Home LiteratureArticle Details
PMID: 14747767 Published · ppublish English Case Reports Comparative Study Journal Article Review

Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature.

Medicine ·Vol. 83 ·No. 1 ·2004-01-00 ·Pages 43-83

Gibril F, Schumann M, Pace A, Jensen RT

Abstract

In patients with multiple endocrine neoplasia type 1 (MEN1), the most common functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison syndrome (ZES). ZES has been well studied in its sporadic form (that is, without MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), and the long-term natural history is largely unknown. To address this issue we report the results of a prospective long-term National Institutes of Health (NIH) study of 107 MEN1/ZES patients and compare our results with those of 1009 MEN1/ZES patients in 278 case reports and small series in the literature. Patients were clinically, radiologically, and biochemically evaluated yearly for all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with patients from the literature, the NIH MEN1/ZES patients more frequently had pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas (5%). Twenty-five percent of both the NIH and the literature patients lacked a family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES was rarely (8%) the only initial manifestation of MEN1 if careful testing was done. ZES occurred before age 40 years in 50%-60% of the current patients, in contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its onset and is delayed as long as in sporadic ZES cases. Pituitary disease and carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES without a family history of MEN1, the MEN1 manifestations are not as severe. This study shows that MEN1/ZES patients differ in many aspects from those commonly reported in older studies involving few MEN1/ZES patients. In this study we have identified a number of important clinical and laboratory features of MEN1/ZES that were not previously appreciated, which should contribute to earlier diagnosis and improve both short- and long-term management.

MeSH Terms
Adult Age Distribution Age of Onset Aged Diagnosis, Differential Disease Progression Female Follow-Up Studies Gastrinoma/blood,diagnosis Genetic Testing Hormones/blood Humans Male Middle Aged Multiple Endocrine Neoplasia Type 1/diagnosis,epidemiology,therapy Prospective Studies Survival Analysis Tomography, X-Ray Computed Treatment Outcome Zollinger-Ellison Syndrome/blood,diagnosis,epidemiology
Chemicals
Hormones
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Gibril Fathia
From Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland.
Schumann Michael
Pace Andrea
Jensen Robert T
References (485)
485 references, click to expand
  1. Abboud P#, Bart H, Mansour G, Pinteaux A, Birembaut P. Ovarian gastrinoma in multiple endocrine neoplasia type I: A case report. Am J Obstet Gynecol. 2001; 184:237–8.
  2. Agarwal SK, Kester MB, Debelenko LV, Heppner C, Emmert-Buck MR, Skarulis MC, Doppman JL, Kim YS, Lubensky IA, Zhuang Z, Green JS, Guru SC, Manickam P, Olufemi SE, Liotta LA, Chandrasekharappa SC, Collins FS, Spiegel AM, Burns AL, Marx SJ. Germline mutations of the MEN1 gene in familial multiple endocrine neoplasia type 1 and related states. Hum Mol Genet. 1997; 6:1169–75.
  3. Akerstrom G, Hessman O, Skogseid B. Timing and extent of surgery in symptomatic and asymptomatic neuroendocrine tumors of the pancreas in MEN1. Langenbecks Arch Surg. 2002; 386:558–69.
  4. Alexander HR, Fraker DL, Norton JA, Barlett DL, Tio L, Benjamin SB, Doppman JL, Goebel SU, Serrano J, Gibril F, Jensen RT. Prospective study of somatostatin receptor scintigraphy and its effect on operative outcome in patients with Zollinger-Ellison syndrome. Ann Surg. 1998; 228:228–38.
  5. Andant C, Godeberge B, Chaussade S, Dupas JL, Soule JC. [Clinical course of symptomatic duodeno-jejunal Crohn’s disease]. Gastroenterol Clin Biol. 1999; 23:1134–8.
  6. Aoyagi T, Summerskill WH. Gastric secretion with ulcerogenic islet cell tumor. Importance of basal acid output. Arch Intern Med. 1966; 117:667–72.
  7. Arnold R, Benning R, Neuhaus C, Rolwage M, Trautmann ME. Gastroenteropancreatic endocrine tumors: effect of Sandostatin on tumor growth. The German Sandostatin Study Group. Metabolism. 1992; 41:116–8.
  8. Arnold R, Simon B, Wied M. Treatment of neuroendocrine GEP tumours with somatostatin analogues: a review. Digestion. 2000; 62:84–91.
  9. Arnold R, Trautmann ME, Creutzfeldt W, Benning R, Benning M, Neuhaus C, Jurgensen R, Stein K, Schafer H, Bruns C, Dennler HJ. Somatostatin analogue octreotide and inhibition of tumour growth in metastatic endocrine gastroenteropancreatic tumours. Gut. 1996; 38:430–8.
  10. Asa SL, Singer W, Kovacs K, Horvath E, Murray D, Colapinto N, Thorner MO. Pancreatic endocrine tumour producing growth hormone-releasing hormone associated with multiple endocrine neoplasia type I syndrome. Acta Endocrinol (Copenh). 1987; 115:331–7.
  11. Bader JP, Bonfils S, Ferrier JP, Dubrasquet M, Potet F, Martin E, Thiery JP, Lambling A. [The Zollinger-Ellison syndrome. Clinical, biological, and anatomical study of 10 cases]. Presse Med. 1967; 75:2709–13.
  12. Bahn RS, Scheithauer BW, vanHeerden JA, Laws ER Jr, Horvath E, Gharib H. Nonidentical expressions of multiple endocrine neoplasia, type I, in identical twins. Mayo Clin Proc. 1986; 61:689–96.
  13. Ballard HS, Frame B, Harstock RT. Familial endocrine adenoma-peptic ulcer disease. Medicine (Baltimore). 1964; 43:481–515.
  14. Bardram L, Stadil F. Omeprazole in the Zollinger-Ellison syndrome. Scand J Gastroenterol. 1986; 21:374–8.
  15. Bardram L, Stage JG. Frequency of endocrine disorders in patients with the Zollinger-Ellison syndrome. Scand J Gastroenterol. 1985; 20:233–8.
  16. Barreras RF, Donaldson RM Jr. Role of calcium in gastric hypersecretion, parathyroid adenoma and peptic ulcer. N Engl J Med. 1967; 276:1122–4.
  17. Bartsch D, Kopp I, Bergenfelz A, Rieder H, Munch K, Jager K, Deiss Y, Schudy A, Barth P, Arnold R, Rothmund M, Simon B. MEN1 gene mutations in 12 MEN1 families and their associated tumors. Eur J Endocrinol. 1998; 139:416–20.
  18. Bartsch DK, Langer P, Wild A, Schilling T, Celik I, Rothmund M, Nies C. Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: Surgery or surveillance? Surgery. 2000; 128:958–66.
  19. Barzon L, Pasquali C, Grigoletto C, Pedrazzoli S, Boscaro M, Fallo F. Multiple endocrine neoplasia type 1 and adrenal lesions. J Urol. 2001; 166:24–7.
  20. Bashir S, Gibril F, Ojeaburu J, Asgharian B, Entsuah L, Ferraro G, Crafa P, Bordi C, Jensen RT. Prospective study of the ability of histamine, serotonin or chromogranin A levels to identify gastric carcinoids in patients with gastrinomas. Aliment Pharmacol Ther. 2002; 16:1367–82.
  21. Bassett JH, Forbes SA, Pannett AA, Lloyd SE, Christie PT, Wooding C, Harding B, Besser GM, Edwards CR, Monson JP, Sampson J, Wass JA, Wheeler MH, Thakker RV. Characterization of mutations in patients with multiple endocrine neoplasia type 1. Am J Hum Genet. 1998; 62:232–44.
  22. Bastagli A, Nosenzo MA, Galimberti A, Vitri P, Rovati V. [Gastrinoma and MEN I]. Ann Ital Chir. 1992; 63:573–6.
  23. Baudin E, Bidart JM, Rougier P, Lazar V, Ruffie P, Ropers J, Ducreux M, Troalen F, Sabourin JC, Comoy E, Lasser P, DeBaere T, Schlumberger M. Screening for multiple endocrine neoplasia type 1 and hormonal production in apparently sporadic neuroendocrine tumors. J Clin Endocrinol Metab. 1999; 84:69–75.
  24. Bear JC, Briones-Urbina R, Fahey JF, Farid NR. Variant multiple endocrine neoplasia I (MEN 1 Burin): further studies and non-linkage to HLA. Hum Hered. 1985; 35:15–20.
  25. Beckers A, Abs R, Willems PJ, van der Auwera B, Kovacs K, Reznik M, Stevenaert A. 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. 1992; 75:564–70.
  26. Benson L, Ljunghall S, Akerstrom G, Oberg K. Hyperparathyroidism presenting as the first lesion in multiple endocrine neoplasia type I. Am J Med. 1987; 82:731–7.
  27. Benson L, Ljunghall S, Groth T, Falk H, Hvarfner A, Rastad J, Wide L, Akerstrom G. Optimal discrimination of mild hyperparathyroidism with total serum calcium, ionized calcium and parathyroid hormone measurements. Ups J Med Sci. 1987; 92:147–76.
  28. Benson L, Lundqvist G, Wide L, Akerstrom G, Oberg K, Ljunghall S. Effects of secretin on parathyroid hormone and calcium in normal subjects, patients with hyperparathyroidism and patients with gastrinoma. Acta Med Scand. 1985; 217:205–11.
  29. Benya RV, Metz DC, Venzon DJ, Fishbeyn VA, Strader DB, Orbuch M, Jensen RT. Zollinger-Ellison syndrome can be the initial endocrine manifestation in patients with multiple endocrine neoplasia type 1. Am J Med. 1994; 97:436–44.
  30. Berdjis CC. Pluriglandular syndrome. Oncologia. 1962; 15:288–311.
  31. Berger MW, Stephens DH. Gastric carcinoid tumors associated with chronic hypergastrinemia in a patient with Zollinger-Ellison syndrome. Radiology. 1996; 201:371–3.
  32. Bergman L, Teh B, Cardinal J, Palmer J, Walters M, Shepherd J, Cameron D, Hayward N. Identification of MEN1 gene mutations in families with MEN1 and related disorders. Br J Cancer. 2000; 83:1009–114.
  33. Betts JB, O’Malley BP, Rosenthal FD. Hyperparathyroidism: a prerequisite for Zollinger-Ellison syndrome in multiple endocrine adenomatosis type I—report of a further family and a review of the literature. Q J Med. 1980; 193:69–76.
  34. Beukes E, Dent DM, DeVilliers JC, Miller JL. Multiple endocrine neoplasia type I. S Afr Med J. 1985; 68:219–22.
  35. Blair EL, Grund ER, Miller IT, Redd JD, Sanders DJ, Thompson MH, Venables CW. Metiamide in the Zollinger-Ellison syndrome. Am J Dig Dis. 1975; 20:1123–30.
  36. Blomley MJ, Strickland NH, Jackson JE. Case report: multiple duodenal gastrinomas demonstrated with spiral CT. Clin Radiol. 1996; 51:811–3.
  37. Boey JH, Cooke TJC, Gilbert JM, Sweeney EC, Taylor S. Occurrence of other endocrine tumours in primary hyperparathyroidism. Lancet. 1975; 2:781–4.
  38. Bohm N, Nagele E, Grundner HG, Wagner E, Beneke G. XXXIV. Syndrom multipler endokriner hyperplasien and adenome (MEHA-syndrom) mit Zollinger-Ellison-syndrom. Med Welt. 1967; 44:2633–40.
  39. Bonfils S, Bernades P, Letonturier Ph. Diagnostic biologique du syndrome de Zollinger-Ellison dans le cadre d’une adenomatose poly-endorinienne. Nouv Presse Med. 1975; 4:2027–31.
  40. Bonfils S, Bernier JJ, Mignon M, Lambert R, Bernades P, L’Hirondel C, Accary J-P, Kloeti G. Metiamide treatment in five patients with Zollinger-Ellison syndrome. Digestion. 1977; 15:43–52.
  41. Bonfils S, Vallot T, Garcia del risco F, Mignon M. Traitement de l’hypersecretion gastrique acide du syndrome de Zollinger-Ellison par un derive des thioamides (40749 RP). Comparaison avec la ranitidine. Gastroenterol Clin Biol. 1986; 10:486–91.
  42. Bordi C. Endocrine tumours of the stomach. Pathol Res Pract. 1995; 191:373–80.
  43. Bordi C, Azzoni C, Ferraro G, Corleto VD, Gibril F, Delle Fave G, Lubensky IA, Venzon DJ, Jensen RT. Sampling strategies for analysis of enterochromaffin-like cell changes in Zollinger-Ellison syndrome. Am J Clin Pathol. 2000; 114:419–25.
  44. Bordi C, Cocconi G, Togni R, Vezzadini P, Missale G. Gastric endocrine cell proliferation. Association with Zollinger- Ellison syndrome. Arch Pathol. 1974; 98:274–8.
  45. Bordi C, Corleto V, Azzoni C, Pizzi S, Ferraro G, Gibril F, Delle Fave G, Jensen RT. The antral mucosa as a new site for endocrine tumors in multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome. J Clin Endocrinol Metab. 2001; 86:2236–42.
  46. Bordi C, D’Adda T, Azzoni C, Pilato FP, Caruana P. Hypergastrinemia and gastric enterochromaffin-like cells. Am J Surg Pathol. 1995; 19:S8–S19.
  47. Bordi C, DeVita O, Pilato FP, Carfagna G, D’Adda T, Missale G, Peracchia A. Multiple islet cell tumors with predominance of glucagon-producing cells and ulcer disease. Am J Clin Pathol. 1987; 88:153–61.
  48. Bordi C, Falchetti A, Azzoni C, D’Adda T, Canavese G, Guariglia A, Santini D, Tomassetti P, Brandi ML. Aggressive forms of gastric neuroendocrine tumors in multiple endocrine neoplasia type I. Am J Surg Pathol. 1997; 21:1075–82.
  49. Bordi C, Yu JY, Baggi MT, Davoli C, Pilato FP, Baruzzi G, Gardini G, Zamboni G, Franzin G, Papotti M, Bussolati G. Gastric carcinoids and their precursor lesions. A histologic and immunohistochemical study of 23 cases. Cancer. 1991; 67:663–72.
  50. Bozymski EM, Woodruff K, Sessions JT Jr. Zollinger-Ellison syndrome with hypoglycemia associated with calcification of the tumor and its metastases. Gastroenterology. 1973; 65:658–61.
  51. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJM, Lombardi G, Mannelli M, Pacini F, Ponder BAJ, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA Jr, Marx SJ. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001; 86:5658–71.
  52. Brandi ML, Marx SJ, Aurbach GD, Fitzpatrick LA. Familial multiple endocrine neoplasia type I: a new look at pathophysiology. Endocr Rev. 1987; 8:391–405.
  53. Brandi ML, Weber G, Svensson A, Falchetti A, Tonelli F, Castello R, Furlani L, Scappaticci S, Fraccaro M, Larsson C. Homozygotes for the autosomal dominant neoplasia syndrome (MEN1). Am J Hum Genet. 1993; 53:1167–72.
  54. Bricaire H, Courjaret J, Ferrier JP. Syndrome de Zollinger-Ellison associe’ a une tumeur de la loge hypophysaire. Interet pratique de la recherche du pouvoir secretagogue urinaire. Societe Medicale des Hopitaux de Paris. 1966; 117:829–39.
  55. Burgess JR, Giles N, Shepherd JJ. Malignant thymic carcinoid is not prevented by transcervical thymectomy in multiple endocrine neoplasia type 1. Clin Endocrinol (Oxf). 2001; 55:689–93.
  56. Burgess JR, Greenaway TM, Parameswaran V, Challis DR, David R, Shepherd JJ. Enteropancreatic malignancy associated with multiple endocrine neoplasia type 1. Cancer. 1998; 83:428–34.
  57. Burgess JR, Greenaway TM, Parameswaran V, Shepherd JJ. Octreotide improves biochemical, radiologic, and symptomatic indices of gastroenteropancreatic neoplasia in patients with multiple endocrine neoplasia type 1 (MEN-1). Implications for an integrated model of MEN-1 tumorigenesis. Cancer. 1999; 86:2154–9.
  58. Burgess JR, Greenaway TM, Shepherd JJ. Expression of the MEN-1 gene in a large kindred with multiple endocrine neoplasia type 1. J Intern Med. 1998; 243:465–70.
  59. Burgess JR, Harle RA, Tucker P, Parameswaran V, Davies P, Greenaway TM, Shepherd JJ. Adrenal lesions in a large kindred with multiple endocrine neoplasia type 1. Arch Surg. 1996; 131:699–702.
  60. Burgess JR, Shepherd JJ, Murton FJ, Parameswaran V, Greenaway TM. Effective control of bone pain by octreotide in a patient with metastatic gastrinoma. Med J Aust. 1996; 164:725–7.
  61. Burgess JR, Shepherd JJ, Parameswaran V, Hoffman L, Greenaway TM. Prolactinomas in a large kindred with multiple endocrine neoplasia type 1: clinical features and inheritance pattern. J Clin Endocrinol Metab. 1996; 81:1841–5.
  62. Burgess JR, Shepherd JJ, Parameswaran V, Hoffman L, Greenaway TM. Spectrum of pituitary disease in multiple endocrine neoplasia type 1 (MEN1): clinical, biochemical, and radiological features of pituitary disease in a large MEN1 kindred. J Clin Endocrinol Metab. 1996; 81:2642–6.
  63. Burke G, Finer JR, Sandlow LJ. Ulcerogenic tumor of the pancreas and hyperparathyroidism. Gastroenterology. 1964; 47:88–96.
  64. Cadiot G, Houillier P, Allouch A, Paillard M, Mignon M. Oral calcium tolerance test in the early diagnosis of primary hyperparathyroidism and multiple endocrine neoplasia type 1 in patients with the Zollinger-Ellison syndrome. Groupe de Recherche et d’Etude du Syndrome de Zollinger-Ellison. Gut. 1996; 39:273–8.
  65. Cadiot G, Laurent-Puig P, Thuille B, Lehy T, Mignon M, Olschwang S. Is the multiple endocrine neoplasia type 1 gene a suppressor for fundic argyrophil tumors in the Zollinger-Ellison syndrome? Gastroenterology. 1993; 105:579–82.
  66. Cadiot G, Vuagnat A, Doukhan I, Murat A, Bonnaud G, Delemer B, Thiefin G, Beckers A, Veyrac M, Proye C, Ruszniewski P, Mignon M. Prognostic factors in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1. Gastroenterology. 1999; 116:286–93.
  67. Cadranel JF, Ruszniewski P, Elouaer-Blanc L, Lehy T, Delchier JC, Cadiot G, Florent C, Vatier J, Mignon M. [Long term efficacy and tolerability of omeprazole in 20 patients with severe Zollinger-Ellison syndrome]. Gastroenterol Clin Biol. 1989; 13:654–62.
  68. Calendar A. Genetic testing in multiple endocrine neoplasia and related syndromes. FORUM Trends Exp Clin Med. 1998; 8:146–59.
  69. Cameron AJ, Hoffman HN. Zollinger-Ellison syndrome; Clinical features and long-term follow-up. Mayo Clin Proc. 1974; 49:44–51.
  70. Carlson HE, Levine GA, Goldberg NJ, Hershman JM. Hyperprolactinemia in multiple endocrine adenomatosis, type 1. Arch Intern Med. 1978; 138:1807–8.
  71. Carty SE, Helm AK, Amico JA, Clarke MR, Foley TP, Watson CG, Mulvihill JJ. The variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1. Surgery. 1998; 124:1106–14.
  72. Case records of the Massachusetts General Hospital (Case 43242). N Engl J Med. 1957; 256:1153–7.
  73. Case Records of the Massachusetts General Hospital: Weekly clinicopathological exercises. Case 47-1974. N Engl J Med. 1974; 291:1179–86.
  74. Case records of the Massachusetts General Hospital: Case 1-1976. N Engl J Med. 1976; 294:37–42.
  75. Cassar J, Polak JM, Cooke WM. Possible parathyroid origin of gastrin in a patient with multiple endocrine adenopathy type 1. Br J Surg. 1975; 62:313–6.
  76. Caughey JE, Parendeh G, Cohen LB, Vameghi M. Multiple endocrine adenomatosis with metabolic myopathy. N Z Med J. 1971; 74:28–31.
  77. Cavaco BM, Domingues R, Bacelar MC, Cardoso H, Barros L, Gomes L, Ruas MMA, Agapito A, Garrao A, Pannett AAJ, Silva JL, Sobrinho LG, Thakker RV, Leite V. Mutational analysis of Portuguese families with multiple endocrine neoplasia type 1 reveals large germline deletions. Clin Endocrinol. 2002; 56:465–73.
  78. Cebrian A, Herrera-Pombo JL, Diez JJ, Sanchez-Vilar O, Lara JI, Vazquez C, Pico A, Osorio A, Martinez-Delgado B, Benitez J, Robledo M. Genetic and clinical analysis in 10 Spanish patients with multiple endocrine neoplasia type 1. Eur J Hum Genet. 1999; 7:585–9.
  79. Champault G, Chapuis Y, Patel JC, Louvel A, Ghozlan R, Cachin M, Leger L. [Zollinger-Ellison syndrome and parathyroid adenoma. Limits of Wermer’s syndrome]. J Chir (Paris). 1976; 112:131–44.
  80. Chandrasekharappa SC, Guru SC, Manickam P, Olufemi SE, Collins FS, Emmert-Buck MR, Debelenko LV, Zhuang Z, Lubensky IA, Liotta LA, Crabtree JS, Wang Y, Roe BA, Weisemann J, Boguski MS, Agarwal SK, Kester MB, Kim YS, Heppner C, Dong Q, Spiegel AM, Burns AL, Marx SJ. Positional cloning of the gene for multiple endocrine neoplasia Type 1. Science. 1997; 276:404–7.
  81. Chanson P, Cadiot G, Murat A. Management of patients and subjects at risk for multiple endocrine neoplasia type 1: MEN1. Horm Res. 1997; 47:211–20.
  82. Cherner JA, Doppman JL, Norton JA, Miller DL, Krudy AG, Raufman JP, Collen MJ, Maton PN, Gardner JD, Jensen RT. Selective venous sampling for gastrin to localize gastrinomas. A prospective study. Ann Intern Med. 1986; 105:841–7.
  83. Cherner JA, Sawyers JL. Benefit of resection of metastatic gastrinoma in multiple endocrine neoplasia type I. Gastroenterology. 1992; 102:1049–53.
  84. Chiang HC, O’Dorisio TM, Huang SC, Maton PN, Gardner JD, Jensen RT. Multiple hormone elevations in patients with Zollinger-Ellison syndrome: Prospective study of clinical significance and of the development of a second symptomatic pancreatic endocrine tumor syndrome. Gastroenterology. 1990; 99:1565–75.
  85. Chico A, Gallart L, Martin-Campos JM, Catasus L, Mayoral C, Mato E, Tortosa F, Berna L, Rodriquez-Espinosa J, Blanco-Vaca F, Matias-Guiu X, deLeiva A, Mauricio D. Genetic, clinical, and biochemical analysis of unrelated Spanish families with multiple endocrine neoplasia type I. Endocr Pract. 2000; 6:13–9.
  86. Child DF, Laing I, Ganguli PC. Failure of oral bromocriptine to affect hypergastrinemia in two patients with the Zollinger-Ellison syndrome. J Endocrinol Invest. 1978; 1:351–3.
  87. Chöng JL, Christofides ND, Kraenzlin ME, Keshavarzian A, Burrin JM, Woolf IL, Hodgson HJ, Bloom SR. Growth hormone secretion dynamics in a patient with ectopic growth hormone-releasing factor production. Am J Med. 1985; 79:135–8.
  88. Christlieb AR, Schuster MM. Zollinger-Ellison syndrome. Arch Intern Med. 1964; 114:381–8.
  89. Christoforidis AJ, Nelson SW. Radiologic manifestations of ulcerogenic tumors of the pancreas. The Zollinger-Ellison syndrome. JAMA. 1966; 198:511–6.
  90. Chvojka VE. Islet-cell tumors and peptic ulcers: case report of the Zollinger-Ellison syndrome. Ann Intern Med. 1960; 53:1180–95.
  91. Clark CG, Chowcat NL, Lewin MR, Gilbert JM, Gelister JS, Boulos PB. Surgery for peptic ulceration associated with hypergastrinaemia. Br J Surg. 1986; 73:248–52.
  92. Collins JSA, Buchanan KD, Kennedy TL, Johnston CF, Ardill JES, Sloan JM, McIlrath EM, Russell C. Changing patterns in presentation and management of the Zollinger-Ellison syndrome in Northern Ireland, 1970-1988. Q J Med. 1991; 78:215–25.
  93. Condon RE, Granville GE, Jordan PH Jr, Helgason AH. Hypercalcemic crisis and intractable gastrointestinal ulceration in a patient with endocrine polyglandular syndrome. Ann Surg. 1968; 167:185–90.
  94. Conte N, LoGiudice C, Valmachino VG, Manente P, Fioretti D, Gasparoni P, Pedrazzoli S. Zollinger-Ellison syndrome associated with primary hyperparathyroidism and pituitary prolactin-secreting adenoma (MEN1). Ital J Gastroenterol. 1981; 13:196–8.
  95. Cook HB, French AB. Physiologic responses to gastric acid hypersecretion in Zollinger-Ellison syndrome. Am J Dig Dis. 1968; 13:191–203.
  96. Cook HB, Lennard-Jones JE. Effect of antisecretory drugs on gastric hypersecretion in endocrine-adenoma syndromes. Lancet. 1966; 2:247–50.
  97. Cope O, Keynes WM, Roth SI, Castleman B. Primary chief-cell hyperplasia of the parathyroid glands: a new entity in the surgery of hyperparathyroidism. Ann Surg. 1958; 148:375–88.
  98. Corbetta S, Pizzocaro A, Peracchi M, Beck-Peccoz P, Faglia G, Spada A. Multiple endocrine neoplasia type 1 in patients with recognized pituitary tumours of different types. Clin Endocrinol. 1997; 47:507–12.
  99. Corenblum B, Sirek AM, Horvath E, Kovacs K, Ezrin C. Human mixed somatotrophic and lactotrophic pituitary adenomas. J Clin Endocrinol Metab. 1976; 42:857–63.
  100. Corleto V, Annibale B, D’Ambra G, Saggioro A, Ferrua B, Cassetta MR, Della Fave G. Efficacy of long-term therapy with low doses of omeprazole in the control of gastric acid secretion in Zollinger-Ellison syndrome patients. Aliment Pharmacol Ther. 1993; 7:167–73.
  101. Cortes M, Mora J, Benitez A, J Gonzalez M, Castell M, Martin-Comin J. [Bone metastasis from gastrinoma without hepatic involvement. Utility of the scintigraphy with 111 In-pentetreotide]. Rev Esp Med Nucl. 2001; 20:23–6.
  102. Cowley DJ, Dymock IW, Boyes BE, Wilson RY, Stagg BH, Lewin MR, Polak JM, Pearse AGE. Zollinger-Ellison syndrome type 1: clinical and pathological correlations in a case. Gut. 1973; 14:25–9.
  103. Crane SA, Summers RW, Heeringa WG. Long-term cimetidine and anticholinergic therapy in patients with gastrinoma. Am J Surg. 1979; 138:446–50.
  104. Craven DE, Goodman AD, Carter JH. Familial multiple endocrine adenomatosis. Arch Intern Med. 1972; 129:567–9.
  105. Creutzfeldt W. The achlorhydria-carcinoid sequence: role of gastrin. Digestion. 1988; 39:61–79.
  106. Croisier JC, Azerad E, Lubetzki J. Adenomatose polyendocrinenne (syndrome de Wermer): a propos d’une observation personelle. Revue de la Litterature Paris. Sem Hop. 1971; 47:494–525.
  107. Croisier JC, Croisier G, Schaison G. Les proliferations insulaires pancreatiques associees. Actual Endocrinol (Paris). 1973; 13:15–27.
  108. Croisier JC, Lehy T, Zeitoun P. A2 cell pancreatic microadenomas in a case of multiple endocrine adenomatosis. Cancer. 1971; 28:707–13.
  109. Cunningham L, Hawe P, Evans RW. Islet-celled tumour of the pancreas with unusual clinico-pathological features. Br J Surg. 1952; 39:319–24.
  110. Dackiw AP, Cote GJ, Fleming JB, Schultz PN, Stanford P, Vassilopoulou-Sellin R, Evans DB, Gagel RF, Lee JE. Screening for MEN1 mutations in patients with atypical endocrine neoplasia. Surgery. 1999; 126:1097–104.
  111. Darling TN, Skarulis MC, Steinberg SM, Marx SJ, Spiegel AM, Turner M. Multiple facial angiofibromas and collagenomas in patients with multiple endocrine neoplasia type 1. Arch Dermatol. 1997; 133:853–7.
  112. Dean PG, vanHeerden JA, Farley DR, Thompson GB, Grant CS, Harmsen WS, Ilstrup DM. Are patients with multiple endocrine neoplasia type I prone to premature death? World J Surg. 2000; 24:1437–41.
  113. DeAngelis C, Carucci P, Repici A, Rizzetto M. Endosonography in decision making and management of gastrointestinal endocrine tumors. Eur J Ultrasound. 1999; 10:139–50.
  114. Debelenko LV, Emmert-Buck MR, Zhuang Z, Epshteyn E, Moskaluk CA, Jensen RT, Liotta LA, Lubensky IA. The multiple endocrine neoplasia type 1 gene locus is involved in the pathogenesis of Type II gastric carcinoids. Gastroenterology. 1997; 113:773–82.
  115. Debelenko LV, Zhuang ZP, Emmert-Buck MR, Chandrasekharappa S, Manickam P, Guru SC, Marx SJ, Skarulis M, Spiegel AM, Collins FS, Jensen RT, Liotta LA, Lubensky IA. Allelic deletions on chromosome 11q13 in Multiple Endocrine Neoplasia Type-I-associated sporadic gastrinomas and pancreatic endocrine tumors. Cancer Res. 1997; 57:2238–43.
  116. Delaney JP, Jacobson ME. Multiple endocrine adenomatosis. Minn Med. 1968; 51:1731–4.
  117. Delchier JC, Soule JC, Mignon M, Goldfain D, Cortot A, Travers B, Isal JP, Bader JP. Effectiveness of omeprazole in seven patients with Zollinger- Ellison syndrome resistant to histamine H2-receptor antagonists. Dig Dis Sci. 1986; 31:693–9.
  118. Delle Fave G, Marignani M, Moretti A, D’Ambra G, Martino G, Annibale B. Hypergastrinemia and enterochromaffin-like cell hyperplasia. Yale J Biol Med. 1998; 71:291–301.
  119. Demeure MJ, Klonoff DC, Karam JH, Duh QY, Clark OH. Insulinomas associated with multiple endocrine neoplasia type I: The need for a different surgical approach. Surgery. 1991; 110:998–1005.
  120. DeMuth WE. Zollinger-Ellison syndrome. Arch Surg. 1964; 88:244–8.
  121. DeNeef JC, Krull GH, Vonk J, Vos AK, Bool J. Zollinger-Ellison syndrome in a patient with secondary amenorrhea; report of a case with fatal outcome. Fertil Steril. 1970; 21:43–63.
  122. Deveney CW, Deveney KS, Jaffe BM, Jones RS, Way LW. Use of calcium and secretin in the diagnosis of gastrinoma (Zollinger-Ellison syndrome). Ann Intern Med. 1977; 87:680–6.
  123. Dionisi S, Minisola S, Pepe J, DeGeronimo S, Paglia F, Memeo L, Fitzpatrick LA. Concurrent parathyroid adenomas and carcinoma in the setting of multiple endocrine neoplasia type 1: presentation as hypercalcemic crisis. Mayo Clin Proc. 2002; 77:866–9.
  124. Doherty GM, Olson JA, Frisella MM, Lairmore TC, Wells SA Jr, Norton JA. Lethality of multiple endocrine neoplasia Type I. World J Surg. 1998; 22:581–7.
  125. Donaldson RM Jr, Von Eigen DR, Dwight RW. Gastric hypersecretion, peptic ulceration and islet-cell tumor of the pancreas (The Zollinger-Ellison syndrome). N Engl J Med. 1957; 257:965–70.
  126. Dong Q, Debelenko LV, Chandrasekharappa S, Lotterman C, Skarulis M, Emmert-Buck MR, Zhuang Z, Guru SC, Manickam P, Lubensky IA, Liotta LA, Collins FS, Marx SJ, Spiegel AM. Loss of heterozygosity at 11q13: analysis of pituitary tumors, lung carcinoids, lipomas, and other uncommon tumors in subjects with familial multiple endocrine neoplasia type 1. J Clin Endocrinol Metab. 1997; 82:1416–20.
  127. Donow C, Pipeleers-Marichal M, Schroder S, Stamm B, Heitz PU, Kloppel G. Surgical pathology of gastrinoma: site, size, multicentricity, association with multiple endocrine neoplasia type 1, and malignancy. Cancer. 1991; 68:1329–34.
  128. Dotzenrath C, Goretzki PE, Cupisti K, Yang Q, Simon D, Roher HD. Malignant endocrine tumors in patients with MEN1 disease. Surgery. 2001; 129:91–5.
  129. Duggan M, Anderson C. Mixed type Zollinger-Ellison syndrome in a florid case of multiple endocrine neoplasia type I. Am J Clin Pathol. 1984; 82:481–6.
  130. Duh QY, Hybarger CP, Geist R, Gamsu G, Goodman PC, Gooding GA, Clark OH. Carcinoids associated with multiple endocrine neoplasia syndromes. Am J Surg. 1987; 154:142–8.
  131. Dutta P, Wallace MR, Wrong OM, Taylor S, Welbourn RB. Familial multiple endocrine adenopathy (primary hyperparathyroidism and Zollinger-Ellison syndrome) in two siblings. Proc R Soc Med. 1968; 61:658–60.
  132. Eberle F, Grun R. Multiple endocrine neoplasia, type I (MEN I). Ergeb Inn Med Kinderheilkd. 1981; 46:76–149.
  133. Eberle F, Martini GA, Scheuer A, Rohr G, Sohl R, Bischof W, Wahl R. Thymus carcinoid tumor and multiple endocrine neoplasia type I (MEN1). Internist (Berl). 1982; 23:718–24.
  134. Eiseman B, Maynard RM. A non-insulin producing islet cell adenoma associated with progressive peptic ulceration (the Zollinger-Ellison syndrome). Surgery. 1956; 31:296–304.
  135. Eissele R, Anlauf M, Schafer MK, Eiden LE, Arnold R, Weihe E. Expression of vesicular monoamine transporters in endocrine hyperplasia and endocrine tumors of the oxyntic stomach. Digestion. 1999; 60:428–39.
  136. Ellison EC, Carey LC, Sparks J, O’Dorisio TM, Mekhjian HS, Fromkes JJ, Caldwell JH, Thomas FB. Early surgical treatment of gastrinoma. Am J Med. 1987; 82:17–24.
  137. Ellison EH, Wilson SD. The Zollinger-Ellison syndrome: Re-appraisal and evaluation of 260 registered cases. Ann Surg. 1964; 160:512–30.
  138. Farhangi M, Taylor J, Havey A, O’Dorisio TM. Neuroendocrine (carcinoid) tumor of the lung and type I multiple endocrine neoplasia. South Med J. 1987; 80:1459–62.
  139. Farid NR, Buchler S, Russell NA, Maroun FB, Allerdice P, Smyth HS. Prolactinomas in familial multiple endocrine neoplasia syndrome type I. Relationship to HLA and carcinoid tumors. Am J Med. 1980; 69:874–80.
  140. Farley DR, Van Heerden JA, Grant CS, Miller LJ, Ilstrup DM. The Zollinger-Ellison syndrome. A collective surgical experience. Ann Surg. 1992; 215:561–9.
  141. Ferriss JB, Brown JJ, Cumming AM, Fraser R, Lever AF, Peacock M, Robertson JI. Primary hyperparathyroidism associated with primary hyperaldosteronism. Acta Endocrinol (Copenh). 1983; 103:365–70.
  142. Feurle GE, Helmstaeder V, Hoevels J, Wenzel-Herzer G, Klempa J. [Changes in the diagnosis and treatment of Zollinger-Ellison syndrome]. Dtsch Med Wohenschr. 1982; 107:697–704.
  143. Fiasse R, DeGraef J, Pauwels S, Woussen-Colle MC, Rahier J, Kestens PJ, Dive C. [Hypergastrinemia. Physiopathological and clinical aspects with a study of a series of Zollinger-Ellison cases]. Acta Gastroenterol Belg. 1982; 45:293–309.
  144. Fiasse R, Pauwels S, Rahier J, Jamar F, Ketelslegers JM, Hassoun A, deLongueville M. [Use of octreotide in the treatment of digestive neuroendocrine tumours. Seven year experience in 20 cases including 9 cases of metastatic midgut carcinoid and 5 cases of metastatic gastrinoma]. Acta Gastroenterol Belg. 1993; 56:279–91.
  145. Fishbeyn VA, Norton JA, Benya RV, Pisegna JR, Venzon DJ, Metz DC, Jensen RT. Assessment and prediction of long-term cure in patients with Zollinger-Ellison syndrome: the best approach. Ann Intern Med. 1993; 119:199–206.
  146. Fisher ER, Flandreau RH. Multiple endocrine tumors and peptic ulcer. Gastroenterology. 1957; 32:1075–94.
  147. Fisher ER, Hicks J. Further pathologic observations on the syndrome of peptic ulcer and multiple endocrine tumors. Gastroenterology. 1960; 38:458–66.
  148. Fontana D, Aloesio R, Della Beffa V, Rollino R, Bertero D, Bronda M, Oliaro A, Gaetini A. [A case of multiple endocrine adenomatosis with atypical mesenteric localization of 2 gastrinomas]. Minerva Chir. 1984; 39:1165–70.
  149. Fox PS, Hofmann JW, Wilson SD, Decosse JJ. Surgical management of the Zollinger-Ellison syndrome. Surg Clin North Am. 1974; 54:395–407.
  150. Friesen SR. The development of endocrinopathies in the prospective screening of two families with multiple endocrine adenopathy, type I. World J Surg. 1979; 3:753–64.
  151. Friesen SR, Bolinger RE, Pearse AG, McGuigan JE. Serum gastrin levels in malignant Zollinger-Ellison syndrome after total gastrectomy and hypophysectomy. Ann Surg. 1970; 172:504–21.
  152. Friesen SR, Hermreck AS, Brackett CE, Schimke RN. Cimetidine in the management of synchronous crises of MEA I. World J Surg. 1980; 4:123–9.
  153. Friesen SR, McGuigan JE. Ectopic apudocarcinomas and associated endocrine hyperplasias of the foregut. Ann Surg. 1975; 182:371–85.
  154. Friesen SR, Schimke RN, Pearse AG. Genetic aspects of the Z-E syndrome: prospective studies in two kindreds: antral gastrin cell hyperplasia. Ann Surg. 1972; 176:370–83.
  155. Frilling A, Becker H, Roeher HD. Unusual features of multiple endocrine neoplasia. Henry Ford Hosp Med J. 1992; 40:253–5.
  156. Frucht H, Howard JM, Slaff JI, Wank SA, McCarthy DM, Maton PN, Vinayek R, Gardner JD, Jensen RT. Secretin and calcium provocative tests in the Zollinger-Ellison syndrome: A prospective study. Ann Intern Med. 1989; 111:713–22.
  157. Frucht H, Maton PN, Jensen RT. Use of omeprazole in patients with the Zollinger-Ellison syndrome. Dig Dis Sci. 1991; 36:394–404.
  158. Frucht H, Norton JA, London JF, Vinayek R, Doppman JL, Gardner JD, Jensen RT, Maton PN. Detection of duodenal gastrinomas by operative endoscopic transillumination: a prospective study. Gastroenterology. 1990; 99:1622–7.
  159. Gerstel G. Uber multiple tumoren der drusen mit innerer sekretion bei einem akromegalen. Frankf Z Pathol. 1938; 52:485–99.
  160. Gibril F, Chen Y-J, Schrump DS, Vortmeyer A, Zhuang ZP, Lubensky IA, Reynolds JG, Louie JV, Entsuah L, Huang K, Asgharian B, Jensen RT. Prospective study of thymic carcinoids in patients with multiple endocrine neoplasia type 1. J Clin Endocrinol Metab. 2003; 88:1066–81.
  161. Gibril F, Doppman JL, Chang R, Weber HC, Termanini B, Jensen RT. Metastatic gastrinomas: localization with selective arterial injection of secretin. Radiology. 1996; 198:77–84.
  162. Gibril F, Doppman JL, Reynolds JC, Chen CC, Sutliff VE, Yu F, Serrano J, Venzon DJ, Jensen RT. Bone metastases in patients with gastrinomas: a prospective study of bone scanning, somatostatin receptor scanning, and MRI in their detection, their frequency, location and effect of their detection on management. J Clin Oncol. 1998; 16:1040–53.
  163. Gibril F, Reynolds JC, Doppman JL, Chen CC, Venzon DJ, Termanini B, Weber HC, Stewart CA, Jensen RT. Somatostatin receptor scintigraphy: its sensitivity compared with that of other imaging methods in detecting primary and metastatic gastrinomas: a prospective study. Ann Intern Med. 1996; 125:26–34.
  164. Gibril F, Reynolds JC, Roy PK, Peghini PL, Doppman JL, Jensen RT. Ability of somatostatin receptor scintigraphy to identify patients with localized gastric carcinoids: A prospective study. J Nucl Med. 2000; 41:1646–56.
  165. Gibril F, Venzon DJ, Ojeaburu JV, Bashir S, Jensen RT. Prospective study of the natural history of gastrinoma in patients with MEN1: Definition of an aggressive and a nonaggressive form. J Clin Endocrinol Metab. 2001; 86:5282–93.
  166. Giraud S, Zhang CX, Serova-Sinilnikova O, Wautot V, Salandre J, Buisson N, Waterlot C, Bauters C, Porchet N, Aubert JP, Emy P, Cadiot G, Delemer B, Chabre O, Niccoli P, Leprat F, Duron F, Emperauger B, Cougard P, Goudet P, Sarfati E, Riou JP, Guichard S, Rodier M, Meyrier A, et al. Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders. Am J Hum Genet. 1998; 63:455–67.
  167. Glickman MH, Hart MJ, White TT. Insulinoma in Seattle: 39 cases in 30 years. Am J Surg. 1980; 140:119–25.
  168. Glowniak JV, Shapiro B, Vinik AI, Glaser B, Thompson NW, Cho KJ. Percutaneous transhepatic venous sampling of gastrin: value in sporadic and familial islet-cell tumors and G-cell hyperfunction. N Engl J Med. 1982; 307:293–7.
  169. Gogel KH, Buckman MT, Cadieux T, McCarthy DM. Gastric secretion and hormonal interactions in multiple endocrine neoplasia type 1. Arch Intern Med. 1985; 145:855–9.
  170. Grama D, Eriksson B, Martensson H, Cedermark B, Ahrön B, Kristoffersson A, Rastad J, Oberg K, Akerström G. Clinical characteristics, treatment and survival in patients with pancreatic tumors causing hormonal syndromes. World J Surg. 1992; 16:632–9.
  171. Grieco A, Bianco A, Alfei B, Rufini V, Vecchio FM, Marcoccia S, Gasbarrini G. Liver metastases of endocrine tumour associated with multiple endocrine neoplasia type 1: a sustained response to interferon therapy or a peculiar benign course? Hepatogastroenterology. 2000; 47:1269–72.
  172. Guida PM, Todd JE, Moore SW, Beal JM. Zollinger-Ellison syndrome with interesting variations. Am J Surg. 1966; 112:807–17.
  173. Hakanson R, Bottcher G, Sundler F, Vallgren S. Activation and hyperplasia of gastrin and enterochromaffin-like cells in the stomach. Digestion. 1986; 1:23–41.
  174. Hansky J, Soveny C, Korman MG. Value of serum gastrin in the diagnosis of the Zollinger-Ellison syndrome. Aust N Z J Med. 1973; 3:349–54.
  175. Herd GW. A case of primary hyperparathyroidism, primary hyperaldosteronism and Cushing’s disease. Acta Endocrinol (Copenh). 1984; 107:371–4.
  176. Hermans MMH, Lips CJM, Bravenboer B. Growth hormone overproduction in a patient with multiple endocrine neoplasia type I. J Intern Med. 2000; 248:525–30.
  177. Hershon KS, Kelly WA, Shaw CM, Schwartz R, Bierman EL. Prolactinomas as part of the multiple endocrine neoplastic syndrome type 1. Am J Med. 1983; 74:713–20.
  178. Hessman O, Skogseid B, Westin G, Akerstrom G. Multiple allelic deletions and intratumoral genetic heterogeneity in MEN1 pancreatic tumors. J Clin Endocrinol Metab. 2001; 86:1355–61.
  179. Heynen GO, Luyckx A, Franchimont P, van Cauwenberge H. La Regulation des secretions endocriniennes dans un cas de polyadenomatose (’). Act Endocrinol (Paris). 1973; 13:275–87.
  180. Hill DM, Anderson J, Marks V. Pluriglandular syndrome: multiple endocrine neoplasia. Proc R Soc Med. 1967; 60:650–1.
  181. Hirai S, Hamanaka Y, Mitsui N, Kumagai H, Kobayashi T. Thymic carcinoids in multiple endocrine neoplasia type 1. Jpn J Thorac Cardiovasc Surg. 2001; 49:525–7.
  182. Hirschowitz BI, Mohnen J, Shaw S. Long-term treatment with lansoprazole for patients with Zollinger-Ellison syndrome. Aliment Pharmacol Ther. 1996; 10:507–22.
  183. Ho AC, Horton KM, Fishman EK. Gastric carcinoid tumors as a consequence of chronic hypergastrinemia: spiral CT findings. Clin Imaging. 2000; 24:200–3.
  184. Hofmann M, Schilling T, Heilmann P, Haisken O, Wuster C, Brandi ML, Ziegler R, Nawroth PP. [Multiple endocrine neoplasia associated with multiple lipomas]. Med Klin. 1998; 93:546–9.
  185. Howard TJ, Zinner MJ, Stabile BE, Passaro E Jr. Gastrinoma excision for cure. A prospective analysis. Ann Surg. 1990; 211:9–14.
  186. Huizenga KA, Goodirck WIM, Summerskill WHJ. Peptic ulcer with islet cell tumor: a reappraisal. Am J Med. 1964; 37:564–77.
  187. Imamura M, Takahashi K, Adachi H, Minematsu S, Shimada Y, Naito M, Suzuki T, Tobe T, Azuma T. Usefulness of selective arterial secretin injection test for localization of gastrinoma in the Zollinger-Ellison syndrome. Ann Surg. 1987; 205:230–9.
  188. Isenberg J, Csendes A, Walsh JH. Resting and pentagastrin-stimulated gastroesophageal sphincter pressure in patients with Zollinger-Ellison syndrome. Gastroenterology. 1971; 61:655–8.
  189. Isenberg JI, Walsh JH, Passaro E Jr, Moore EW, Grossman ME. Unusual effect of secretin on serum gastrin, serum calcium and gastric acid secretion in a patient with suspected Zollinger- Ellison syndrome. Gastroenterology. 1972; 62:626–31.
  190. Jackson CE, Boonstra CE. The relationship of hereditary hyperparathyroidism to endocrine adenomatosis. Am J Med. 1967; 43:727–34.
  191. Jaffe BM, Peskin GW, Kaplan EL. Serum levels of parathyroid hormone in the Zollinger-Ellison syndrome. Surgery. 1973; 74:621–5.
  192. Jahnke RW, Gnekow W, Harell GS. Non-beta islet cell tumor calcification associated with Zollinger-Ellison syndrome and multiple endocrine adenomatosis. Gastrointest Radiol. 1977; 1:345–7.
  193. Jakobsen AM, Andersson P, Saglik G, Andersson E, Kolby L, Erickson JD, Forssell-Aronsson E, Wangberg B, Ahlman H, Nilsson O. Differential expression of vesicular monoamine transporter (VMAT) 1 and 2 in gastrointestinal endocrine tumours. J Pathol. 2001; 195:463–72.
  194. Jansen JB, Lamers CB. Effects of changes in serum calcium on secretin-stimulated serum gastrin in patients with Zollinger-Ellison syndrome. Gastroenterology. 1982; 83:173–8.
  195. Jensen RT. Gastrinoma. Baillieres Clin Gastroenterol. 1996; 10:555–766.
  196. Jensen RT. Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type 1. J Intern Med. 1998; 243:477–88.
  197. Jensen RT, Fraker DL. Zollinger-Ellison syndrome: Advances in Treatment of the Gastric Hypersecretion and the Gastrinoma. JAMA. 1994; 271:1–7.
  198. Jensen RT, Gardner JD, Raufman JP, Pandol SJ, Doppman JL, Collen MJ. Zollinger-Ellison syndrome: current concepts and management. Ann Intern Med. 1983; 98:59–75.
  199. Johnson GJ, Summerskill WH, Anderson VE, Keating FR Jr. Clinical and genetic investigation of a large kindred with multiple endocrine adenomatosis. N Engl J Med. 1967; 277:1379–85.
  200. Jones CF, Liang D, Moffitt RE. Polyglandular endocrine adenomatosis—presenting as hyperaldosteronism. R I Med J. 1967; 50:338–40.
  201. Jordan PH Jr. A personal experience with pancreatic and duodenal neuroendocrine tumors. J Am Coll Surg. 1999; 189:470–82.
  202. Jose CC, Varghese CV, Singh AD. Thymic carcinoid in a case of Zollinger-Ellison syndrome. A case report. Australas Radiol. 1988; 32:408–9.
  203. Kaczirek K, Prager G, Schindler M, Kaserer K, Gessl A, Vierhapper H, Niederle B. Multiple endocrine neoplasia 1—current recommendations for diagnosis and treatment. Wien Klin Wochenschr. 2002; 114:258–66.
  204. Kaiser VW, Schmidt GA, Beutler W. Polyendokrine adenomatose—ein beitrag zur differentialdiagnose hyperparathyreotischer symptome. Zschr inn Med. 1972; 27:355–61.
  205. Kaplan EL, Horvath K, Udekwu A, Straus F, Schark C, Ferguson DJ, Skinner DB. Gastrinomas: a 42-year experience. World J Surg. 1990; 14:365–75.
  206. Karges W, Schaaf L, Dralle H, Boehm BO. Concepts for screening and diagnostic follow-up in multiple endocrine neoplasia type 1 (MEN). Exp Clin Endocrinol Diabetes. 2000; 108:334–400.
  207. Kato M, Imamura M, Hosotani R, Shimada Y, Doi R, Itami A, Komoto I, Kosaka MTT, Konishi J. Curative resection of microgastrinomas based on the intraoperative secretin test. World J Surg. 2000; 24:1425–30.
  208. Kaura D, Singh K, Murlidharan R, Singh V, Bhasin D, Suri S, Katariya RN. Multiple endocrine neoplasia type-1. Indian J Gastroenterol. 1994; 13:141–2.
  209. Kerr GD, Smith R. Hypercalcaemia and gastric hypersecretion in the familial endocrine-adenoma syndrome. Lancet. 1967; 1:1074–7.
  210. Kinoshita Y, Nonaka H, Yamaguchi H, Ichikawa M, Suzuki S, Watanabe J, Kondo T, Chiba T, Chihara K, Fukase M, Fujita T. [A case of multiple endocrine neoplasia type 1 with growth hormone and prolactin secreting pituitary adenoma, functioning large parathyroid cyst, and Zollinger-Ellison syndrome]. Nippon Naika Gakkai Zasshi. 1986; 75:512–21.
  211. Kloppel G, Willemar S, Stamm B, Hacki WH, Heitz PU. Pancreatic lesions and hormonal profile of pancreatic tumors in multiple endocrine neoplasia type I. An immunocytochemical study of nine patients. Cancer. 1986; 57:1824–32.
  212. Kocandrle V, Kittle F, Oberhelman HA Jr. Pulmonary resection for metastasis in nonbeta islet cell tumor of pancreas. Am Surg. 1970; 172:49–52.
  213. Kolby L, Wangberg B, Ahlman H, Jansson S, Foressell-Aronsson E, Erickson JD, Nilsson O. Gastric carcinoid with histamine production, histamine transporter and expression of somatostatin receptors. Digestion. 1998; 59:160–6.
  214. Komminoth P, Heitz PU, Kloppel G. Pathology of MEN-1: morphology, clinicopathologic correlations and tumour development. J Intern Med. 1998; 243:455–64.
  215. Kouvaraki MA, Lee JE, Shapiro SE, Gagel RF, Sherman SI, Sellin RV, Cote GJ, Evans DB. Genotype-phenotype analysis in multiple endocrine neoplasia type 1. Arch Surg. 2002; 137:641–7.
  216. Kovacs K, Horvath E, Kerenyi NA. Prolactin cell adenomas associated with the multiple endocrine adenomatosis syndrome. Pathology. 1977; 5:446–52.
  217. Lamers CB, Berretty PJ, Bijvoet OL, Joosten HJ, Lubbers EJ, Muller H, Nadorp JH, Nielander DO, Penn W, Prenen H, Schillings PH, Termote UA, Van Tongeren JH. The Zollinger-Ellison syndrome. Neth J Med. 1974; 17:94–107.
  218. Lamers CB, Diemel CM. Basal and postatropine serum pancreatic polypeptide concentrations in familial multiple endocrine neoplasia type 1. J Clin Endocrinol Metab. 1982; 55:774–8.
  219. Lamers CB, Festen HP, Van Tongeren JH. Long-term treatment with histamine H2-receptor antagonists in Zollinger-Ellison syndrome. Am J Gastroenterol. 1978; 70:286–91.
  220. Lamers CB, Jansen JB, Roeffen W. Secretin injection test in the diagnosis of gastrinoma. Gastroenterology. 1981; 80:1615–6.
  221. Lamers CB, Rotter JI, Jansen JB. Gastrin cell function in familial multiple endocrine neoplasia type I. Gut. 1988; 29:1358–63.
  222. Lamers CB, Stadil F, Van Tongeren JH. Prevalence of endocrine abnormalities in patients with the Zollinger-Ellison syndrome and in their families. Am J Med. 1978; 64:607–12.
  223. Lammli J, Ammann R, Knoblauch M, Largiader F, Sauberli H, Hedinger C, Joris F, Woodtli W, Siegenthaler W. Daslinische spektrum des Zollinger-Ellison syndroms. Schweiz Med Wochenschr. 1976; 106:1745–7.
  224. Langer P, Cupisti K, Bartsch DK, Nies C, Goretzki PE, Rothmund M, Roher HD. Adrenal involvement in multiple endocrine neoplasia type 1. World J Surg. 2002; 26:891–6.
  225. Larkin CJ, Joy ES, Ardill JE, Johnston CF, Collins JS, Buchanan KD. Gastrinomas and the change in their presentation and management in Northern Ireland, UK, from 1970 to 1996. Eur J Gastroenterol Hepatol. 1998; 10:947–52.
  226. LeBodic MF, Heymann MF, Lecomte M, Berger N, Berger F, Louvel A, DeMicco C, Patey M, deMascarel A, Burtin F, Saint-Andre JP. Immunohistochemical study of 100 pancreatic tumors in 28 patients with Multiple Endocrine Neoplasia, Type 1. Am J Surg Pathol. 1996; 20:1378–84.
  227. LeBriggs R, Powell JR. Chiari-Frommel syndrome as a part of the Zollinger-Ellison multiple endocrine adenomatosis complex. Calif Med. 1969; 111:92–6.
  228. Lehy T, Cadiot G, Mignon M, Ruszniewski P, Bonfils S. Influence of multiple endocrine neoplasia type 1 on gastric endocrine cells in patients with the Zollinger-Ellison syndrome. Gut. 1992; 33:1275–9.
  229. Lehy T, Mignon M, Cadiot G, Elouaer-Blanc L, Ruszniewski P, Lewin MJ, Bonfils S. Gastric endocrine cell behavior in Zollinger-Ellison patients upon long-term potent antisecretory treatment. Gastroenterology. 1989; 96:1029–40.
  230. Lips CJ, Vasen HF, Lamers CB. Multiple endocrine neoplasia syndromes. Crit Rev Oncol Hematol. 1984; 2:117–84.
  231. Liu SW, van de Velde JH, Heslinga JM, Kievit J, Roelfsema F. Acromegaly caused by growth hormone-relating hormone in a patient with multiple endocrine neoplasia type 1. Jpn J Clin Oncol. 1996; 26:49–52.
  232. London JF, Shawker TH, Doppman JL, Frucht HH, Vinayek R, Stark HA, Miller LS, Miller DL, Norton JA, Gardner JD, Jensen RT. Zollinger-Ellison syndrome: prospective assessment of abdominal US in the localization of gastrinomas. Radiology. 1991; 178:763–7.
  233. Lucey MR, McCann S, Weir DG. A family with Multiple Endocrine Neoplasia Type 1. Ir J Med Sci. 1983; 152:99–102.
  234. Lulu DJ, Corcoran TE, Andre M. Familial endocrine adenomatosis with associated Zollinger-Ellison syndrome. Wermer’s syndrome. Am J Surg. 1968; 115:695–701.
  235. Luttwak EM, Saltz NJ. The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia. Surg Gynecol Obstet. 1988; 167:415–9.
  236. Lye WC, Lee KO, Tay HH. Multiple endocrine neoplasia type 1—presenting with impotence. Singapore Med J. 1990; 31:277–9.
  237. MacFarlane MP, Fraker DL, Alexander HR, Norton JA, Jensen RT. A prospective study of surgical resection of duodenal and pancreatic gastrinomas in multiple endocrine neoplasia Type 1. Surgery. 1995; 118:973–80.
  238. Macleod AF, Ayers B, Young AE, Medd WE, Sonksen PH. Resolution of hypergastrinaemia after parathyroidectomy in multiple endocrine neoplasia syndrome type I (MEN type I). Clin Endocrinol (Oxf). 1987; 26:693–8.
  239. Mai HD, Sanowski RA. Regression of duodenal gastrinomas in a patient with multiple endocrine neoplasia type I after parathyroidectomy. Gastrointest Endosc. 1992; 38:706–8.
  240. Maiter D, Lambert M, Alhajje A, Dugernier T, Crabbe J, Fiasse R. Multiple endocrine neoplasia type I (MEN I). A wide range of clinical presentation. Report of three cases. Acta Clin Belg. 1986; 41:89–95.
  241. Maitra A, Krueger JE, Tascilar M, Offerhaus GJ, Angeles-Angeles A, Klimstra DS, Hruban RH, Albores-Saavedra J. Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases. Am J Surg Pathol. 2000; 24:1501–10.
  242. Majewski JT, Wilson SD. The MEN-I syndrome: an all or none phenomenon? Surgery. 1979; 86:475–84.
  243. Malagelada JR. Pathophysiological responses to meals in the Zollinger-Ellison syndrome: 1. Paradoxical postprandial inhibition of gastric secretion. Gut. 1978; 19:284–9.
  244. Malagelada JR, Davis CS, O’Fallon WM, Go VL. Laboratory diagnosis of gastrinoma. I. A prospective evaluation of gastric analysis and fasting serum gastrin levels. Mayo Clin Proc. 1982; 57:211–8.
  245. Malagelada JR, Edis AJ, Adson MA, Van Heerden JA, Go VLW. Medical and surgical options in the management of patients with gastrinoma. Gastroenterology. 1983; 84:1524–32.
  246. Mansbach CM2, Wilkins RM, Dobbins WO, Tyor MP. Intestinal mucosal function and structure in the steatorrhea of Zollinger-Ellison syndrome. Arch Intern Med. 1968; 121:487–94.
  247. Markowitz AM, Slanetz CA Jr, Franz VK. Functioning islet cell tumors of the pancreas: 25-year follow up. Ann Surg. 1961; 154:877–84.
  248. Marx S, Spiegel AM, Skarulis MC, Doppman JL, Collins FS, Liotta LA. Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med. 1998; 129:484–94.
  249. Marx SJ, Agarwal SK, Kester MB, Heppner C, Kim YS, Skarulis MC, Goldsmith PK, Saggar SK, Park SY, Spiegel AM, Burns AL, Debelenko LV, Zhuang Z, Lubensky IA, Liotta LA, Emmert-Buck MR, Guru SC, Manickam P, Crabtree J, Erdos MR, Collins FS, Chandrasekharappa SC. Multiple endocrine neoplasia type 1: clinical and genetic features of the hereditary endocrine neoplasias. Recent Prog Horm Res. 1999; 54:397–439.
  250. Marx SJ, Spiegel AM, Brown EM, Aurbach GD. Family studies in patients with primary parathyroid hyperplasia. Am J Med. 1977; 62:698–706.
  251. Marx SJ, Vinik AI, Santen RJ, Floyd JC Jr, Mills JL, Green J. Multiple endocrine neoplasia type I: assessment of laboratory tests to screen for the gene in a large kindred. Medicine (Baltimore). 1986; 65:226–41.
  252. Mathis P, Segal S, Beer Gabel M, Diebold MD, Hochlaf S. [Familial forms of endocrine tumors of the pancreas. A propos of 4 cases in the same family]. Chirurgie. 1989; 115:461–5.
  253. Maton PN, Frucht H, Vinayek R, Wank SA, Gardner JD, Jensen RT. Medical management of patients with Zollinger-Ellison syndrome who have had previous gastric surgery: A prospective study. Gastroenterology. 1988; 94:294–9.
  254. Maton PN, Gardner JD, Jensen RT. Cushing’s syndrome in patients with Zollinger-Ellison syndrome. N Engl J Med. 1986; 315:1–5.
  255. Maton PN, Gardner JD, Jensen RT. Diagnosis and management of Zollinger-Ellison syndrome. Endocrinol Metab Clin North Am. 1989; 18:519–43.
  256. Maton PN, Lack EE, Collen MJ, Cornelius MJ, David E, Gardner JD, Jensen RT. The effect of Zollinger-Ellison syndrome and omeprazole therapy on gastric oxyntic endocrine cells. Gastroenterology. 1990; 99:943–50.
  257. Maton PN, Miller DL, Doppman JL, Collen MJ, Norton JA, Vinayek R, Slaff JI, Wank SA, Gardner JD, Jensen RT. Role of selective angiography in the management of Zollinger- Ellison syndrome. Gastroenterology. 1987; 92:913–8.
  258. Mayr B, Apenberg S, Rothamel T, von zur Muhlen A, Brabant G. Menin mutations in patients with multiple endocrine neoplasia type 1. Eur J Endocrinol. 1997; 137:684–7.
  259. McCarthy DM, Olinger EJ, May RJ, Long BW, Gardner JD. H2-histamine receptor blocking agents in the Zollinger-Ellison syndrome. Experience in seven cases and implications for long-term therapy. Ann Intern Med. 1977; 87:668–75.
  260. McCarthy DM, Peikin SR, Lopatin RN, Long BW, Spiegel A, Marx S, Brennan A. Hyperparathyroidism a reversible cause of cimetidine-resistant gastric hypersecretion. Br Med J. 1979; 1:1765–6.
  261. McGuigan JE, Colwell JA, Franklin J. Effect of parathyroidectomy on hypercalcemic hypersecretory peptic ulcer disease. Gastroenterology. 1974; 66:269–72.
  262. McGuigan JE, Wolfe MM. Secretin injection test in the diagnosis of gastrinoma. Gastroenterology. 1980; 79:1324–31.
  263. McKeeby JL, Li X, Zhuang Z, Vortmeyer AO, Huang S, Pirner M, Skarulis MC, James-Newton L, Marx SJ, Lubensky IA. Multiple leiomyomas of the esophagus, lung, and uterus in multiple endocrine neoplasia type 1. Am J Pathol. 2001; 159:1121–7.
  264. Mee AS, Ismail S, Bornman PC, Marks IN. Changing concepts in the presentation, diagnosis and management of the Zollinger-Ellison syndrome. Q J Med. 1983; 52:256–67.
  265. Melvin WS, Johnson JA, Sparks J, Innes JT, Ellison EC. Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia. Surgery. 1993; 114:1183–8.
  266. Metz DC, Pisegna JR, Fishbeyn VA, Benya RV, Feigenbaum KM, Koviack PD, Jensen RT. Currently used doses of omeprazole in Zollinger-Ellison syndrome are too high. Gastroenterology. 1992; 103:1498–508.
  267. Metz DC, Pisegna JR, Fishbeyn VA, Benya RV, Jensen RT. Control of gastric acid hypersecretion in the management of patients with Zollinger-Ellison syndrome. World J Surg. 1993; 17:468–80.
  268. Metz DC, Pisegna JR, Ringham GL, Feigenbaum KM, Koviack PD, Maton PN, Gardner JD, Jensen RT. Prospective study of efficacy and safety of lansoprazole in Zollinger-Ellison syndrome. Dig Dis Sci. 1993; 38:245–56.
  269. Metz DC, Strader DB, Orbuch M, Koviack PD, Feigenbaum KM, Jensen RT. Use of omeprazole in Zollinger-Ellison: A prospective nine-year study of efficacy and safety. Aliment Pharmacol Ther. 1993; 7:597–610.
  270. Mieher WC, Hartsock RJ, Geokas MC, Ballard HS, Frame B. Peptic ulcer as a manifestation of familial polyendocrine disease. JAMA. 1962; 179:854–8.
  271. Mignon M, Accary JP, Bonfils S. Signification diagnostique du test a la secretine dans le syndrome de Zollinger-Ellison. Gastroenterol Clin Biol. 1977; 1:425–34.
  272. Mignon M, Cadiot G. Diagnostic and therapeutic criteria in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1. J Intern Med. 1998; 243:489–94.
  273. Mignon M, Lehy T, Bonnefond A, Ruszniewski P, Labeille D, Bonfils S. Development of gastric argyrophil carcinoid tumors in a case of Zollinger-Ellison syndrome with primary hyperparathyroidism during long term antisecretory treatment. Cancer. 1987; 59:1959–62.
  274. Mignon M, Rigaud D, Cambray S, Chayvialle JA, Accary JP, Rene E, Vatier J, Bonfils S. A comparative evaluation of secretin bolus and secretin infusion as secretin provocation tests in the Zollinger-Ellison syndrome. Scand J Gastroenterol. 1985; 20:791–7.
  275. Mignon M, Ruszniewski P, Haffar S, Rignaud D, Rene E, Bonfils S. Current approach to the management of tumoral process in patients with gastrinoma. World J Surg. 1986; 10:703–10.
  276. Mignon M, Ruszniewski P, Podevin P, Sabbagh L, Cadiot G, Rigaud D, Bonfils S. Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia type 1. World J Surg. 1993; 17:489–97.
  277. Mignon M, Vallot T, Galmiche JP, Dupas JL, Bonfils S. Interest of a combined anti-secretory treatment, cimetidine and pirenzepin, in the management of severe forms of Zollinger-Ellison syndrome. Digestion. 1981; 20:56–61.
  278. Miller LS, Doppman J, Maton PN, Gardner JD, Jensen RT. Zollinger-Ellison syndrome. Advances in diagnosis and treatment. Handbk Exp Pharmacol. 1991; 99:349–400.
  279. Miller LS, Vinayek R, Frucht H, Gardner JD, Jensen RT, Maton PN. Reflux esophagitis in patients with Zollinger-Ellison syndrome. Gastroenterology. 1990; 98:341–6.
  280. Miyata M, Nakao K, Sakamoto T, Tsumori T, Hamaji M, Himeno S, Kawashima Y. Removal of mesenteric gastrinoma: a case report. Surgery. 1986; 99:245–8.
  281. Modlin IM, Lamers CBH. Multiple endocrine neoplasia. Part I. Wermer’s syndrome. S Afr Med J. 1979; 56:485–8.
  282. Moldawer M. Multiple endocrine tumors and Zollinger-Ellison syndrome in families: one or two syndromes? A report of two new families. Metabolism. 1962; 11:153–66.
  283. Moldawer MP, Nardi GL, Raker JW. Concomitance of multiple adenomas of the parathyroids and pancreatic islet cells with tumor of the pituitary; a syndrome with familial incidence. Am J Med Sci. 1954; 228:190–206.
  284. Mommens JP, Limet R, Vosse-Matagne G, Heynen G, Franchimont P, Epstein S, Honore D. Role of calcitonin in the Zollinger-Ellison syndrome combined with hyperparathyroidism. Acta Chir Belg. 1976; 6:606–11.
  285. Morelli A, Falchetti A, Martineti V, Becherini L, Mark M, Friedman E, Brandi ML. MEN1 gene mutation analysis in Italian patients with multiple endocrine neoplasia type 1. Eur J Endocrinol. 2000; 142:131–7.
  286. Murakami S, Satomi A, Ishida K, Matsuki M, Koda H, Miura S. Multiple endorcine neoplasia type 1 presenting as insulinoma, gastrinoma, and postubulbar duodenal ulcers: report of a case. Surg Today. 1995; 25:848–51.
  287. Murat A, Heymann MF, Bernat S, Dupas B, Delajartre AY, Calender A, Despins P, Michaud JL, Giraud S, LeBodic MF, Charbonnel B. [Thymic and bronchial neuroendocrine tumors in multiple endocrine neoplasia type 1. GENEM1]. Presse Med. 1997; 26:1616–21.
  288. Murie N, Chauvel P, Faire J, Croisier JC, Martin E, Moreaux J, Sabouraud O. Wermer’s syndrome or familial polyendocrine adenomatous hyperplasia (2 anatomo-clinical cases. Ann Endocrinol (Paris). 1973; 34:190–205.
  289. Murphy RT, Goodsitt E, Morales H, Bilton JL. Peptic ulceration with associated endocrine tumors. Am J Surg. 1960; 100:764–78.
  290. Nakayasu A, Dowell AJ, Reynolds C, Stordy SN, Cleator IG. Zollinger-Ellison syndrome associated with parathyroid adenomas and ectopic gastric tissue in the lower esophageal mucosa. Can J Surg. 1979; 22:71–8.
  291. Nguyen BD, Wang EA. Multiple endocrine neoplasia type 1: scintigraphic and magnetic resonance demonstration. Clin Nucl Med. 1999; 24:73–4.
  292. Nord B, Platz A, Smoczynski K, Kytola S, Robertson G, Calender A, Murat A, Weintraub D, Burgess J, Edwards M, Skogseid B, Owen D, Lassam N, Hogg D, Larsson C, Teh BT. Malignant melanoma in patients with multiple endocrine neoplasia type 1 and involvement of the MEN1 gene in sporadic melanoma. Int J Cancer. 2000; 87:463–7.
  293. Norton JA, Alexander HA, Fraker DL, Venzon DJ, Gibril F, Jensen RT. Possible primary lymph node gastrinomas: occurrence, natural history and predictive factors: A prospective study. Ann Surg. 2003; 237:650–9.
  294. Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT. Comparison of surgical results in patients with advanced and limited disease with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome. Ann Surg. 2001; 234:495–506.
  295. Norton JA, Cornelius MJ, Doppman JL, Maton PN, Gardner JD, Jensen RT. Effect of parathyroidectomy in patients with hyperparathyroidism, Zollinger-Ellison syndrome and multiple endocrine neoplasia Type I: A prospective study. Surgery. 1987; 102:958–66.
  296. Norton JA, Cromack DT, Shawker TH, Doppman JL, Comi R, Gorden P, Maton PN, Gardner JD, Jensen RT. Intraoperative ultrasonographic localization of islet cell tumors. A prospective comparison to palpation. Ann Surg. 1988; 207:160–8.
  297. Norton JA, Doppman JL, Collen MJ, Harmon JW, Maton PN, Gardner JD, Jensen RT. Prospective study of gastrinoma localization and resection in patients with Zollinger-Ellison syndrome. Ann Surg. 1986; 204:468–79.
  298. Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J, Goebel SU, Peghini P, Roy PK, Gibril F, Jensen RT. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999; 341:635–44.
  299. Norton JA, Jensen RT. Unresolved surgical issues in the management of patients with the Zollinger-Ellison syndrome. World J Surg. 1991; 15:151–9.
  300. Norton JA, Jensen RT. Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia type 1 (MEN1). Surg Oncol. 2003; 12:145–51.
  301. Norton JA, Warren RS, Kelly MG, Zuraek MB, Jensen RT. Aggressive surgery for metastatic liver neuroendocrine tumors. Surgery. 2003; 134:1051–65
  302. Norton KS, Zibari GB, Johnson LW. Use of the sestomabi scan to identify hyperplatic parathyroid tissue in the forearm of a patient with MEN1 syndrome. J La State Med Soc. 2001; 153:605–7.
  303. Obara T, Fujimoto Y, Ito Y. Primary hyperparathyroidism in patients with multiple endocrine neoplasia type 1: experience by a single surgical team in Japan. Henry Ford Hosp Med J. 1992; 40:191–4.
  304. Oberg K, Bostrom H, Fahrenkrug J, Dymling JF, Shaffalitsky de Muckadell OB, Lundqvist G. Streptozotocin treatment of a pancreatic tumour producing VIP and gastrin associated with Verner-Morrison syndrome. Acta Med Scand. 1979; 206:223–7.
  305. Oberg K, Skogseid B, Eriksson B. Multiple endocrine neoplasia type 1 (MEN-1). Clinical, biochemical and genetical investigations. Acta Oncol. 1989; 28:383–7.
  306. Oberg K, Walinder O, Bostrom H, Lundqvist G, Wide L. Peptide hormone markers in screening for endocrine tumors in multiple endocrine adenomatosis type I. Am J Med. 1982; 73:619–30.
  307. O’Brien T, O’Riordan DS, Gharib H, Scheithauer BW, Ebersold MJ, vanHeerden JA. Results of treatment of pituitary disease in multiple endocrine neoplasia, type 1. Neurosurgery. 1996; 39:273–8.
  308. O’Byrne KJ, Goggins MG, McDonald GS, Daly PA, Kelleher DP, Weir DG. A metastatic neuroendocrine anaplastic small cell tumor in a patient with multiple endocrine neoplasia type 1 syndrome. Cancer. 1994; 74:2374–8.
  309. Ohye H, Sato M, Matsubara S, Miyauchi A, Kishi-Imai K, Murao K, Takahara J. A novel germline mutation of Multiple Endocrine Neoplasia Type 1 (MEN1) gene in a Japanese MEN1 patient and her daughter. Endocrine J. 1999; 46:325–9.
  310. Otten MH, Birkenhager JC, van Blankenstein M. Zollinger-Ellison syndrome treated by endoscopic removal of a duodenal gastrinoma. Neth J Med. 1978; 21:248–51.
  311. Pack S, Turner ML, Zhuang Z, Vortmeyer AO, Boni R, Skarulis M, Marx SJ, Darling TN. Cutaneous tumors in patients with multiple endocrine neoplasia type 1 show allelic deletion of the MEN1 gene. J Invest Dermatol. 1998; 110:438–40.
  312. Paja Fano M, Goni Goicoechea F, Yoldi Arrieta A, Ramon Elorza Olabegoya J. [Type 1 multiple enodcrine neoplasia associated to thymic carcinoid. Clinical and genetic study of one case]. Med Clin (Barc). 2001; 116:39.
  313. Papillon E, Rolachon A, Calender A, Chabre O, Barnoud R, Fournet J. A malignant gastrointestinal stromal tumour in a patient with multiple endocrine neoplasia type 1. Eur J Gastroenterol Hepatol. 2001; 13:207–11.
  314. Passaro E Jr, Basso N, Sanchez RE, Gordon HE. Newer studies in the Zollinger-Ellison syndrome. Am J Surg. 1970; 120:138–43.
  315. Passaro E Jr, Basso N, Walsh JH. Calcium challenge in the Zollinger-Ellison syndrome. Surgery. 1972; 72:60–7.
  316. Pearl MA, Sternberg WH, Dingman JF. Unusual cases of hyperparathyroidism. Arch Intern Med. 1962; 110:481–92.
  317. Peghini PL, Annibale B, Azzoni C, Milione M, Corleto VD, Gibril F, Venzon DJ, Delle Fave G, Bordi C, Jensen RT. Effect of chronic hypergastrinemia on human enterochromaffin-like cells: insights from patients with sporadic gastrinomas. Gastroenterology. 2002; 123:68–85.
  318. Pender B. Islet-cell tumour of pancreas associated with peptic ulceration. Lancet. 1959; 1:123–4.
  319. Perrier CV. The Zollinger-Ellison syndrome. Its place in the pathophysiology of gastric acid secretion and of its hormonal regulation. Ergeb Inn Med Kinderheilkd. 1965; 23:89–124.
  320. Petty EM, Green JS, Marx SJ, Taggart RT, Farid N, Bale AE. Mapping the gene for hereditary hyperparathyroidism and prolactinoma (MEN1Burin) to chromosome 11q: evidence for a founder effect in patients from Newfoundland. Am J Hum Genet. 1994; 54:1060–6.
  321. Peurifoy JT, Gomez LG, Thompson JC. Separate pancreatic gastrin cell and beta-cell adenomas: report of a patient with multiple endocrine adenomatosis type 1. Arch Surg. 1979; 114:956–8.
  322. Pinakapani S, Pai KR, Devi CS, Reddy CRRM, Rao NR. Multiple endocrine adenoma peptic ulcer complex. J Assoc Physicians India. 1969; 17:105–8.
  323. Pipeleers-Marichal M, Somers G, Willems G, Foulis A, Imrie C, Bishop AE, Polak JM, Hacki WH, Stamm B, Heitz PU, Kloppel G. Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. N Engl J Med. 1990; 322:723–7.
  324. Pisegna JR, Doppman JL, Norton JA, Metz DC, Jensen RT. Prospective comparative study of ability of MR imaging and other imaging modalities to localize tumors in patients with Zollinger-Ellison syndrome. Dig Dis Sci. 1993; 38:1318–28.
  325. Poncin J, Abs R, Velkeniers B, Bonduelle M, Abramowicz M, Legros JJ, Verloes A, Meurisse M, VanGaal L, Verellen C, Koulischer L, Beckers A. Mutation analysis of the MEN1 gene in Belgian patients with multiple endocrine neoplasia type 1 and related diseases. Hum Mutat. 1999; 13:54–60.
  326. Poynard T, Mignon M, Accary JP, Bonfils S. Sensitivity, specificity and predictive values of the secretin infusion test in the diagnosis of gastrinoma. Gastroenterol Clin Biol. 1986; 10:492–6.
  327. Price DE, Absalom SR, Davidson K, Bolia A, Bell PR, Howlett TA. A case of multiple endocrine neoplasia: hyperparathyroidism, insulinoma, GRF-oma, hypercalcitoninaemia and intractable peptic ulceration. Clin Endocrinol (Oxf). 1992; 37:187–8.
  328. Primrose JN, Joffe SN. Hyperparathyroidism and hypergastrinemia revisited. Surgery. 1984; 96:1144–50.
  329. Prosser PR, Karam JH, Townsend JJ, Forsham PH. Prolactin-secreting pituitary adenomas in multiple endocrine adenomatosis, type I. Ann Intern Med. 1979; 91:41–4.
  330. Proye C, Lagache G, Jonas C, Roger J. Ulceres recidivants apres gastrectomie et hyperparathyroidie primaire. Chirurgie. 1979; 105:56–60.
  331. Pumarino H, Csendes A, Vivanco N, Stevenson C, Pineda G. Sindrome de adenomas endocrinos multiples (AEM) tipo I (SAEM I) forma familiar. Rev Med Chile. 1980; 108:221–32.
  332. Quintana D, Walter M, Jacocks A, Kida M. 47-year-old man with left flank pain: a clinical pathological correlation conference of the University of Oklahoma College of Medicine. J Okla State Med Assoc. 1998; 91:494–503.
  333. Raufman JP, Collins SM, Pandol SJ, Korman LY, Collen MJ, Cornelius MJ, Feld MK, McCarthy DM, Gardner JD, Jensen RT. Reliability of symptoms in assessing control of gastric acid secretion in patients with Zollinger-Ellison syndrome. Gastroenterology. 1983; 84:108–13.
  334. Regan PT, Malagelada JR. A reappraisal of clinical, roentgenographic, and endoscopic features of the Zollinger-Ellison syndrome. Mayo Clin Proc. 1978; 53:19–23.
  335. Reis HE, Kanzler G, Schulz P, Teske H-J. Polyendokrine adenomatose. Dtsch Med Wochenschr. 1971; 96:1920–3.
  336. Reiswig OK, Carter R. Polyendocrine adenomatosis with the Zollinger-Ellison syndrome. Successful treatment by total gastrectomy with a Lawrence Pouch. Am Surg. 1965; 31:695–9.
  337. Richard JL, Ribstein J, Bringer J, Pages M, Jaffiol C, Mirouze J. Adenomatose polyendocrinienne de type I avec tumeur a prolactine revelee par une apoplexie hypophysaire. Nouv Presse Med. 1981; 10:3149–52.
  338. Richardson CT, Peters MN, Feldman M, McClelland RN, Walsh JH, Cooper KA, Willeford G, Dickerman RM, Fordtran JS. Treatment of Zollinger-Ellison syndrome with exploratory laparotomy, proximal gastric vagotomy, and H2-receptor antagonists. A prospective study. Gastroenterology. 1985; 89:357–67.
  339. Rigaud D, Bauer P, Hervoir P, Fasano JJ, Moreau J, Mignon M, Bonfils S. Association hyperparathyroidie-syndrome de Zollinger-Ellison. Nouv Presse Med. 1982; 11:1299–303.
  340. Rindi G, Azzoni C, La Rosa S, Klersy C, Paolotti D, Rappel S, Stolte M, Capella C, Bordi C, Solcia E. ECL cell tumor and poorly differentiated endocrine carcinoma of the stomach: prognostic evaluation by pathological analysis. Gastroenterology. 1999; 116:532–42.
  341. Rindi G, Bordi C, Rappel S, La Rosa S, Stolte M, Solcia E. Gastric carcinoids and neuroendocrine carcinomas: pathogenesis, pathology, and behavior. World J Surg. 1996; 20:168–72.
  342. Rindi G, Luinetti O, Cornaggia M, Capella C, Solcia E. Three subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine carcinoma: a clinicopathologic study. Gastroenterology. 1993; 104:994–1006.
  343. Rindi G, Paolotti D, Fiocca R, Wiedenmann B, Henry JP, Solcia E. Vesicular monoamine transporter 2 as a marker of gastric enterochromaffin-like cell tumors. Virchows Arch. 2000; 436:217–23.
  344. Rogers HM. Parathyroid adenoma and hypertrophy of the parathyroid glands. JAMA. 1946; 130:22–8.
  345. Rogers HM, Keating FR Jr, Morlock CG, Barker NW. Primary hypertrophy and hyperplasia of the parathyroid glands associated with duodenal ulcer. Arch Intern Med. 1947; 79:307–21.
  346. Roijers JF, deWit MJ, van der Luijt RB, Ploos van Amstel HK, Hoppener JW, Lips CJ. Criteria for mutation analysis in MEN1-suspected patients: MEN1 case-findings. Eur J Clin Invest. 2000; 30:487–92.
  347. Rosai J, Higa E. Mediastinal endocrine neoplasm, of probable thymic origin, related to carcinoid tumor. Clinicopathologic study of 8 cases. Cancer. 1972; 29:1061–74.
  348. Rosai J, Higa E, Davie J. Mediastinal endocrine neoplasm in patients with multiple endocrine adenomatosis. A previously unrecognized association. Cancer. 1972; 29:1075–83.
  349. Roy PK, Venzon DJ, Feigenbaum KM, Koviack PD, Bashir S, Ojeaburu JV, Gibril F, Jensen RT. Gastric secretion in Zollinger-Ellison syndrome: correlation with clinical expression, tumor extent and role in diagnosis---A prospective NIH study of 235 patients and a review of 984 cases in the literature. Medicine (Baltimore). 2001; 80:189–222.
  350. Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL, Gibril F, Jensen RT. Zollinger-Ellison syndrome: clinical presentation in 261 patients. Medicine (Baltimore). 2000; 79:379–411.
  351. Rudolf LE, Dammin GF, Moore FD. Intractable peptic ulcer and endocrine adenomas with pituitary amphophilic hyperplasia. Surgery. 1960; 48:170–84.
  352. Ruppert RD, Greenberger NJ, Beman FM, McCullough FM. Gastric secretion in ulcerogenic tumors of the pancreas. Ann Intern Med. 1967; 67:808–15.
  353. Ruszniewski P, Amouyal P, Amouyal G, Grange JD, Mignon M, Bouch O, Bernades P. Localization of gastrinomas by endoscopic ultrasonography in patients with Zollinger-Ellison syndrome. Surgery. 1995; 117:629–35.
  354. Ruszniewski P, Podevin P, Cadiot G, Marmuse JP, Mignon M, Vissuzaine C, Bonfils S, Lehy T. Clinical, anatomical, and evolutive features of patients with the Zollinger-Ellison syndrome combined with type I multiple endocrine neoplasia. Pancreas. 1993; 8:295–304.
  355. Ruszniewski P, Ramdani A, Cadiot G, Lehy T, Mignon M, Bonfils S. Long-term treatment with octreotide in patients with the Zollinger-Ellison syndrome. Eur J Clin Invest. 1993; 23:296–301.
  356. Ruttman E, Kloppel G, Bommer G, Kiehn M, Heitz PU. Pancreatic glucagonoma with and without syndrome. Virchows Arch A Path Anat Histol. 1980; 338:51–67.
  357. Sahdev A, Jager R. Bilateral pituitary adenomas occurring with multiple endocrine neoplasia type one. Am J Neuroradiol. 2000; 21:1067–9.
  358. Saint EG, Finlay-Jones R, Stewart HH. Familial endocrine adenomatosis with ulcerogenic tumour of the pancreas. Aust Ann Med. 1961; 10:308–16.
  359. Samaan NA, Ouais S, Ordonez NG, Choksi UA, Sellin RV, Hickey RC. Multiple endocrine syndrome type I. Cancer. 1989; 64:741–52.
  360. Sanchez GC, Sommers SC. Peptic ulcer diathesis with a mixed adenocarcinoma of the pancreas. Gastroenterology. 1960; 38:467–70.
  361. Sanchez RE, Longmire WP Jr, Passaro E Jr. Acid secretion and serum gastrin levels in the Zollinger-Ellison syndrome. Calif Med. 1972; 116:1–7.
  362. Sano T, Yamasaki R, Saito H, Hirose T, Kudo E, Kameyama K, Hiraishi K, Saito G, Hizawa K. Growth hormone-releasing hormone (GHRH)-secreting pancreatic tumor in a patient with multiple endocrine neoplasia type I. Am J Surg Pathol. 1987; 11:810–9.
  363. Santini D, Pasquinelli G, Alessandro LD, Mazzolem G, Taffurelli M, Campione O, Marrano D, Martinelli GN. Parathyroid gastrin and parathormone-producing tumour in the Zollinger-Ellison syndrome of MEN 1 origin. Virchows Archiv A Pathol Anat. 1991; 419:433–7.
  364. Sardi A, Singer JA. Insulinoma and gastrinoma in Wermer’s disease (MEN I). Arch Surg. 1987; 122:835–6.
  365. Satta J, Ahonen A, Parkkila S, Leinonen L, Apaja-Sarkkinen M, Lepojarvi M, Juvonen T. Multiple endocrine neoplastic-associated thymic carcinoid tumor in close relatives: octreotide scan as a new diagnostic and follow-up modality. Scand Cardiovasc J. 1999; 33:49–53.
  366. Scappaticci S, Maraschio P, del Ciotto N, Fossati GS, Zonta A, Fraccaro M. Chromosome abnormalities in lymphocytes and fibroblasts of subjects with multiple endocrine neoplasia type 1. Cancer Genet Cytogenet. 1991; 52:85–92.
  367. Schaaf L, Greschner M, Geissler W, Eckert B, Seif FJ, Usadel KH. The importance of multiple endocrine neoplasia syndromes in differential diagnosis. Klin Wochenschr. 1990; 68:669–72.
  368. Scheithauer BW, Laws ER Jr, Kovacs K, Horvath E, Randall RV, Carney JA. Pituitary adenomas of the multiple endocrine neoplasia type 1 syndrome. Semin Diagn Pathol. 1987; 4:205–11.
  369. Schlaeger R, LeMay M, Wermer P. Upper gastrointestinal tract alterations in adenomatosis of the endocrine glands. Radiology. 1960; 75:517–30.
  370. Schmid JR, Labhart A, Rossier PH. Relationship of multiple endocrine adenomas to the syndrome of ulcerogenic islet cell adenomas (Zollinger-Ellison). Am J Med. 1961; 31:343–53.
  371. Schrumpf E, Stadaas J. Effect of gastric distention on motility and plasma gastrin concentration before and after secretin administration. Scand J Gastroenterol. 1974; 9:119–22.
  372. Shelburne SA, McLaughlin CW. Coincidental adenomas of islet-cells, parathyroid gland and pituitary gland. J Clin Endocrinol. 1945; 5:232–4.
  373. Shepherd JJ. Latent familial multiple endocrine neoplasia in Tasmania. Med J Aust. 1985; 142:395–7.
  374. Shepherd JJ. The natural history of multiple endocrine neoplasia type I. Highly uncommon or highly unrecognized. Arch Surg. 1991; 126:935–52.
  375. Shepherd JJ, Challis DR, Davies PF, McArdle JP, Teh BT, Wilkinson S. Multiple endocrine neoplasm, type 1: Gastrinomas, pancreatic neoplasms, microcarcinoids, the Zollinger-Ellison syndrome, lymph nodes, and hepatic metastases. Arch Surg. 1993; 128:1133–42.
  376. Shepherd JJ, Teh BT, Parameswaran V, David R. Hyperparathyroidism with normal albumin-corrected total calcium in patients with multiple endocrine neoplasia type 1. Henry Ford Hosp Med J. 1992; 40:186–90.
  377. Sheppard BC, Norton JA, Doppman JL, Maton PN, Gardner JD, Jensen RT. Management of islet cell tumors in patients with multiple endocrine neoplasia: A prospective study. Surgery. 1989; 106:1108–17.
  378. Shigematsu S, Shimoyama N, Sato Y, Horita M, Oribe Y, Ono J, Takaki R, Ishimatsu T, Kodama M, Iwashita K. [A case of multiple endocrine neoplasia, type 1, with parathyroid adenoma, multinodular adrenal cortical hyperplasia, islet cell tumor and hyperplasia of thyroid with concomitant hypersecretion of gastrin and total glucagon-like immunoreactivity]. Nippon Naika Gakkai Zasshi. 1989; 78:357–62.
  379. Shoemaker WC, Bland RD, Appel PL. Therapy of critically ill postoperative patients based on outcome prediction and prospective clinical trails. Surg Clin North Am. 1985; 65:811–33.
  380. Shojamanesh H, Gibril F, Louie A, Ojeaburu JV, Bashir S, Abou-Saif A, Jensen RT. Prospective study of the anti-tumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinomas. Cancer. 2002; 94:331–43.
  381. Simeone DM, Scheiman JM, Thompson NW. The “serendipitous” surgical cure of the Zollinger-Ellison syndrome in a patient with multiple endocrine neoplasia type 1 despite an unsuspected diagnosis of either disease. J Clin Gastroenterol. 2001; 32:268–71.
  382. Sircus W. Vagotomy in Zollinger-Ellison syndrome. Gastroenterology. 1979; 79:607–8.
  383. Sitges Serra A. [Zollinger-Ellison syndrome. Experience in 14 cases and a review of the recent literature]. Med Clin (Barc). 1984; 83:550–6.
  384. Sizemore GW, Go VL, Kaplan EL, Sanzenbacher LJ, Holtermuller KH, Arnaud CD. Relations of calcitonin and gastrin in the Zollinger-Ellison syndrome and medullary carcinoma of the thyroid. N Engl J Med. 1973; 288:641–4.
  385. Skogseid B, Ericksson B, Lundqvist G, Lorelius L-E, Rastad J, Wide L, Akerstrom G, Oberg K. Multiple endocrine neoplasia type I: A 10-year prospective screening study in four kindreds. J Clin Endocrinol Metab. 1991; 73:281–7.
  386. Skogseid B, Larsson C, Lindgren PG, Kvanta E, Rastad J, Theodorsson E, Wide L, Wilander E, Oberg K. Clinical and genetic features of adrenocortical lesions in multiple endocrine neoplasia type 1. J Clin Endocrinol Metab. 1992; 75:76–81.
  387. Skogseid B, Oberg K, Eriksson B, Juhlin C, Granberg D, Akerstrom G, Rastad J. Surgery for asymptomatic pancreatic lesion in multiple endocrine neoplasia type 1. World J Surg. 1996; 20:872–7.
  388. Smith R. The Zollinger-Ellison syndrome. Ann R Coll Surg Engl. 1965; 37:160–5.
  389. Snyder N, Scurry M, Hughes W. Hypergastrinemia in familial multiple endocrine adenomatosis. Ann Intern Med. 1974; 80:321–5.
  390. Snyder N, III, Scurry MT, Deiss WP. Five families with multiple endocrine adenomatosis. Ann Intern Med. 1972; 76:53–8.
  391. Soga J, Yakuwa Y. The gastrinoma/Zollinger-Ellison syndrome: statistical evaluation of a Japanese series of 359 cases. J Hep Bil Pancr Surg. 1998; 5:77–85.
  392. Solcia E, Bordi C, Creutzfeldt W, Dayal Y, Dayan AD, Falkmer S, Grimelius L, Havu N. Histopathological classification of nonantral gastric endocrine growths in man. Digestion. 1988; 41:185–200.
  393. Solcia E, Capella C, Fiocca R, Rindi G, Rosai J. Gastric argyrophil carcinoidosis in patients with Zollinger- Ellison syndrome due to type 1 multiple endocrine neoplasia. A newly recognized association. Am J Surg Pathol. 1990; 14:503–13.
  394. Solcia E, Rindi G, LaRosa S, Capella C. Morphological, molecular, and prognostic aspects of gastric endocrine tumors. Microsc Res Tech. 2000; 48:339–48.
  395. Stabile BE, Passaro E Jr, Carlson HE. Elevated serum prolactin level in the Zollinger-Ellison syndrome. Arch Surg. 1981; 116:449–53.
  396. Stage JG, Stadil F. The clinical diagnosis of the Zollinger-Ellison syndrome. Scand J Gastroenterol Suppl. 1979; 53:79–91.
  397. Stamm B, Hedinger CE, Saremaslani P. Duodenal and ampullary carcinoid tumors. A report of 12 cases with pathological characteristics, polypeptide content and relation to MEN 1 syndrome and von Recklinghausen’s disease (neurofibromatosis). Virchows Arch A Pathol Anat Histopathol. 1986; 408:475–89.
  398. Stefanini P, Carboni M, Patrassi N, Basoli A. Beta-islet cell tumors of the pancreas: results of a study on 1,067 cases. Surgery. 1974; 75:597–609.
  399. Stevens MH, Thirlby RC, Richardson CT. Lack of effect of parathyroidectomy or calcium channel blockade on serum gastrin concentration and gastric acid secretion in a patient with hyperparathyroidism and Zollinger-Ellison syndrome. Surgery. 1987; 101:108–13.
  400. St Goar WT. Gastrointestinal symptoms as a clue to the diagnosis of primary hyperparathyroidism: a review of 45 cases. Ann Intern Med. 1956; 46:102–18.
  401. Stocks AE. Problems of the syndrome of familial multiple endocrine adenomas. Australas Ann Med. 1967; 16:278–88.
  402. Stremple JF, Watson CG. Serum calcium, serum gastrin, and gastric acid secretion before and after parathyroidectomy for hyperparathyroidism. Surgery. 1974; 75:841–52.
  403. Sugg SL, Norton JA, Fraker DL, Metz DC, Pisegna JR, Fishbeyn V, Benya RV, Shawker TH, Doppman JL, Jensen RT. A prospective study of intraoperative methods to diagnose and resect duodenal gastrinomas. Ann Surg. 1993; 218:138–44.
  404. Sugimoto K, Oosawa S, Furuta T, Arai H, Sato Y, Iluma M, Yamada M, Suzuki M, Hanai H, Kaneko E. Multiple endocrine neoplasia type 1 accompanied by duodenal carcinoid tumors and hypergastrinemia: a familial case. Intern Med. 1995; 34:649–53.
  405. Summerskill WH, Code CF, Hallenbeck GA, Priestley JT. Intractable peptic ulcer in hereditary endocrine-ulcer disease: “Gastrin” content of endocrine tissues. Proc Staff Meetings of the Mayo Clin. 1961; 36:611–7.
  406. Sutliff VE, Doppman JL, Gibril F, Yu F, Serrano J, Venzon DJ, Jensen RT. Growth of newly diagnosed, untreated metastatic gastrinomas and predictors of growth patterns. J Clin Oncol. 1997; 15:2420–31.
  407. Synder N, Scurry M, Hughes W. Hypergastrinemia in familial multiple endocrine adenomatosis. Ann Intern Med. 1974; 80:321–5.
  408. Taylor TJ, Donlon SS, Bale AE, Smallridge RC, Francis TB, Christensen RS, Burman KD. Treatment of a thyrotropinoma with octreotide-LAR in a patient with Multiple Endocrine Neoplasia 1. Thyroid. 2000; 10:1001–7.
  409. Tebar Masso FJ, Ascaso Gimilio J, Ramirez Munoz L, Lozano Teruel MG. [MEN1 with involvement of 4 endocrine organs]. Med Clin (Barc). 1985; 84:80–1.
  410. Teh BT, Kytola S, Farnebo F, Bergman L, Wong FK, Weber G, Hayward N, Larsson C, Skogseid B, Beckers A, Phelan C, Edwards M, Epstein M, Alford F, Hurley D, Grimmond S, Silins G, Walters M, Stewart C, Cardinal J, Khodaei S, Parente F, Tranebjaerg L, Jorde R, Salmela P. Mutation analysis of the MEN1 gene in multiple endocrine neoplasia type 1, familial acromegaly and familial isolated hyperparathyroidism. J Clin Endocrinol Metab. 1998; 83:2621–6.
  411. Teh BT, McArdle J, Chan SP, Menon J, Hartley L, Pullan P, Ho J, Khir A, Wilkinson S, Larsson C, Cameron D, Shepherd J. Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1. Medicine (Baltimore). 1997; 76:21–9.
  412. Teh BT, Zedenius J, Kytola S, Skogseid B, Trotter J, Choplin H, Twigg S, Farnebo F, Giraud S, Cameron D, Robinson B, Calender A, Larsson C, Salmela P. Thymic carcinoids in multiple endocrine neoplasia type 1. Ann Surg. 1998; 228:99–105.
  413. Tellaroli JC, Charleux H, Semin AM, Parrot B, Fasano JJ, Cain P. Neoplasie endocrine multiple AVEC Association syndrome de Zollinger-Ellison-hyperparathyroidie. Ann Gastroenterol Hepatol. 1984; 20:199–201.
  414. Temperley JM. Zollinger-Ellison syndrome and hyperparathyroidism. Proc R Soc Med. 1971; 64:1004.
  415. Termanini B, Gibril F, Doppman JL, Reynolds JC, Stewart CA, Sutliff VE, Venzon DJ, Jensen RT. Distinguishing small hepatic hemangiomas from vascular liver metastases in patients with gastrinoma: Use of a somatostatin-receptor scintigraphic agent. Radiology. 1997; 202:151–8.
  416. Termanini B, Gibril F, Reynolds JC, Doppman JL, Chen CC, Stewart CA, Sutliff V, Jensen RT. Value of somatostatin receptor scintigraphy: A prospective study in gastrinoma of its effect on clinical management. Gastroenterology. 1997; 112:335–47.
  417. Thakker RV. Multiple endocrine neoplasia type 1. Endocrinol Metab Clin North Am. 2000; 29:541–67.
  418. Theile R, Scharplatz D, Stamm B, Veraguth UP. Zollinger-Ellison syndrome, duodenal carcinoid (gastrinoma), and hyperthyroidism. Virchows Arch (Pathol Anat). 1989; 415:577–8.
  419. Thompson GB, Van Heerden JA, Grant CS, Carney JA, Ilstrup DM. Islet cell carcinomas of the pancreas: a twenty-year experience. Surgery. 1988; 104:1011–7.
  420. Thompson JC, Reeder DD, Villar HV, Fender HR. Natural history and experience with diagnosis and treatment of the Zollinger-Ellison syndrome. Surg Gynecol Obstet. 1975; 140:721–39.
  421. Thompson MH, Sanders DJ, Grund ER. The relationship of the serum gastrin and calcium concentrations in patients with multiple endocrine neoplasia type 1. Br J Surg. 1976; 63:779–83.
  422. Thompson NW. 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. 1998; 243:495–500.
  423. Thompson NW. Management of pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1. Surg Oncol Clin North Am. 1998; 7:881–91.
  424. Thompson NW, Bondeson AG, Bondeson L, Vinik A. The surgical treatment of gastrinoma in MEN I syndrome patients. Surgery. 1989; 106:1081–5.
  425. Thompson NW, Lloyd RV, Nishiyama RH, Vinik AI, Strodel WE, Allo MD, Eckhauser FE, Talpos G, Mervak T. MEN I pancreas: a histological and immunohistochemical study. World J Surg. 1984; 8:561–74.
  426. Thompson NW, Pasieka J, Fukuuchi A. Duodenal gastrinomas, duodenotomy, and duodenal exploration in the surgical management of Zollinger-Ellison syndrome. World J Surg. 1993; 17:455–62.
  427. Thomsen HL, Ersgaard H. Synchronous perforation of the jejunum as presenting feature of Zollinger-Ellison syndrome. Acta Chir Scand. 1985; 151:299–300.
  428. Tiengo A, Fedele D, Marchiori E, Nosadini R, Muggeo M. Suppression and stimulation mechanisms controlling glucagon secretion in a case of islet-cell tumor producing glucagon, insulin, and gastrin. Diabetes. 1976; 25:408.
  429. Tisell LE, Ahlman H. Treatment of the pancreatic disease of multiple endocrine neoplasia type 1 (MEN 1). Acta Oncol. 1989; 28:415–7.
  430. Tisell LE, Ahlman H, Jansson S, Grimelius L. Total pancreatectomy in the MEN-1 syndrome. Br J Surg. 1988; 75:154–7.
  431. Tomassetti P, Migliori M, Caletti GC, Fusaroli P, Corinaldesi R, Gullo L. Treatment of type II gastric carcinoid tumors with somatostatin analogues. N Engl J Med. 2000; 343:551–4.
  432. Tomassetti P, Migliori M, Corinaldesi R, Gullo L. Treatment of gastroenteropancratic neuroendocrine tumours with octreotide LAR. Aliment Pharmacol Ther. 2000; 14:557–60.
  433. Tonelli F, Spini S, Tommasi M, Gabbrielli G, Amorosi A, Brocchi A, Brandi ML. Intraoperative parathormone measurement in patients with multiple endocrine neoplasia type 1 syndrome and hyperparathyroidism. World J Gastroenterol. 2000; 24:556–63.
  434. Trudeau WL, McGuigan JE. Effects of calcium on serum gastrin levels in the Zollinger- Ellison syndrome. N Engl J Med. 1969; 281:862–6.
  435. Trump D, Farren B, Wooding C, Pang JT, Besser GM, Buchanan KD, Edwards CR, Heath DA, Jackson CE, Jansen S, Lips K, Monson JP, O’Halloran D, Sampson J, Shalet SM, Wheeler MH, Zink A, Thakker RV. Clinical studies of multiple endocrine neoplasia type 1 (MEN1). Q J Med. 1996; 89:957–8.
  436. Turbey WJ, Passaro E Jr. Hyperparathyroidism in the Zollinger-Ellison syndrome. Influence of hypercalcemia on clinical course. Arch Surg. 1972; 105:62–6.
  437. Turner JJ, Wren AM, Jackson JE, Thakker RV, Meeran K. Localization of gastrinomas by selective intra-arterial calcium injection. Clin Endocrinol (Oxf). 2002; 57:821–5.
  438. Underdahl LO, Woolner LB, Black BM. Multiple endocrine adenomas: report of 8 cases in which the parathyroids, pituitary and pancreatic islets were involved. J Clin Endocrinol Metab. 1953; 13:20–47.
  439. Underwood LE, Jacobs NM. Familial endocrine adenomatosis. Am J Dis Child. 1963; 106:218–23.
  440. Uriarte C, Olarreaga M, Olaizola A, Irazusta M, Aramburu V, Alvarez Caperochipi FJ. [Acute abdomen caused by jejunal perforation as a clinical presentation of MEN-1 syndrome]. Rev Esp Enferm Dig. 1996; 88:289–91.
  441. Valdes N, de Nanclares GP, Alvarez V, Castano L, Diaz-Cadorniga F, Aller J, Coto E. Multiple endocrine neoplasia type 1 (MEN1): clinical heterogeneity in a large family with a nonsense mutation in the MEN1 gene (Trp471Stop). Clin Endocrinol. 1999; 50:309–13.
  442. Vance JE, Stoll RW, Kitabchi AE, Hollander D, Williams RH. Familial nesidioblastosis as the predominant manifestation of multiple endocrine adenomatosis. Am J Med. 1972; 52:211–27.
  443. Vandeweghe M, Schutyser J, Braxel K, Vermeulen A. A case of multiple endocrine adenomatosis with primary amenorrhoea. Postgrad Med. 1978; 54:618–22.
  444. vanHeerden JA, Smith SL, Miller LJ. Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type 1. Surgery. 1986; 100:971–7.
  445. Vasen HF, Lamers CB, Lips CJ. Screening for the multiple endocrine neoplasia syndrome type I. A study of 11 kindreds in the Netherlands. Arch Intern Med. 1989; 149:2717–22.
  446. Veldhuis JD, Green JE, III, Kovacs E, Worgul TJ, Murray FT, Hammond JM. Prolactin-sescreting pituitary adenomas. Am J Med. 1979; 67:830–7.
  447. Vella MA, Cowie AGA, Gorsuch AN, Watson LCA. Giant gastrinoma in a patient with multiple endocrine adenopathy (type 1). J R Soc Med. 1988; 81:359–60.
  448. Vellar D, Henderson M, Vellar ID, Desmond P. The Zollinger-Ellison syndrome: a review of the St. Vincent’s hospital, Melbourne experience. Aust N Z J Surg. 1985; 55:455–62.
  449. Verges B, Boureille F, Goudet P, Murat A, Beckers A, Sassolas G, Cougard P, Montvernay C, Calender A. Pituitary disease in MEN type 1 (MEN1): data from the France-Belgium MEN1 multicenter study. J Clin Endocrinol Metab. 2002; 87:457–65.
  450. Vezzadini P, Sternini C, Bonora G, Labo G. Gastric acid secretion in Zollinger-Ellison syndrome. Pan Med. 1984; 26:93–6.
  451. Vieto RJ, Hickey RC, Samann NA. Type I multiple endocrine neoplasias. Curr Probl Cancer. 1982; 7:1–25.
  452. Vortmeyer AO, Lubensky IA, Skarulis M, Li G, Moon YW, Park WS, Weil R, Barlow C, Spiegel AM, Marx SJ, Zhuang Z. Multiple endocrine neoplasia type 1: atypical presentation, clinical course, and genetic analysis of multiple tumors. Mod Pathol. 1999; 12:919–24.
  453. Waddell WR, Loughry RW. Gastric acid secretion after pancreaticoduodenectomy. Arch Surg. 1968; 96:574–85.
  454. Wank SA, Doppman JL, Miller DL, Collen MJ, Maton PN, Vinayek R, Slaff JI, Norton JA, Gardner JD, Jensen RT. Prospective study of the ability of computerized axial tomography to localize gastrinomas in patients with Zollinger-Ellison syndrome. Gastroenterology. 1987; 92:905–12.
  455. Way L, Goldman L, Dunphy JE. Zollinger-Ellison syndrome. An analysis of twenty-five cases. Am J Surg. 1968; 116:293–304.
  456. Weber HC, Venzon DJ, Lin JT, Fishbein VA, Orbuch M, Strader DB, Gibril F, Metz DC, Fraker DL, Norton JA, Jensen RT. Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study. Gastroenterology. 1995; 108:1637–49.
  457. Weber JM, Lewis S, Heasley KH. Observations on small bowel pattern associated with the Zollinger- Ellison syndrome. Am J Roentgenol. 1959; 82:973–7.
  458. Weichert RF3, Roth LM, Krementz ET, Hewitt RL, Drapanas T. Carcinoid-islet cell tumors of the duodenum. Report of twenty-one cases. Am J Surg. 1971; 121:195–205.
  459. Weinhausel A, Kaserer K, Vierhapper H, Niederle B, Haas OA, Study Group of Multiple Endocrine Neoplasia Austria. Multiple endocrine neoplasia type 1 (MEN1) in Austria. Wien Klin Wochenschr. 2002; 114:252–7.
  460. Wells SA Jr. Surgery for the Zollinger-Ellison syndrome. N Engl J Med. 1999; 341:689–90.
  461. Weltman MD, Wing JR, Diamond TH, Botha JR, Myburgh JA. Time-dependent changes in serum gastrin measurements after parathyroidectomy. S Afr Med J. 1989; 27:8–9.
  462. Wermer P. Endocrine adenomatosis and peptic ulcer in a large kindred. Am J Med. 1963; 35:205–12.
  463. Wick MR, Scott RE, Li CY, Carney JA. Carcinoid tumor of the thymus: a clinicopathologic report of seven cases with a review of the literature. Mayo Clin Proc. 1980; 55:246–54.
  464. Wiersinga WM, Schellekens AP, Thornton WE, Touber JL, Tijtgat GN. Modulation of gastrin release by acute changes in plasma calcium. Digestion. 1978; 18:290–3.
  465. Wilkinson S, Teh BT, Davey KR, McArdle JP, Young M, Shepherd JJ. Cause of death in multiple endocrine neoplasia type 1. Arch Surg. 1993; 128:683–90.
  466. Wilson SD, Singh RB, Kalkhoff RK, Go VLW. Does hyperparathyroidism cause hypergastrinemia? Surgery. 1976; 80:231–7.
  467. Wolfe JHN, Pinkerton JR, Mackie DB, Gent AE. Pneumopericardium from recurrent peptic ulceration in a patient with multiple endocrine adenomatosis. Br J Clin Pract. 1979; 33:113–5.
  468. Wolfe MM, Jensen RT. Zollinger-Ellison syndrome: Current concepts in diagnosis and management. N Engl J Med. 1987; 317:1200–9.
  469. Wood PJ. The measurement of parathyroid hormone. Ann Clin Biochem. 1992; 29:11–21.
  470. Woodtli W, Hedinger C, Ammann R, Zimmermann K, Sauberli H. Glukagonproduzierende insel-adenomatose des pankreas mit polyendokriner symptomatik. Schweiz Med Wochenschr. 1976; 106:749–54.
  471. Wu WC, Hogan WJ, Whalen GE, Hoke SE, Go VL, Kalkhoff RK. Lower esophageal sphincter responses to enteric hormones in two patients with Zollinger-Ellison syndrome. Am J Dig Dis. 1975; 20:716–20.
  472. Wynick D, Dollery CT, Bloom SR, Polak JM, Lynn JA. Gastrinoma syndrome in multiple endocrine neoplasia. BMJ. 1990; 301:489–90.
  473. Wynick D, Williams SJ, Bloom SR. Symptomatic secondary hormone syndromes in patients with established malignant pancreatic endocrine tumors. N Engl J Med. 1988; 319:605–7.
  474. Yamaguchi K, Kameya T, Abe K. Multiple endocrine neoplasia type 1. Clin Endocrinol Metab. 1980; 9:261–84.
  475. Yazawa K, Kuroda T, Watanabe H, Shimozawa N, Nakata S, Fujimori Y, Koide N, Koike S, Kajikawa S, Adachi W, Amano J. A case of Zollinger-Ellison syndrome produced by gastrinoma in the duodenum accompanied with multiple endocrine neoplasia type 1. Hepatogastroenterology. 1999; 46:257–60.
  476. Yu F, Venzon DJ, Serrano J, Goebel SU, Doppman JL, Gibril F, Jensen RT. Prospective study of the clinical course, prognostic factors and survival in patients with longstanding Zollinger-Ellison syndrome. J Clin Oncol. 1999; 17:615–30.
  477. Zahner J, Borchard F, Schmitz U, Schneider W. [Thymus carcinoid in multiple endocrine neoplasms]. Dtsch Med Wochenschr. 1994; 119:135–40.
  478. Zaniewski M, Jordan PH Jr, Yip B, Thornby JI, Mallette LE. Serum gastrin level is increased by chronic hypercalcemia of parathyroid or nonparathyroid origin. Arch Intern Med. 1986; 146:478–82.
  479. Zeiger MA, Swartz SE, MacGillivray DC, Linnoila I, Shakir M. Thymic carcinoid in association with MEN syndromes. Am Surg. 1992; 58:430–4.
  480. Zhuang ZP, Vortmeyer AO, Pack S, Huang S, Pham TA, Wang C, Park WS, Agarwal SK, Debelenko LV, Kester MB, Guru SC, Manickam P, Olufemi SE, Yu F, Heppner C, Crabtree JS, Skarulis MC, Venzon DI, Emmert-Buck AM, Spiegel AM, Chandrasekharappa SC, Collins FS, Burns AL, Marx SJ, Jensen RT, Liotta LA, Lubensky IA. Somatic mutations of the MEN1 tumor suppressor gene detected in sporadic gastrinomas and insulinomas. Cancer Res. 1997; 57:4682–6.
  481. Ziegler R, Minne H, Raue F. Primary hyperparathyroidism and multiple endocrine neoplasia (MEN). Rev Roum Med Endocrinol. 1982; 20:49–55.
  482. Zollinger RM. Gastrinoma: factors influencing prognosis. Surgery. 1985; 97:49–54.
  483. Zollinger RM, Ellison EC, O’Dorisio TM, Sparks J. Thirty years’ experience with gastrinoma. World J Surg. 1984; 8:427–35.
  484. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. 1955; 142:709–28.
  485. Zubrod CG, Pieper W, Hilbish TF, Smith R, Dutcher T, Wermer P. Acromegaly, jejunal ulcers and hypersecretion of gastric juice: clinical-pathological conference at the National Institutes of Health. Ann Intern Med. 1958; 49:1389–409.
Article Info
Journal
Medicine
Abbr.
Medicine (Baltimore)
ISSN
0025-7974
DOI
10.1097/01.md.0000112297.72510.32
Published
2004-01-00
Pages
43-83
Language
English
Region
United States
NLM ID
2985248R
Subset
IM
Corrections
ErratumIn
-
External Links
PubMed source DOI: 10.1097/01.md.0000112297.72510.32 View journal details
Analysis Services
Analysis Services

Contact

No. 2 Wenbo Road, Zhangqiu District, Jinan, Shandong

Qilu Normal University · Genelibs Bioinformatics Lab

750 Shunhua Rd, Jinan

2F, Bldg F, University Science Park

Tel: 0531-88819269

WeChat Official Account

Follow our WeChat subscription account for real-time updates and the latest in medical and biological research.


Business Email

E-mail: product@genelibs.com

Copyright © 2026.Genelibs Jinan Honghui Biotechnology Co., Ltd. 鲁ICP备20007159号-2