Guidelines Summary

Practice guidelines from the National Comprehensive Cancer Network (NCCN) recommend resection as the primary treatment for locoregional disease. Patients with other life-limiting co-morbidities or high surgical risk should be excluded, particularly when tumors are small and indolent. Additional recommendation include the following^[57]:

Incidental nonfunctional local tumors ≤1 cm - In selected cases, observation alone is an option.
Nonfunctional tumors 1-2 cm in size - Resection of lymph nodes should be considered.
Large (> 2 cm) node-positive nonfunctional tumors - Resection should involve removal of the tumor with negative margins (including adjacent organs) and regional lymph nodes.
Occult gastrinoma - Observation or exploratory surgery are options.
Gastrinomas in the duodenum require duodenotomy and intraoperative ultrasound with local resection or enucleation and periduodenial node dissection.
Gastrinomas in the distal pancreas - Distal pancreatectomy is indicated.
Insulinomas - The primary treatment is enucleation.
Glucagonomas - Treatment is distal pancreatectomy with splenectomy and peripancreatic lymph node resection.

Medication

Aluri V, Dillon JS. Biochemical Testing in Neuroendocrine Tumors. Endocrinol Metab Clin North Am. 2017 Sep. 46 (3):669-677. [QxMD MEDLINE Link].
Wilder RM, Allan FN, Power WH. Carcinoma of the islands of the pancreas: Hyperinsulinism and hypoglycemia. JAMA. 1927. 89:348.
Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. 1955. 142:709.
Verner JV, Morrison AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med. 1958. 25:374.
Ganda OP, Weir GC, Soeldner JS, et al. "Somatostatinoma": a somatostatin-containing tumor of the endocrine pancreas. N Engl J Med. 1977 Apr 28. 296(17):963-7. [QxMD MEDLINE Link].
Larsson LI, Hirsch MA, Holst JJ, et al. Pancreatic somatostatinoma. Clinical features and physiological implications. Lancet. 1977 Mar 26. 1(8013):666-8. [QxMD MEDLINE Link].
Howard JM, Gohara AF, Cardwell RJ. Malignant islet cell tumor of the pancreas associated with high plasma calcitonin and somatostatin levels. Surgery. 1989 Feb. 105(2 Pt 1):227-9. [QxMD MEDLINE Link].
McLeod MK, Vinik AI. Calcitonin immunoreactivity and hypercalcitoninemia in two patients with sporadic, nonfamilial, gastroenteropancreatic neuroendocrine tumors. Surgery. 1992 May. 111(5):484-8. [QxMD MEDLINE Link].
Mao C, Carter P, Schaefer P, et al. Malignant islet cell tumor associated with hypercalcemia. Surgery. 1995 Jan. 117(1):37-40. [QxMD MEDLINE Link].
Meko JB, Norton JA. Endocrine tumors of the pancreas. Curr Opin Gen Surg. 1994. 186-94. [QxMD MEDLINE Link].
Langstein HN, Norton JA, Chiang V, et al. The utility of circulating levels of human pancreatic polypeptide as a marker for islet cell tumors. Surgery. 1990 Dec. 108(6):1109-15; discussion 1115-6. [QxMD MEDLINE Link].
Yeo CJ. Neoplasms of the endocrine pancreas. In: Greenfield LJ, Mulholland MW, Oldham KT, eds. Surgery: Scientific Principles and Practice, 3rd ed. Philadelphia, Pa: Lippincott. 2001: 899-913.
Pearse AG. Common cytochemical and ultrastructural characteristics of cells producing polypeptide hormones (the APUD series) and their relevance to thyroid and ultimobranchial C cells and calcitonin. Proc R Soc Lond B Biol Sci. 1968 May 14. 170(18):71-80. [QxMD MEDLINE Link].
Andrew A, Kramer B, Rawdon BB. The origin of gut and pancreatic neuroendocrine (APUD) cells--the last word?. J Pathol. 1998 Oct. 186(2):117-8. [QxMD MEDLINE Link].
Kloppel G, Heitz PU. Pancreatic endocrine tumors. Pathol Res Pract. 1988 Apr. 183(2):155-68. [QxMD MEDLINE Link].
Heitz PU, Kasper M, Polak JM, Kloppel G. Pancreatic endocrine tumors. Hum Pathol. 1982 Mar. 13(3):263-71. [QxMD MEDLINE Link].
Metz DC. Diagnosis and treatment of pancreatic neuroendocrine tumors. Semin Gastrointest Dis. 1995 Apr. 6(2):67-78. [QxMD MEDLINE Link].
Norton JA, Levin B, Jensen RT. Cancer of the endocrine system. In: DeVita VT, Hellman S, Rosenberg SA, eds. Cancer: Principles and Practice of Oncology. 4th ed. Philadelphia, Pa:. Lippincott-Raven. 1993:1333-435.
Helmrath MA. Miscellaneous endocrine disorders. In: Berry SM, Bass RC, Heaton KM, eds. The Mont Reid Surgical Handbook. 4th ed. St Louis, Mo:. Mosby. 1997: 355-67.
Buchanan KD, Johnston CF, O''Hare MM, et al. Neuroendocrine tumors. A European view. Am J Med. 1986 Dec 22. 81(6B):14-22. [QxMD MEDLINE Link].
Eriksson B, Oberg K, Skogseid B. Neuroendocrine pancreatic tumors. Clinical findings in a prospective study of 84 patients. Acta Oncol. 1989. 28(3):373-7. [QxMD MEDLINE Link].
Weil C. Gastroenteropancreatic endocrine tumors. Klin Wochenschr. 1985 May 15. 63(10):433-59. [QxMD MEDLINE Link].
Jensen RT, Norton JA. Endocrine tumors of the pancreas. In: Feldman M, Scharschmidt BF, Sleisenger M, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 6th ed. Philadelphia, Pa:. WB Saunders. 1998: 871-94.
Eriksson B, Oberg K. PPomas and nonfunctioning endocrine pancreatic tumors: clinical presentation, diagnosis, and advances in management. In: Mignon M, Jensen RT, eds. Endocrine Tumors of the Pancreas. Frontiers of Gastrointestinal Research. Vol 23. Basel, Switzerland:. Karger. 1995: 208.
Phan GQ, Yeo CJ, Hruban RH, et al. Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: review of 125 patients. J Gastrointest Surg. 1998 Sep-Oct. 2(5):472-82. [QxMD MEDLINE Link].
Welbourne RB, Wood SM, Polak JM. Pancreatic endocrine tumors. In: Bloom SR, Polak JM, eds. Gut Hormones. 2nd ed. New York, NY: Churchill-Livingstone. 1981: 547-54.
Menegaux F, Schmitt G, Mercadier M, Chigot JP. Pancreatic insulinomas. Am J Surg. 1993 Feb. 165(2):243-8. [QxMD MEDLINE Link].
Thompson GB, Service FJ, van Heerden JA, et al. Reoperative insulinomas, 1927 to 1992: an institutional experience. Surgery. 1993 Dec. 114(6):1196-204; discussion 1205-6. [QxMD MEDLINE Link].
Metz DC, Jensen RT. Endocrine tumors of the pancreas. In: Haubrich WB, Berk F, Schaffner JE, eds. Bockus Gastroenterology. 5th ed. Philadelphia, Pa:. WB Saunders. 1994: 3002-34.
Whipple AO, Frantz VK. Adenoma of islet cells with hyperinsulinism: a review. Ann Surg. 1935. 101:1299.
Fajans SS, Vinik AI. Insulin-producing islet cell tumors. Endocrinol Metab Clin North Am. 1989 Mar. 18(1):45-74. [QxMD MEDLINE Link].
Service FJ. Hypoglycemic disorders. N Engl J Med. 1995 Apr 27. 332(17):1144-52. [QxMD MEDLINE Link].
Wolfe MM, Jensen RT. Zollinger-Ellison syndrome. Current concepts in diagnosis and management. N Engl J Med. 1987 Nov 5. 317(19):1200-9. [QxMD MEDLINE Link].
Way L, Goldman L, Dunphy JE. Zollinger-Ellison syndrome. An analysis of twenty-five cases. Am J Surg. 1968 Aug. 116(2):293-304. [QxMD MEDLINE Link].
Orloff SL, Debas HT. Advances in the management of patients with Zollinger-Ellison syndrome. Surg Clin North Am. 1995 Jun. 75(3):511-24. [QxMD MEDLINE Link].
Miller LS, Vinayek R, Frucht H, et al. Reflux esophagitis in patients with Zollinger-Ellison syndrome. Gastroenterology. 1990 Feb. 98(2):341-6. [QxMD MEDLINE Link].
Bieligk S, Jaffe BM. Islet cell tumors of the pancreas. Surg Clin North Am. 1995 Oct. 75(5):1025-40. [QxMD MEDLINE Link].
Stabile BE, Passaro E Jr. Recurrent peptic ulcer. Gastroenterology. 1976 Jan. 70(1):124-35. [QxMD MEDLINE Link].
Vinik AI, Thompson NW, Averbuch SD. Neoplasms of the gastroenteropancreatic endocrine system. In: Holland JF, Frei E, Bast RC, eds. Cancer Medicine. Philadelphia, Pa: Lea & Febiger. 1993: 1180-209.
Shimoda SS, Saunders DR, Rubin CE. The Zollinger-Ellison syndrome with steatorrhea. II. The mechanism of fat and vitamin B 12 malabsorption. Gastroenterology. 1968 Dec. 55(6):705-23. [QxMD MEDLINE Link].
Wynick D, Williams SJ, Bloom SR. Symptomatic secondary hormone syndromes in patients with established malignant pancreatic endocrine tumors. N Engl J Med. 1988 Sep 8. 319(10):605-7. [QxMD MEDLINE Link].
Chiang HC, O''Dorisio TM, Huang SC, et al. Multiple hormone elevations in Zollinger-Ellison syndrome. Prospective study of clinical significance and of the development of a second symptomatic pancreatic endocrine tumor syndrome. Gastroenterology. 1990 Dec. 99(6):1565-75. [QxMD MEDLINE Link].
O''Dorisio TM, Mekhjian HS, Gaginella TS. Medical therapy of VIPomas. Endocrinol Metab Clin North Am. 1989 Jun. 18(2):545-56. [QxMD MEDLINE Link].
Higgins GA, Recant L, Fischman AB. The glucagonoma syndrome: surgically curable diabetes. Am J Surg. 1979 Jan. 137(1):142-8. [QxMD MEDLINE Link].
Wilkinson DS. Necrolytic migratory erythema with carcinoma of the pancreas. Trans St Johns Hosp Dermatol Soc. 1973. 59(2):244-50. [QxMD MEDLINE Link].
Stacpoole PW. The glucagonoma syndrome: clinical features, diagnosis, and treatment. Endocr Rev. 1981 Summer. 2(3):347-61. [QxMD MEDLINE Link].
Leichter SB. Clinical and metabolic aspects of glucagonoma. Medicine (Baltimore). 1980 Mar. 59(2):100-13. [QxMD MEDLINE Link].
Guillausseau PJ, Guillausseau C, Villet R, et al. [Glucagonomas. Clinical, biological, anatomopathological and therapeutic aspects (general review of 130 cases]. Gastroenterol Clin Biol. 1982 Dec. 6(12):1029-41. [QxMD MEDLINE Link].
Vinik AI, Strodel WE, Eckhauser FE, et al. Somatostatinomas, PPomas, neurotensinomas. Semin Oncol. 1987 Sep. 14(3):263-81. [QxMD MEDLINE Link].
Brodish RJ, Kuvshinoff BW, McFadden DW, Fink AS. Somatostatin inhibits cholecystokinin-induced pancreatic protein secretion via cholinergic pathways. Pancreas. 1995 May. 10(4):401-6. [QxMD MEDLINE Link].
Boden G, Shimoyama R. Somatostatinoma. In: Cohen S, Soloway RD, eds. Hormone-Producing Tumors of the Gastrointestinal Tract. New York, NY: Churchill Livingstone. 1985: 85.
Long PP, Hruban RH, Lo R, et al. Chromosome analysis of nine endocrine neoplasms of the pancreas. Cancer Genet Cytogenet. 1994 Oct. 77(1):55-9. [QxMD MEDLINE Link].
Zeiger MA, Norton JA. Gs alpha--identification of a gene highly expressed by insulinoma and other endocrine tumors. Surgery. 1993 Aug. 114(2):458-62; discussion 462-3. [QxMD MEDLINE Link].
Eubanks PJ, Sawicki MP, Samara GJ, et al. Pancreatic endocrine tumors with loss of heterozygosity at the multiple endocrine neoplasia type I locus. Am J Surg. 1997 Jun. 173(6):518-20. [QxMD MEDLINE Link].
Miller JA, Norton JA. Multiple endocrine neoplasia. Cancer Treat Res. 1997. 90:213-25. [QxMD MEDLINE Link].
Nakeeb A, Lillemoe KD, Yeo CJ. Neoplasms of the exocrine pancreas. In: Greenfield LJ, Mulholland MW, Oldham KT, eds. Surgery: Scientific Principles and Practice. 3rd ed. Philadelphia, Pa:. Lippincott-Raven. 2001:885-99.
[Guideline] NCCN Clinical Practice Guidelines in Oncology. Neuroendocrine and Adrenal Tumors. National Comprehensive Cancer Network. Available at http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Version 1.2021 — April 14, 2021; Accessed: June 11. 2021.
Ekeblad S, Skogseid B, Dunder K, Oberg K, Eriksson B. Prognostic factors and survival in 324 patients with pancreatic endocrine tumor treated at a single institution. Clin Cancer Res. 2008 Dec 1. 14(23):7798-803. [QxMD MEDLINE Link]. [Full Text].
Warner RR, Curran T, Shafir MK, et al. Serum and ascites chromogranin-A in patients with metastatic neuroendocrine tumors. Pancreas. 2011 May. 40(4):622-6. [QxMD MEDLINE Link].
Mekhjian HS, O''Dorisio TM. VIPoma syndrome. Semin Oncol. 1987 Sep. 14(3):282-91. [QxMD MEDLINE Link].
Krejs GJ. VIPoma syndrome. Am J Med. 1987 May 29. 82(5B):37-48. [QxMD MEDLINE Link].
Krejs GJ, Orci L, Conlon JM, et al. Somatostatinoma syndrome. Biochemical, morphologic and clinical features. N Engl J Med. 1979 Aug 9. 301(6):285-92. [QxMD MEDLINE Link].
Naswa N, Sharma P, Kumar A, et al. Gallium-68-DOTA-NOC PET/CT of patients with gastroenteropancreatic neuroendocrine tumors: a prospective single-center study. AJR Am J Roentgenol. 2011 Nov. 197(5):1221-8. [QxMD MEDLINE Link].
Pelley RJ, Bukowski RM. Recent advances in diagnosis and therapy of neuroendocrine tumors of the gastrointestinal tract. Curr Opin Oncol. 1997 Jan. 9(1):68-74. [QxMD MEDLINE Link].
Moore NR, Rogers CE, Britton BJ. Magnetic resonance imaging of endocrine tumours of the pancreas. Br J Radiol. 1995 Apr. 68(808):341-7. [QxMD MEDLINE Link].
Berger JF, Laissy JP, Limot O, et al. Differentiation between multiple liver hemangiomas and liver metastases of gastrinomas: value of enhanced MRI. J Comput Assist Tomogr. 1996 May-Jun. 20(3):349-55. [QxMD MEDLINE Link].
Gibril F, Reynolds JC, Doppman JL, et al. 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 Jul 1. 125(1):26-34. [QxMD MEDLINE Link].
Mitchell DG. Diagnosis and staging of pancreatic tumors by magnetic resonance imaging. Neoplasms of the Digestive System. 1998. Lippincott Raven:
Krausz Y, Bar-Ziv J, de Jong RB, et al. Somatostatin-receptor scintigraphy in the management of gastroenteropancreatic tumors. Am J Gastroenterol. 1998 Jan. 93(1):66-70. [QxMD MEDLINE Link].
Frilling A, Malago M, Martin H, Broelsch CE. Use of somatostatin receptor scintigraphy to image extrahepatic metastases of neuroendocrine tumors. Surgery. 1998 Dec. 124(6):1000-4. [QxMD MEDLINE Link].
Glover JR, Shorvon PJ, Lees WR. Endoscopic ultrasound for localisation of islet cell tumours. Gut. 1992 Jan. 33(1):108-10. [QxMD MEDLINE Link].
Rosch T, Lightdale CJ, Botet JF, et al. Localization of pancreatic endocrine tumors by endoscopic ultrasonography. N Engl J Med. 1992 Jun 25. 326(26):1721-6. [QxMD MEDLINE Link].
Gooding GA. Adrenal, pancreatic, and scrotal ultrasound in endocrine disease. Radiol Clin North Am. 1993 Sep. 31(5):1069-83. [QxMD MEDLINE Link].
Norton JA, Sigel B, Baker AR, et al. Localization of an occult insulinoma by intraoperative ultrasonography. Surgery. 1985 Mar. 97(3):381-4. [QxMD MEDLINE Link].
Grant CS, van Heerden J, Charboneau JW, et al. Insulinoma. The value of intraoperative ultrasonography. Arch Surg. 1988 Jul. 123(7):843-8. [QxMD MEDLINE Link].
Imamura M, Takahashi K, Adachi H, et al. Usefulness of selective arterial secretin injection test for localization of gastrinoma in the Zollinger-Ellison syndrome. Ann Surg. 1987 Mar. 205(3):230-9. [QxMD MEDLINE Link].
Thom AK, Norton JA, Doppman JL, et al. Prospective study of the use of intraarterial secretin injection and portal venous sampling to localize duodenal gastrinomas. Surgery. 1992 Dec. 112(6):1002-8; discussion 1008-9. [QxMD MEDLINE Link].
Pereira PL, Roche AJ, Maier GW, et al. Insulinoma and islet cell hyperplasia: value of the calcium intraarterial stimulation test when findings of other preoperative studies are negative. Radiology. 1998 Mar. 206(3):703-9. [QxMD MEDLINE Link].
Vinik AI, Delbridge L, Moattari R, et al. Transhepatic portal vein catheterization for localization of insulinomas: a ten-year experience. Surgery. 1991 Jan. 109(1):1-11; discussion 111. [QxMD MEDLINE Link].
Nasir A, Helm J, Turner L, et al. RUNX1T1: a novel predictor of liver metastasis in primary pancreatic endocrine neoplasms. Pancreas. 2011 May. 40(4):627-33. [QxMD MEDLINE Link].
Somogyi L, Mishra G. Diagnosis and staging of islet cell tumors of the pancreas. Curr Gastroenterol Rep. 2000 Apr. 2(2):159-64. [QxMD MEDLINE Link].
Macdonald JS, Haller D, McDougall IR. Endocrine system: pancreatic islet cell tumors. In: Abeloff MD, Armitage JO, Lichter AS, eds. Clinical Oncology. 2nd ed. New York, NY:. Churchill-Livingstone. 2000: 1384-97.
Modlin IM, Kidd M, Drozdov I, Siddique ZL, Gustafsson BI. Pharmacotherapy of neuroendocrine cancers. Expert Opin Pharmacother. 2008 Oct. 9(15):2617-26. [QxMD MEDLINE Link].
Broder MS, Beenhouwer D, Strosberg JR, Neary MP, Cherepanov D. Gastrointestinal neuroendocrine tumors treated with high dose octreotide-LAR: a systematic literature review. World J Gastroenterol. 2015 Feb 14. 21 (6):1945-55. [QxMD MEDLINE Link]. [Full Text].
Al-Efraij K, Aljama MA, Kennecke HF. Association of dose escalation of octreotide long-acting release on clinical symptoms and tumor markers and response among patients with neuroendocrine tumors. Cancer Med. 2015 Jun. 4 (6):864-70. [QxMD MEDLINE Link]. [Full Text].
Vinik AI, Moattari AR. Treatment of endocrine tumors of the pancreas. Endocrinol Metab Clin North Am. 1989 Jun. 18(2):483-518. [QxMD MEDLINE Link].
Strosberg J, El-Haddad G, Wolin E, Hendifar A, Yao J, Chasen B, et al. Phase 3 Trial of ¹⁷⁷Lu-Dotatate for Midgut Neuroendocrine Tumors. N Engl J Med. 2017 Jan 12. 376 (2):125-135. [QxMD MEDLINE Link]. [Full Text].
Cherk MH, Kong G, Hicks RJ, Hofman MS. Changes in biodistribution on ⁶⁸Ga-DOTA-Octreotate PET/CT after long acting somatostatin analogue therapy in neuroendocrine tumour patients may result in pseudoprogression. Cancer Imaging. 2018 Jan 24. 18 (1):3. [QxMD MEDLINE Link]. [Full Text].
Yao JC, Shah MH, Ito T, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011 Feb 10. 364(6):514-23. [QxMD MEDLINE Link].
Raymond E, Dahan L, Raoul JL, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011 Feb 10. 364(6):501-13. [QxMD MEDLINE Link].
Anthony L, Vinik AI. Evaluating the characteristics and the management of patients with neuroendocrine tumors receiving octreotide LAR during a 6-year period. Pancreas. 2011 Oct. 40(7):987-94. [QxMD MEDLINE Link].
Hobday TJ, Qin R, Reidy-Lagunes D, Moore MJ, Strosberg J, Kaubisch A, et al. Multicenter Phase II Trial of Temsirolimus and Bevacizumab in Pancreatic Neuroendocrine Tumors. J Clin Oncol. 2014 Dec 8. [QxMD MEDLINE Link].
Ducreux M, Dahan L, Smith D, O'Toole D, Lepère C, Dromain C, et al. Bevacizumab combined with 5-FU/streptozocin in patients with progressive metastatic well-differentiated pancreatic endocrine tumours (BETTER trial) - A phase II non-randomised trial. Eur J Cancer. 2014 Dec. 50(18):3098-106. [QxMD MEDLINE Link].
Boden G. Glucagonomas and insulinomas. Gastroenterol Clin North Am. 1989 Dec. 18(4):831-45. [QxMD MEDLINE Link].
Moertel CG, Johnson CM, McKusick MA, et al. The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann Intern Med. 1994 Feb 15. 120(4):302-9. [QxMD MEDLINE Link].
Bilchik AJ, Sarantou T, Foshag LJ, et al. Cryosurgical palliation of metastatic neuroendocrine tumors resistant to conventional therapy. Surgery. 1997 Dec. 122(6):1040-7; discussion 1047-8. [QxMD MEDLINE Link].
Mazzaglia PJ, Berber E, Milas M, Siperstein AE. Laparoscopic radiofrequency ablation of neuroendocrine liver metastases: a 10-year experience evaluating predictors of survival. Surgery. 2007 Jul. 142(1):10-9. [QxMD MEDLINE Link].
Lillemoe KD, Kaushal S, Cameron JL, et al. Distal pancreatectomy: indications and outcomes in 235 patients. Ann Surg. 1999 May. 229(5):693-8; discussion 698-700. [QxMD MEDLINE Link].
Phan GQ, Yeo CJ, Cameron JL, et al. Pancreaticoduodenectomy for selected periampullary neuroendocrine tumors: fifty patients. Surgery. 1997 Dec. 122(6):989-96; discussion, 996-7. [QxMD MEDLINE Link].
Udelsman R, Yeo CJ, Hruban RH, et al. Pancreaticoduodenectomy for selected pancreatic endocrine tumors. Surg Gynecol Obstet. 1993 Sep. 177(3):269-78. [QxMD MEDLINE Link].
Sugg SL, Norton JA, Fraker DL, et al. A prospective study of intraoperative methods to diagnose and resect duodenal gastrinomas. Ann Surg. 1993 Aug. 218(2):138-44. [QxMD MEDLINE Link].
Farley DR, van Heerden JA, Grant CS, Thompson GB. Extrapancreatic gastrinomas. Surgical experience. Arch Surg. 1994 May. 129(5):506-11; discussion 511-2. [QxMD MEDLINE Link].
Abood GJ, Go A, Malhotra D, Shoup M. The surgical and systemic management of neuroendocrine tumors of the pancreas. Surg Clin North Am. 2009 Feb. 89(1):249-66, x. [QxMD MEDLINE Link].
Mazzaglia PJ, Berber E, Milas M, Siperstein AE. Laparoscopic radiofrequency ablation of neuroendocrine liver metastases: a 10-year experience evaluating predictors of survival. Surgery. 2007 Jul. 142(1):10-9. [QxMD MEDLINE Link].
Gibril F, Doppman JL, Jensen RT. Recent advances in the treatment of metastatic pancreatic endocrine tumors. Semin Gastrointest Dis. 1995 Apr. 6(2):114-21. [QxMD MEDLINE Link].
Keutgen XM, Nilubol N, Glanville J, Sadowski SM, Liewehr DJ, Venzon DJ, et al. Resection of primary tumor site is associated with prolonged survival in metastatic nonfunctioning pancreatic neuroendocrine tumors. Surgery. 2016 Jan. 159 (1):311-8. [QxMD MEDLINE Link]. [Full Text].
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 Apr. 75(4):597-609. [QxMD MEDLINE Link].
Moertel CG, Hanley JA, Johnson LA. Streptozocin alone compared with streptozocin plus fluorouracil in the treatment of advanced islet-cell carcinoma. N Engl J Med. 1980 Nov 20. 303(21):1189-94. [QxMD MEDLINE Link].
Moertel CG, Lefkopoulo M, Lipsitz S, et al. Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med. 1992 Feb 20. 326(8):519-23. [QxMD MEDLINE Link].
Fjallskog ML, Janson ET, Falkmer UG, Vatn MH, Oberg KE, Eriksson BK. Treatment with combined streptozotocin and liposomal doxorubicin in metastatic endocrine pancreatic tumors. Neuroendocrinology. 2008. 88(1):53-8. [QxMD MEDLINE Link].
Rivera E, Ajani JA. Doxorubicin, streptozocin, and 5-fluorouracil chemotherapy for patients with metastatic islet-cell carcinoma. Am J Clin Oncol. 1998 Feb. 21(1):36-8. [QxMD MEDLINE Link].
Arvold ND, Willett CG, Fernandez-Del Castillo C, Ryan DP, Ferrone CR, Clark JW, et al. Pancreatic Neuroendocrine Tumors With Involved Surgical Margins: Prognostic Factors and the Role of Adjuvant Radiotherapy. Int J Radiat Oncol Biol Phys. 2012 Mar 11. [QxMD MEDLINE Link].
Rothmund M, Angelini L, Brunt LM, et al. Surgery for benign insulinoma: an international review. World J Surg. 1990 May-Jun. 14(3):393-8; discussion 398-9. [QxMD MEDLINE Link].
McGuigan JE. Zollinger-Ellison syndrome and other hypersecretory states. In: Feldman M, Scharschmidt BF, Sleisenger M, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 6th ed. Philadelphia, Pa:. WB Saunders. 1998:679-95.
Stabile BE, Passaro E Jr. Benign and malignant gastrinoma. Am J Surg. 1985 Jan. 149(1):144-50. [QxMD MEDLINE Link].
Fraker DL, Norton JA, Alexander HR, et al. Surgery in Zollinger-Ellison syndrome alters the natural history of gastrinoma. Ann Surg. 1994 Sep. 220(3):320-8; discussion 328-30. [QxMD MEDLINE Link].
Weber HC, Venzon DJ, Lin JT, et al. Determinants of metastatic rate and survival in patients with Zollinger- Ellison syndrome: a prospective long-term study. Gastroenterology. 1995 Jun. 108(6):1637-49. [QxMD MEDLINE Link].
Jensen RT, Fraker DL. Zollinger-Ellison syndrome. Advances in treatment of gastric hypersecretion and the gastrinoma. JAMA. 1994 May 11. 271(18):1429-35. [QxMD MEDLINE Link].
Holst JJ. Glucagon-producing tumors. In: Cohen S, Soloway RD, eds. Hormone-Producing Tumors of the Gastrointestinal Tract. New York, NY: Churchill-Livingstone. 1985: 57.
Verner JV, Morrison AB. Endocrine pancreatic islet disease with diarrhea. Report of a case due to diffuse hyperplasia of nonbeta islet tissue with a review of 54 additional cases. Arch Intern Med. 1974 Mar. 133(3):492-9. [QxMD MEDLINE Link].
Long RG, Bryant MG, Mitchell SJ, et al. Clinicopathological study of pancreatic and ganglioneuroblastoma tumours secreting vasoactive intestinal polypeptide (vipomas). Br Med J (Clin Res Ed). 1981 May 30. 282(6278):1767-71. [QxMD MEDLINE Link].
Kent RB 3rd, van Heerden JA, Weiland LH. Nonfunctioning islet cell tumors. Ann Surg. 1981 Feb. 193(2):185-90. [QxMD MEDLINE Link].
Tomassetti P, Campana D, Piscitelli L. Endocrine pancreatic tumors: factors correlated with survival. Ann Oncol. 2005 Nov. 16(11):1806-10.
Fraker DL, Alexander HR. The surgical approach to endocrine tumors of the pancreas. Semin Gastrointest Dis. 1995 Apr. 6(2):102-13. [QxMD MEDLINE Link].
Fjallskog ML, Sundin A, Westlin JE. Treatment of malignant endocrine pancreatic tumors with a combination of alpha-interferon and somatostatin analogs. Med Oncol. 2002. 19(1):35-42.

Media Gallery

Neoplasms of the endocrine pancreas. CT scan image with oral and intravenous contrast in a patient with biochemical evidence of insulinoma. The 3-cm contrast-enhancing neoplasm (arrow) is seen in the tail of the pancreas (P) posterior to the stomach (S) (Yeo, 1993).
Celiac axis angiography illustrating neoplasms of the endocrine pancreas. Contrast is seen opacifying the common hepatic artery (CHA) and splenic artery (SA). The superior pancreatic artery (arrow) is seen as an early U-shaped branch of the splenic artery.
Highly selective distal angiography illustrating neoplasms of the endocrine pancreas. With the arterial catheter now advanced into the superior pancreatic artery, the contrast blush of this vascular tumor is easily seen (arrows).
Neoplasms of the endocrine pancreas. Intravenous and oral contrast-enhanced CT scan image in a patient with chronic diarrhea and elevated levels of serum vasoactive intestinal peptide. In the venous phase of this scan, the splenic vein (SV) is clearly seen draining the 5-cm tumor (T) located anteromedial to the spleen (S) in the tail of the pancreas.
Neoplasms of the endocrine pancreas. Schematic diagram of provocative angiography. Access to the central venous and arterial systems is obtained through cannulation of a femoral vein and a femoral artery. In the selective arterial secretin stimulation test, secretin is injected selectively into the splenic, gastroduodenal (a branch of the common hepatic), and inferior pancreaticoduodenal (a branch of the superior mesenteric) arteries with concomitant and subsequent hepatic venous sampling for gastrin. Based on the level of gastrin in each hepatic venous sample, the location of the gastrinoma is arterially mapped. An analogous method can be used in the selective arterial calcium stimulation test to determine the location of occult insulinomas that respond to calcium stimulation by secreting insulin.
Neoplasms of the endocrine pancreas. Graphic depiction of the results of a selective arterial secretin stimulation test in a patient with an occult gastrinoma. The gastrin gradient (the rise in hepatic vein gastrin concentration divided by the basal value) is plotted over time. An increase in gastrin gradient from 0 to 2 thus represents a 200% rise compared to the basal level. A significant rise in hepatic vein gastrin concentration is observed both after the injection of secretin into the superior mesenteric artery (SMA) and after secretin injection into the gastroduodenal artery (GDA), but no such increase occurs following secretin injection into the splenic artery (SPL). This patient's neoplasm is thus localized to the head of the pancreas or the duodenum
Neoplasms of the endocrine pancreas. Octreotide scan (anterior view) in a patient with a pancreatic endocrine tumor. The large pancreatic-tail neoplasm is seen retaining tracer in the patient's left upper quadrant. Several tracer-enhancing hepatic metastases are seen in the patient's right upper quadrant and epigastrium. Tracer is also seen in the bladder following renal excretion (round density in the hypogastrium) (Yeo, 2001).
Neoplasms of the endocrine pancreas. CT scan with oral and intravenous contrast in a patient with a glucagon-secreting neoplasm. This 10-cm contrast-enhancing tumor (T) is seen obliterating the normal appearance of the tail of the pancreas (Yeo, 2001).
Neoplasms of the endocrine pancreas. Endoscopic ultrasonography in a patient with an insulinoma. The hypoechoic neoplasm (arrows) is seen in the body of the pancreas anterior to the splenic vein (SV) (Rosch, 1992).

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