimaa O, Ebeling TM, Kytola S et al (2007) Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype–phenotype correlation. Eur J Endocrinol 157(3):285–294

    CAS  PubMed  Google Scholar 

  23. Wautot V, Vercherat C, Lespinasse J et al (2002) Germline mutation profile of MEN1 in multiple endocrine neoplasia type 1: search for correlation between phenotype and the functional domains of the MEN1 protein. Hum Mutat 20(1):35–47

    CAS  PubMed  Google Scholar 

  24. Hao W, Skarulis MC, Simonds WF et al (2004) Multiple endocrine neoplasia type 1 variant with frequent prolactinoma and rare gastrinoma. J Clin Endocrinol Metab 89(8):3776–3784

    PubMed  Google Scholar 

  25. Pannett AA, Kennedy AM, Turner JJ et al (2003) Multiple endocrine neoplasia type 1 (MEN1) germline mutations in familial isolated primary hyperparathyroidism. Clin Endocrinol (Oxf) 58(5):639–646

    CAS  Google Scholar 

  26. Lemos MC, Harding B, Reed AA et al (2009) Genetic background influences embryonic lethality and the occurrence of neural tube defects in Men1 null mice: relevance to genetic modifiers. J Endocrinol 203(1):133–142

    CAS  PubMed  Google Scholar 

  27. Hessman O, Lindberg D, Einarsson A et al (1999) Genetic alterations on 3p, 11q13, and 18q in nonfamilial and MEN 1-associated pancreatic endocrine tumors. Genes Chromosomes Cancer 26(3):258–264

    CAS  PubMed  Google Scholar 

  28. Hughes CM, Rozenblatt-Rosen O, Milne TA et al (2004) Menin associates with a trithorax family histone methyltransferase complex and with the hoxc8 locus. Mol Cell 13(4):587–597

    CAS  PubMed  Google Scholar 

  29. Dreijerink KM, Lips CJ, Timmers HT (2009) Multiple endocrine neoplasia type 1: a chromatin writer’s block. J Intern Med 266(1):53–59

    CAS  PubMed  Google Scholar 

  30. Brandi ML, Gagel RF, Angeli A et al (2001) Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86(12):5658–5671

    CAS  PubMed  Google Scholar 

  31. Ellard S, Hattersley AT, Brewer CM, Vaidya B (2005) Detection of an MEN1 gene mutation depends on clinical features and supports current referral criteria for diagnostic molecular genetic testing. Clin Endocrinol (Oxf) 62(2):169–175

    CAS  Google Scholar 

  32. Tham E, Grandell U, Lindgren E, Toss G, Skogseid B, Nordenskjold M (2007) Clinical testing for mutations in the MEN1 gene in Sweden: a report on 200 unrelated cases. J Clin Endocrinol Metab 92(9):3389–3395

    CAS  PubMed  Google Scholar 

  33. Vierimaa O, Georgitsi M, Lehtonen R et al (2006) Pituitary adenoma predisposition caused by germline mutations in the AIP gene. Science 312(5777):1228–1230

    CAS  PubMed  Google Scholar 

  34. Pellegata NS, Quintanilla-Martinez L, Siggelkow H et al (2006) Germ-line mutations in p27Kip1 cause a multiple endocrine neoplasia syndrome in rats and humans. Proc Natl Acad Sci USA 103(42):15558–15563

    CAS  PubMed  Google Scholar 

  35. Georgitsi M, Raitila A, Karhu A et al (2007) Germline CDKN1B/p27Kip1 mutation in multiple endocrine neoplasia. J Clin Endocrinol Metab 92(8):3321–3325

    CAS  PubMed  Google Scholar 

  36. Georgitsi M, Raitila A, Karhu A et al (2007) Molecular diagnosis of pituitary adenoma predisposition caused by aryl hydrocarbon receptor-interacting protein gene mutations. Proc Natl Acad Sci USA 104(10):4101–4105

    CAS  PubMed  Google Scholar 

  37. Agarwal SK, Mateo CM, Marx SJ (2009) Rare germline mutations in cyclin-dependent kinase inhibitor genes in multiple endocrine neoplasia type 1 and related states. J Clin Endocrinol Metab 94(5):1826–1834

    CAS  PubMed  Google Scholar 

  38. Lourenco-Jr DM, Toledo RA, Coutinho FL et al (2007) The impact of clinical and genetic screenings on the management of the multiple endocrine neoplasia type 1. Clinics 62(4):465–476

    Google Scholar 

  39. Pieterman CR, Schreinemakers JM, Koppeschaar HP et al (2009) Multiple endocrine neoplasia type 1 (MEN1): its manifestations and effect of genetic screening on clinical outcome. Clin Endocrinol (Oxf) 70(4):575–581

    CAS  Google Scholar 

  40. Waldmann J, Fendrich V, Habbe N et al (2009) Screening of patients with multiple endocrine neoplasia type 1 (MEN-1): a critical analysis of its value. World J Surg 33(6):1208–1218

    PubMed  Google Scholar 

  41. Schaaf L, Pickel J, Zinner K et al (2007) Developing effective screening strategies in multiple endocrine neoplasia type 1 (MEN 1) on the basis of clinical and sequencing data of German patients with MEN 1. Exp Clin Endocrinol Diabetes 115(8):509–517

    CAS  PubMed  Google Scholar 

  42. Berglund G, Liden A, Hansson MG, Oberg K, Sjoden PO, Nordin K (2003) Quality of life in patients with multiple endocrine neoplasia type 1 (MEN 1). Fam Cancer 2(1):27–33

    CAS  PubMed  Google Scholar 

  43. Wilson SD, Krzywda EA, Zhu YR et al (2008) The influence of surgery in MEN-1 syndrome: observations over 150 years. Surgery 144(4):695–701; discussion 2

    Google Scholar 

  44. Eller-Vainicher C, Chiodini I, Battista C et al (2009) Sporadic and MEN1-related primary hyperparathyroidism: differences in clinical expression and severity. J Bone Miner Res 24(8):1404–1410

    PubMed  Google Scholar 

  45. Norton JA, Venzon DJ, Berna MJ et al (2008) Prospective study of surgery for primary hyperparathyroidism (HPT) in multiple endocrine neoplasia-type 1 and Zollinger–Ellison syndrome: long-term outcome of a more virulent form of HPT. Ann Surg 247(3):501–510

    PubMed  Google Scholar 

  46. Dotzenrath C, Cupisti K, Goretzki PE et al (2001) Long-term biochemical results after operative treatment of primary hyperparathyroidism associated with multiple endocrine neoplasia types I and IIa: is a more or less extended operation essential? Eur J Surg 167(3):173–178

    CAS  PubMed  Google Scholar 

  47. Elaraj DM, Skarulis MC, Libutti SK et al (2003) Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery 134(6):858–864; discussion 64–65

    Google Scholar 

  48. Hellman P, Skogseid B, Oberg K, Juhlin C, Akerstrom G, Rastad J (1998) Primary and reoperative parathyroid operations in hyperparathyroidism of multiple endocrine neoplasia type 1. Surgery 124(6):993–999

    CAS  PubMed  Google Scholar 

  49. Hubbard JG, Sebag F, Maweja S, Henry JF (2006) Subtotal parathyroidectomy as an adequate treatment for primary hyperparathyroidism in multiple endocrine neoplasia type 1. Arch Surg 141(3):235–239

    PubMed  Google Scholar 

  50. Kraimps JL, Duh QY, Demeure M, Clark OH (1992) Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery 112(6):1080–1086; discussion 6–8

    Google Scholar 

  51. Lambert LA, Shapiro SE, Lee JE et al (2005) Surgical treatment of hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Arch Surg 140(4):374–382

    PubMed  Google Scholar 

  52. Lee CH, Tseng LM, Chen JY, Hsiao HY, Yang AH (2006) Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates. Ann Surg Oncol 13(1):103–109

    PubMed  Google Scholar 

  53. Malmaeus J, Benson L, Johansson H et al (1986) Parathyroid surgery in the multiple endocrine neoplasia type I syndrome: choice of surgical procedure. World J Surg 10(4):668–672

    CAS  PubMed  Google Scholar 

  54. Burgess JR, David R, Parameswaran V, Greenaway TM, Shepherd JJ (1998) The outcome of subtotal parathyroidectomy for the treatment of hyperparathyroidism in multiple endocrine neoplasia type 1. Arch Surg 133(2):126–129

    CAS  PubMed  Google Scholar 

  55. Tonelli F, Marcucci T, Fratini G, Tommasi MS, Falchetti A, Brandi ML (2007) Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1? Ann Surg 246(6):1075–1082

    PubMed  Google Scholar 

  56. Goudet P, Cougard P, Verges B et al (2001) Hyperparathyroidism in multiple endocrine neoplasia type I: surgical trends and results of a 256-patient series from Groupe D’etude des Neoplasies Endocriniennes Multiples Study Group. World J Surg 25(7):886–890

    CAS  PubMed  Google Scholar 

  57. Powell AC, Alexander HR, Pingpank JF et al (2008) The utility of routine transcervical thymectomy for multiple endocrine neoplasia 1-related hyperparathyroidism. Surgery 144(6):878–883; discussion 83–84

    Google Scholar 

  58. Bergenfelz AO, Hellman P, Harrison B, Sitges-Serra A, Dralle H (2009) Positional statement of the European society of endocrine surgeons (ESES) on modern techniques in pHPT surgery. Langenbecks Arch Surg 394(5):761–764

    PubMed  Google Scholar 

  59. Hessman O, Stalberg P, Sundin A et al (2008) High success rate of parathyroid reoperation may be achieved with improved localization diagnosis. World J Surg 32(5):774–781; discussion 82–83

    Google Scholar 

  60. Kivlen MH, Bartlett DL, Libutti SK et al (2001) Reoperation for hyperparathyroidism in multiple endocrine neoplasia type 1. Surgery 130(6):991–998

    CAS  PubMed  Google Scholar 

  61. Yen TW, Wang TS, Doffek KM, Krzywda EA, Wilson SD (2008) Reoperative parathyroidectomy: an algorithm for imaging and monitoring of intraoperative parathyroid hormone levels that results in a successful focused approach. Surgery 144(4):611–619; discussion 9–21

    Google Scholar 

  62. Faggiano A, Tavares LB, Tauchmanova L et al (2008) Effect of treatment with depot somatostatin analogue octreotide on primary hyperparathyroidism (PHP) in multiple endocrine neoplasia type 1 (MEN1) patients. Clin Endocrinol (Oxf) 69(5):756–762

    CAS  Google Scholar 

  63. Falchetti A, Cilotti A, Vaggelli L et al (2008) A patient with MEN1-associated hyperparathyroidism, responsive to cinacalcet. Nat Clin Pract Endocrinol Metab 4(6):351–357

    PubMed  Google Scholar 

  64. Veldman MW, Reading CC, Farrell MA et al (2008) Percutaneous parathyroid ethanol ablation in patients with multiple endocrine neoplasia type 1. AJR Am J Roentgenol 191(6):1740–1744

    PubMed  Google Scholar 

  65. Duh QY, Hybarger CP, Geist R et al (1987) Carcinoids associated with multiple endocrine neoplasia syndromes. Am J Surg 154(1):142–148

    CAS  PubMed  Google Scholar 

  66. Benson L, Ljunghall S, Akerstrom G, Oberg K (1987) Hyperparathyroidism presenting as the first lesion in multiple endocrine neoplasia type 1. Am J Med 82(4):731–737

    CAS  PubMed  Google Scholar 

  67. Burgess JR, Harle RA, Tucker P et al (1996) Adrenal lesions in a large kindred with multiple endocrine neoplasia type 1. Arch Surg 131(7):699–702

    CAS  PubMed  Google Scholar 

  68. Shepherd JJ (1991) The natural history of multiple endocrine neoplasia type 1. Highly uncommon or highly unrecognized? Arch Surg 126(8):935–952

    CAS  PubMed  Google Scholar 

  69. Skogseid B, Eriksson B, Lundqvist G et al (1991) Multiple endocrine neoplasia type 1: a 10-year prospective screening study in four kindreds. J Clin Endocrinol Metab 73(2):281–287

    CAS  PubMed  Google Scholar 

  70. Skogseid B, Larsson C, Lindgren PG et al (1992) Clinical and genetic features of adrenocortical lesions in multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 75(1):76–81

    CAS  PubMed  Google Scholar 

  71. Skogseid B, Oberg K, Eriksson B et al (1996) Surgery for asymptomatic pancreatic lesion in multiple endocrine neoplasia type I. World J Surg 20(7):872–876; discussion 7

    Google Scholar 

  72. Vasen HF, Lamers CB, Lips CJ (1989) Screening for the multiple endocrine neoplasia syndrome type I. A study of 11 kindreds in The Netherlands. Arch Intern Med 149(12):2717–2722

    CAS  PubMed  Google Scholar 

  73. Verges B, Boureille F, Goudet P et al (2002) Pituitary disease in MEN type 1 (MEN1): data from the France-Belgium MEN1 multicenter study. J Clin Endocrinol Metab 87(2):457–465

    CAS  PubMed  Google Scholar 

  74. Jensen RT, Berna MJ, Bingham DB, Norton JA (2008) Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer 113(7 suppl):1807–1843

    PubMed  Google Scholar 

  75. Donow C, Pipeleers-Marichal M, Schroder S, Stamm B, Heitz PU, Kloppel G (1991) Surgical pathology of gastrinoma. Site, size, multicentricity, association with multiple endocrine neoplasia type 1, and malignancy. Cancer 68(6):1329–1334

    CAS  PubMed  Google Scholar 

  76. Pipeleers-Marichal M, Somers G, Willems G et al (1990) Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger–Ellison syndrome. N Engl J Med 322(11):723–727

    CAS  PubMed  Google Scholar 

  77. Garbrecht N, Anlauf M, Schmitt A et al (2008) Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocr Relat Cancer 15(1):229–241

    PubMed  Google Scholar 

  78. Roggli VL, Judge DM, McGavran MH (1979) Duodenal glucagonoma: a case report. Hum Pathol 10(3):350–353

    CAS  PubMed  Google Scholar 

  79. Triponez F, Dosseh D, Goudet P et al (2006) Epidemiology data on 108 MEN 1 patients from the GTE with isolated nonfunctioning tumors of the pancreas. Ann Surg 243(2):265

    PubMed  Google Scholar 

  80. Anlauf M, Schlenger R, Perren A et al (2006) Microadenomatosis of the endocrine pancreas in patients with and without the multiple endocrine neoplasia type 1 syndrome. Am J Surg Pathol 30(5):560–574

    PubMed  Google Scholar 

  81. Levy-Bohbot N, Merle C, Goudet P et al (2004) Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas: study from the GTE (Groupe des Tumeurs Endocrines) registry. Gastroenterol Clin Biol 28(11):1075–1081

    PubMed  Google Scholar 

  82. Hellman P, Hennings J, Akerstrom G, Skogseid B (2005) Endoscopic ultrasonography for evaluation of pancreatic tumours in multiple endocrine neoplasia type 1. Br J Surg 92(12):1508–1512

    CAS  PubMed  Google Scholar 

  83. Langer P, Kann PH, Fendrich V et al (2004) Prospective evaluation of imaging procedures for the detection of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. World J Surg 28(12):1317–1322

    PubMed  Google Scholar 

  84. Thomas-Marques L, Murat A, Delemer B et al (2006) Prospective endoscopic ultrasonographic evaluation of the frequency of nonfunctioning pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. Am J Gastroenterol 101(2):266–273

    PubMed  Google Scholar 

  85. Wamsteker EJ, Gauger PG, Thompson NW, Scheiman JM (2003) EUS detection of pancreatic endocrine tumors in asymptomatic patients with type 1 multiple endocrine neoplasia. Gastrointest Endosc 58(4):531–535

    PubMed  Google Scholar 

  86. Sundin A, Vullierme MP, Kaltsas G, Plockinger U (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: radiological examinations. Neuroendocrinology 90(2):167–183

    CAS  PubMed  Google Scholar 

  87. Yim JH, Siegel BA, DeBenedetti MK, Norton JA, Lairmore TC, Doherty GM (1998) Prospective study of the utility of somatostatin-receptor scintigraphy in the evaluation of patients with multiple endocrine neoplasia type 1. Surgery 124(6):1037–1042

    CAS  PubMed  Google Scholar 

  88. Gibril F, Jensen RT (2004) Diagnostic uses of radiolabelled somatostatin receptor analogues in gastroenteropancreatic endocrine tumours. Dig Liver Dis 36(suppl 1):S106–S120

    CAS  PubMed  Google Scholar 

  89. Schillaci O, Spanu A, Scopinaro F et al (2003) Somatostatin receptor scintigraphy in liver metastasis detection from gastroenteropancreatic neuroendocrine tumors. J Nucl Med 44(3):359–368

    CAS  PubMed  Google Scholar 

  90. Adams S, Baum R, Rink T, Schumm-Drager PM, Usadel KH, Hor G (1998) Limited value of fluorine-18 fluorodeoxyglucose positron emission tomography for the imaging of neuroendocrine tumours. Eur J Nucl Med 25(1):79–83

    CAS  PubMed  Google Scholar 

  91. Binderup T, Knigge U, Loft A et al (2010) Functional imaging of neuroendocrine tumors: a head-to-head comparison of somatostatin receptor scintigraphy, 123I-MIBG scintigraphy, and 18F-FDG PET. J Nucl Med 51(5):704–712

    PubMed  Google Scholar 

  92. Ambrosini V, Tomassetti P, Castellucci P et al (2008) Comparison between 68Ga-DOTA-NOC and 18F-DOPA PET for the detection of gastro-entero-pancreatic and lung neuro-endocrine tumours. Eur J Nucl Med Mol Imaging 35(8):1431–1438

    CAS  PubMed  Google Scholar 

  93. Orlefors H, Sundin A, Garske U et al (2005) Whole-body (11)C-5-hydroxytryptophan positron emission tomography as a universal imaging technique for neuroendocrine tumors: comparison with somatostatin receptor scintigraphy and computed tomography. J Clin Endocrinol Metab 90(6):3392–3400

    CAS  PubMed  Google Scholar 

  94. Jackson JE (2005) Angiography and arterial stimulation venous sampling in the localization of pancreatic neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 19(2):229–239

    PubMed  Google Scholar 

  95. Anlauf M, Bauersfeld J, Raffel A et al (2009) Insulinomatosis: a multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia. Am J Surg Pathol 33(3):339–346

    PubMed  Google Scholar 

  96. Cougard P, Goudet P, Peix JL et al (2000) Insulinomas associated with multiple endocrine neoplasia type 1. Report of a series of 44 cases by ‘the groupe d’etudes des neoplasies endocriniennes multiples type 1’ (GENEM). Ann Chir 125(2):118–123

    CAS  PubMed  Google Scholar 

  97. O’Riordain DS, O’Brien T, van Heerden JA, Service FJ, Grant CS (1994) Surgical management of insulinoma associated with multiple endocrine neoplasia type I. World J Surg 18(4):488

    PubMed  Google Scholar 

  98. Rasbach DA, van Heerden JA, Telander RL, Grant CS, Carney JA (1985) Surgical management of hyperinsulinism in the multiple endocrine neoplasia, type 1 syndrome. Arch Surg 120(5):584

    CAS  PubMed  Google Scholar 

  99. Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT (2001) Comparison of surgical results in patients with advanced and limited disease with multiple endocrine neoplasia type 1 and Zollinger–Ellison syndrome. Ann Surg 234(4):495

    CAS  PubMed  Google Scholar 

  100. Norton JA, Fraker DL, Alexander HR et al (1999) Surgery to cure the Zollinger–Ellison syndrome. N Engl J Med 341(9):635

    CAS  PubMed  Google Scholar 

  101. Nikou GC, Toubanakis C, Nikolaou P et al (2005) Gastrinomas associated with MEN-1 syndrome: new insights for the diagnosis and management in a series of 11 patients. Hepatogastroenterology 52(66):1668

    PubMed  Google Scholar 

  102. Bartsch DK, Fendrich V, Langer P, Celik I, Kann PH, Rothmund M (2005) Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Ann Surg 242(6):757

    PubMed  Google Scholar 

  103. Thompson NW (1992) Surgical treatment of the endocrine pancreas and Zollinger–Ellison syndrome in the MEN 1 syndrome. Henry Ford Hosp Med J 40(3–4):195

    CAS  PubMed  Google Scholar 

  104. Thompson NW (1995) The surgical management of hyperparathyroidism and endocrine disease of the pancreas in the multiple endocrine neoplasia type 1 patient. J Intern Med 238(3):269

    CAS  PubMed  Google Scholar 

  105. Thompson NW (1998) Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger–Ellison syndrome, hypoglycaemia or both. J Intern Med 243(6):495–500

    CAS  PubMed  Google Scholar 

  106. Tonelli F, Fratini G, Nesi G et al (2006) Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias. Ann Surg 244(1):61

    PubMed  Google Scholar 

  107. Mignon M, Ruszniewski P, Podevin P et al (1993) Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia type I. World J Surg 17(4):489

    CAS  PubMed  Google Scholar 

  108. Ruszniewski P, Podevin P, Cadiot G et al (1993) Clinical, anatomical, and evolutive features of patients with the Zollinger–Ellison syndrome combined with type I multiple endocrine neoplasia. Pancreas 8(3):295

    CAS  PubMed  Google Scholar 

  109. Sakurai A, Katai M, Yamashita K, Mori JI, Fukushima Y, Hashizume K (2007) Long-term follow-up of patients with multiple endocrine neoplasia type 1. Endocr J 54(2):295–302

    PubMed  Google Scholar 

  110. Triponez F, Goudet P, Dosseh D et al (2006) Is surgery beneficial for MEN1 patients with small (≤2 cm), nonfunctioning pancreaticoduodenal endocrine tumor? An analysis of 65 patients from the GTE. World J Surg 30(5):654–662; discussion 63–64

    Google Scholar 

  111. Fendrich V, Langer P, Celik I et al (2006) An aggressive surgical approach leads to long-term survival in patients with pancreatic endocrine tumors. Ann Surg 244(6):845–851; discussion 52–53

    Google Scholar 

  112. Gauger PG, Doherty GM, Broome JT, Miller BS, Thompson NW (2009) Completion pancreatectomy and duodenectomy for recurrent MEN-1 pancreaticoduodenal endocrine neoplasms. Surgery 146(4):801–806; discussion 7–8

    Google Scholar 

  113. Berna MJ, Annibale B, Marignani M et al (2008) A prospective study of gastric carcinoids and enterochromaffin-like cell changes in multiple endocrine neoplasia type 1 and Zollinger–Ellison syndrome: identification of risk factors. J Clin Endocrinol Metab 93(5):1582–1591

    CAS  PubMed  Google Scholar 

  114. Bordi C, Falchetti A, Azzoni C et al (1997) Aggressive forms of gastric neuroendocrine tumors in multiple endocrine neoplasia type I. Am J Surg Pathol 21(9):1075–1082

    CAS  PubMed  Google Scholar 

  115. Gibril F, Schumann M, Pace A, Jensen RT (2004) Multiple endocrine neoplasia type 1 and Zollinger–Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore) 83(1):43–83

    Google Scholar 

  116. Lehy T, Cadiot G, Mignon M, Ruszniewski P, Bonfils S (1992) Influence of multiple endocrine neoplasia type 1 on gastric endocrine cells in patients with the Zollinger–Ellison syndrome. Gut 33(9):1275–1279

    CAS  PubMed  Google Scholar 

  117. Peghini PL, Annibale B, Azzoni C et al (2002) Effect of chronic hypergastrinemia on human enterochromaffin-like cells: insights from patients with sporadic gastrinomas. Gastroenterology 123(1):68–85

    PubMed  Google Scholar 

  118. Debelenko LV, Emmert-Buck MR, Zhuang Z et al (1997) The multiple endocrine neoplasia type I gene locus is involved in the pathogenesis of type II gastric carcinoids. Gastroenterology 113(3):773–781

    CAS  PubMed  Google Scholar 

  119. Gibril F, Venzon DJ, Ojeaburu JV, Bashir S, Jensen RT (2001) Prospective study of the natural history of gastrinoma in patients with MEN1: definition of an aggressive and a nonaggressive form. J Clin Endocrinol Metab 86(11):5282

    CAS  PubMed  Google Scholar 

  120. Rindi G, Azzoni C, La Rosa S et al (1999) ECL cell tumor and poorly differentiated endocrine carcinoma of the stomach: prognostic evaluation by pathological analysis. Gastroenterology 116(3):532–542

    CAS  PubMed  Google Scholar 

  121. Rindi G, Bordi C, Rappel S, La Rosa S, Stolte M, Solcia E (1996) Gastric carcinoids and neuroendocrine carcinomas: pathogenesis, pathology, and behavior. World J Surg 20(2):168–172

    CAS  PubMed  Google Scholar 

  122. Ruszniewski P, Delle Fave G, Cadiot G et al (2006) Well-differentiated gastric tumors/carcinomas. Neuroendocrinology 84(3):158–164

    CAS  PubMed  Google Scholar 

  123. Norton JA, Melcher ML, Gibril F, Jensen RT (2004) Gastric carcinoid tumors in multiple endocrine neoplasia-1 patients with Zollinger–Ellison syndrome can be symptomatic, demonstrate aggressive growth, and require surgical treatment. Surgery 136(6):1267–1274

    PubMed  Google Scholar 

  124. Gibril F, Reynolds JC, Lubensky IA et al (2000) Ability of somatostatin receptor scintigraphy to identify patients with gastric carcinoids: a prospective study. J Nucl Med 41(10):1646–1656

    CAS  PubMed  Google Scholar 

  125. Manfredi S, Pagenault M, de Lajarte-Thirouard AS, Bretagne JF (2007) Type 1 and 2 gastric carcinoid tumors: long-term follow-up of the efficacy of treatment with a slow-release somatostatin analogue. Eur J Gastroenterol Hepatol 19(11):1021–1025

    PubMed  Google Scholar 

  126. Tomassetti P, Migliori M, Caletti GC, Fusaroli P, Corinaldesi R, Gullo L (2000) Treatment of type II gastric carcinoid tumors with somatostatin analogues. N Engl J Med 343(8):551–554

    CAS  PubMed  Google Scholar 

  127. Ferolla P, Falchetti A, Filosso P et al (2005) Thymic neuroendocrine carcinoma (carcinoid) in multiple endocrine neoplasia type 1 syndrome: the Italian series. J Clin Endocrinol Metab 90(5):2603–2609

    CAS  PubMed  Google Scholar 

  128. Gibril F, Chen YJ, Schrump DS et al (2003) Prospective study of thymic carcinoids in patients with multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 88(3):1066–1081

    CAS  PubMed  Google Scholar 

  129. Goudet P, Murat A, Cardot-Bauters C et al (2009) 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 33(6):1197–1207

    PubMed  Google Scholar 

  130. Teh BT, McArdle J, Chan SP et al (1997) Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1. Medicine (Baltimore) 76(1):21–29

    CAS  Google Scholar 

  131. Lim LC, Tan MH, Eng C, Teh BT, Rajasoorya RC (2006) Thymic carcinoid in multiple endocrine neoplasia 1: genotype-phenotype correlation and prevention. J Intern Med 259(4):428–432

    CAS  PubMed  Google Scholar 

  132. Teh BT, Zedenius J, Kytola S et al (1998) Thymic carcinoids in multiple endocrine neoplasia type 1. Ann Surg 228(1):99–105

    CAS  PubMed  Google Scholar 

  133. Wilkinson S, Teh BT, Davey KR, McArdle JP, Young M, Shepherd JJ (1993) Cause of death in multiple endocrine neoplasia type 1. Arch Surg 128(6):683–690

    CAS  PubMed  Google Scholar 

  134. O’Toole D, Grossman A, Gross D et al (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: biochemical markers. Neuroendocrinology 90(2):194–202

    PubMed  Google Scholar 

  135. Clark OH, Benson AB III, Berlin JD et al (2009) NCCN clinical practice guidelines in oncology: neuroendocrine tumors. J Natl Compr Canc Netw 7(7):712–747

    CAS  PubMed  Google Scholar 

  136. Oberg K, Jelic S (2009) Neuroendocrine bronchial and thymic tumors: ESMO clinical recommendation for diagnosis, treatment and follow-up. Ann Oncol 20(suppl 4):147–149

    PubMed  Google Scholar 

  137. Burgess JR, Giles N, Shepherd JJ (2001) Malignant thymic carcinoid is not prevented by transcervical thymectomy in multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf) 55(5):689–693

    CAS  Google Scholar 

  138. Dotzenrath C, Goretzki PE, Cupisti K, Yang Q, Simon D, Roher HD (2001) Malignant endocrine tumors in patients with MEN 1 disease. Surgery 129(1):91–95

    CAS  PubMed  Google Scholar 

  139. Sachithanandan N, Harle RA, Burgess JR (2005) Bronchopulmonary carcinoid in multiple endocrine neoplasia type 1. Cancer 103(3):509–515

    PubMed  Google Scholar 

  140. Cadiot G, Vuagnat A, Doukhan I et al (1999) Prognostic factors in patients with Zollinger–Ellison syndrome and multiple endocrine neoplasia type 1. Groupe d’Etude des Neoplasies Endocriniennes Multiples (GENEM and groupe de Recherche et d’Etude du Syndrome de Zollinger–Ellison (GRESZE). Gastroenterology 116(2):286

    CAS  PubMed  Google Scholar 

  141. Tomassetti P, Campana D, Piscitelli L et al (2005) Endocrine pancreatic tumors: factors correlated with survival. Ann Oncol 16(11):1806–1810

    CAS  PubMed  Google Scholar 

  142. Eriksson B, Annibale B, Bajetta E et al (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: chemotherapy in patients with neuroendocrine tumors. Neuroendocrinology 90(2):214–219

    CAS  PubMed  Google Scholar 

  143. Kwekkeboom DJ, Krenning EP, Lebtahi R et al (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: peptide receptor radionuclide therapy with radiolabeled somatostatin analogs. Neuroendocrinology 90(2):220–226

    CAS  PubMed  Google Scholar 

  144. Oberg K, Ferone D, Kaltsas G, Knigge UP, Taal B, Plockinger U (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: biotherapy. Neuroendocrinology 90(2):209–213

    PubMed  Google Scholar 

  145. Steinmuller T, Kianmanesh R, Falconi M et al (2008) Consensus guidelines for the management of patients with liver metastases from digestive (neuro)endocrine tumors: foregut, midgut, hindgut, and unknown primary. Neuroendocrinology 87(1):47–62

    PubMed  Google Scholar 

  146. Cao CQ, Yan TD, Bester L, Liauw W, Morris DL (2010) Radioembolization with yttrium microspheres for neuroendocrine tumour liver metastases. Br J Surg 97(4):537–543

    CAS  PubMed  Google Scholar 

  147. Arnold R, Rinke A, Klose KJ et al (2005) Octreotide versus octreotide plus interferon-alpha in endocrine gastroenteropancreatic tumors: a randomized trial. Clin Gastroenterol Hepatol 3(8):761–771

    CAS  PubMed  Google Scholar 

  148. Faiss S, Pape UF, Bohmig M et al (2003) Prospective, randomized, multicenter trial on the antiproliferative effect of lanreotide, interferon alfa, and their combination for therapy of metastatic neuroendocrine gastroenteropancreatic tumors—the International Lanreotide and Interferon Alfa Study Group. J Clin Oncol 21(14):2689–2696

    Google Scholar 

  149. Rinke A, Muller HH, Schade-Brittinger C et al (2009) Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol 27(28):4656–4663

    CAS  PubMed  Google Scholar 

  150. Ballard HS, Fame B, Hartsock RJ (1964) Familial multiple endocrine adenoma-peptic ulcer complex. Medicine (Baltimore) 43:481–516

    CAS  Google Scholar 

  151. Burgess JR, Shepherd JJ, Parameswaran V, Hoffman L, Greenaway TM (1996) Somatotrophinomas in multiple endocrine neoplasia type 1: a review of clinical phenotype and insulin-like growth factor-1 levels in a large multiple endocrine neoplasia type 1 kindred. Am J Med 100(5):544–547

    CAS  PubMed  Google Scholar 

  152. Burgess JR, Shepherd JJ, Parameswaran V, Hoffman L, Greenaway TM (1996) Prolactinomas in a large kindred with multiple endocrine neoplasia type 1: clinical features and inheritance pattern. J Clin Endocrinol Metab 81(5):1841–1845

    CAS  PubMed  Google Scholar 

  153. O’Brien T, O’Riordan DS, Gharib H, Scheithauer BW, Ebersold MJ, van Heerden JA (1996) Results of treatment of pituitary disease in multiple endocrine neoplasia, type I. Neurosurgery 39(2):273–278; discussion 8–9

    Google Scholar 

  154. Stratakis CA, Schussheim DH, Freedman SM et al (2000) Pituitary macroadenoma in a 5-year-old: an early expression of multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 85(12):4776–4780

    CAS  PubMed  Google Scholar 

  155. Benito M, Asa SL, Livolsi VA, West VA, Snyder PJ (2005) Gonadotroph tumor associated with multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 90(1):570–574

    CAS  PubMed  Google Scholar 

  156. Taylor TJ, Donlon SS, Bale AE et al (2000) Treatment of a thyrotropinoma with octreotide-LAR in a patient with multiple endocrine neoplasia-1. Thyroid 10(11):1001–1007

    CAS  PubMed  Google Scholar 

  157. Trouillas J, Labat-Moleur F, Sturm N et al (2008) 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 32:534–543

    PubMed  Google Scholar 

  158. Gordon MV, Varma D, McLean CA, Bittar RG, Burgess JR, Topliss DJ (2007) Metastatic prolactinoma presenting as a cervical spinal cord tumour in multiple endocrine neoplasia type one (MEN-1). Clin Endocrinol (Oxf) 66(1):150–152

    Google Scholar 

  159. Barzon L, Pasquali C, Grigoletto C, Pedrazzoli S, Boscaro M, Fallo F (2001) Multiple endocrine neoplasia type 1 and adrenal lesions. J Urol 166(1):24–27

    CAS  PubMed  Google Scholar 

  160. Langer P, Cupisti K, Bartsch DK et al (2002) Adrenal involvement in multiple endocrine neoplasia type 1. World J Surg 26(8):891–896

    PubMed  Google Scholar 

  161. Schaefer S, Shipotko M, Meyer S et al (2008) Natural course of small adrenal lesions in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study. Eur J Endocrinol 158(5):699–704

    CAS  PubMed  Google Scholar 

  162. Skogseid B, Rastad J, Gobl A et al (1995) Adrenal lesion in multiple endocrine neoplasia type 1. Surgery 118(6):1077–1082

    CAS  PubMed  Google Scholar 

  163. Waldmann J, Bartsch DK, Kann PH, Fendrich V, Rothmund M, Langer P (2007) Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening. Langenbecks Arch Surg 392(4):437–443

    CAS  PubMed  Google Scholar 

  164. Beckers A, Abs R, Willems PJ et al (1992) Aldosterone-secreting adrenal adenoma as part of multiple endocrine neoplasia type 1 (MEN1): loss of heterozygosity for polymorphic chromosome 11 deoxyribonucleic acid markers, including the MEN1 locus. J Clin Endocrinol Metab 75(2):564–570

    CAS  PubMed  Google Scholar 

  165. Young WF Jr (2007) Clinical practice. The incidentally discovered adrenal mass. N Engl J Med 356(6):601–610

    CAS  PubMed  Google Scholar 

  166. Lenders JW, Eisenhofer G, Mannelli M, Pacak K (2005) Phaeochromocytoma. Lancet 366(9486):665–675

    PubMed  Google Scholar 

  167. Boukhman MP, Karam JH, Shaver J, Siperstein AE, Duh QY, Clark OH (1998) Insulinoma—experience from 1950 to 1995. West J Med 169(2):98–104

    CAS  PubMed  Google Scholar 

  168. Mathur A, Gorden P, Libutti SK (2009) Insulinoma. Surg Clin North Am 89(5):1105–1121

    PubMed  Google Scholar 

  169. Roy PK, Venzon DJ, Shojamanesh H et al (2000) Zollinger–Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore) 79(6):379–411

    CAS  Google Scholar 

  170. Nesi G, Marcucci T, Rubio CA, Brandi ML, Tonelli F (2008) Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol 23(4):521–526

    CAS  PubMed  Google Scholar 

  171. Meijer WG, Kema IP, Volmer M, Willemse PH, de Vries EG (2000) Discriminating capacity of indole markers in the diagnosis of carcinoid tumors. Clin Chem 46(10):1588–1596

    CAS  PubMed  Google Scholar 

  172. Goebel SU, Serrano J, Yu F, Gibril F, Venzon DJ, Jensen RT (1999) Prospective study of the value of serum chromogranin A or serum gastrin levels in the assessment of the presence, extent, or growth of gastrinomas. Cancer 85(7):1470–1483

    CAS  PubMed  Google Scholar 

  173. Granberg D, Stridsberg M, Seensalu R et al (1999) Plasma chromogranin A in patients with multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 84(8):2712–2717

    CAS  PubMed  Google Scholar 

  174. Nehar D, Lombard-Bohas C, Olivieri S et al (2004) Interest of Chromogranin A for diagnosis and follow-up of endocrine tumours. Clin Endocrinol (Oxf) 60(5):644–652

    CAS  Google Scholar 

  175. Peracchi M, Conte D, Gebbia C et al (2003) Plasma chromogranin A in patients with sporadic gastro-entero-pancreatic neuroendocrine tumors or multiple endocrine neoplasia type 1. Eur J Endocrinol 148(1):39–43

    CAS  PubMed  Google Scholar 

  176. Nobels FR, Kwekkeboom DJ, Coopmans W et al (1997) Chromogranin A as serum marker for neuroendocrine neoplasia: comparison with neuron-specific enolase and the alpha-subunit of glycoprotein hormones. J Clin Endocrinol Metab 82(8):2622–2628

    CAS  PubMed  Google Scholar 

  177. Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT (2006) Serum gastrin in Zollinger–Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine (Baltimore) 85(6):331–364

    Google Scholar 

  178. Berna MJ, Hoffmann KM, Serrano J, Gibril F, Jensen RT (2006) Serum gastrin in Zollinger–Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health, comparison with 2229 cases from the literature. Medicine (Baltimore) 85(6):295–330

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Internal Medicine, University Medical Centre Utrecht, Hpnr. L.00.408, PO Box 85500, 3508 GA, Utrecht, The Netherlands

    C. R. C. Pieterman, K. M. A. Dreijerink & G. D. Valk

  2. Department of Surgery, University Medical Centre Utrecht, Utrecht, The Netherlands

    M. R. Vriens

  3. Department of Medical Genetics, University Medical Centre Utrecht, Utrecht, The Netherlands

    R. B. van der Luijt

Authors
  1. C. R. C. Pieterman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. R. Vriens
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. K. M. A. Dreijerink
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R. B. van der Luijt
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. G. D. Valk
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to G. D. Valk.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Pieterman, C.R.C., Vriens, M.R., Dreijerink, K.M.A. et al. Care for patients with multiple endocrine neoplasia type 1: the current evidence base. Familial Cancer 10, 157–171 (2011). https://doi.org/10.1007/s10689-010-9398-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10689-010-9398-6

Keywords

Search

Navigation