Unusual neuroendocrine tumors of the pancreas and duodenum as well as nonfunctional tumors are more typically malignant than either gastrinoma or insulinoma. Despite this fact, patients with these tumors may have a better prognosis than those with other intra-abdominal solid malignancies. Therefore, if the entire extent of tumor can be resected and the patient is otherwise healthy, major operative procedures (e.g., a Whipple pancreaticoduodenectomy, liver lobectomy, subtotal pancreatectomy-splenectomy) may be indicated.83 85 Furthermore, in patients with some rare tumors associated with severe symptoms that are poorly controlled medically, such as somatostatinoma, glucagonoma, PTHrP-releasing tumor, and corticotropin-secreting tumor, major surgery to debulk tumor or to remove end organs (adrenal in patients with corticotropin-secreting tumor) may be necessary. Therefore, aggressive surgery has an important role in the therapy of these patients and is the only potentially curative treatment.


1. Buchanan KD, Johnston CF, O'Hare MMT, et al. Neuroendocrine tumors: A European view. Am J Med 1986;81(Suppl 68):14.

2. Erickson B, Oberg K, Skogseid B. Neuroendocrine pancreatic tumors. Acta Oncol 1989;28:433.

3. Klöppel G, Heitz PU. Pancreatic endocrine tumors. Pathol Res Pract 1988:183:155.

4. Creutzfeldt W, Arnold R, Creutzfeldt C, Track NS. Pathomorphological, biochemical and diagnostic aspects of gastrinomas (Zollinger-Ellison syndrome). Hum Pathol 1975:6:47.

5. Heitz PU, Kasper M, Polak JM, Klöppel G. Pancreatic endocrine tumors: Immunocytochemical analysis of 125 tumors. Hum Pathol 1982;13:263.

6. Jensen RT, Norton JA. Endocrine tumors of the pancreas in gastrointestinal disease. In: Sleisenger MH, Fordtran JS (eds), Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Philadelphia, WB Saunders, 1993, p 1695.

7. Pareja-Megia MJ, Rios-Martin JJ, Garcia-Escudero A, Gonzalez-Campora R. Papillary and cystic insulinoma of the pancreas. Histopathology 2002;40:483.

8. Bolande RP. The neurocrestopathies: A unifying concept of disease arising from neural crest maldevelopment. Hum Pathol 1974:5:409.

9. Pearse AGE, Tabor TT. Embryology of the diffuse neuroendocrine system and its relationship to the common peptides. Fed Proc 1979:38:2288.

10. Pearse HGE. The APUD concept and hormone production. Clin Endocrinol Metab 1980;9:211.

11. Lloyd RV, Mervak T, Schidt K, et al. Immunohistochemical detection of chromogranin and neurospecific enolase in pancreatic endocrine tumors. Am J Surg Pathol 1984;8:607.

12. 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. Philadelphia, JB Lippincott, 1993, p 1333.

13. Bartsch D, Hahn SA, Danichevski KD, et al. Mutations of the DPC4/ Smad4 gene in endocrine pancreatic tumors. Oncogene 1999,18:2367.

14. Friedman EM, Larsson C, Amorosi A, et al. Multiple endocrine neoplasia type 1 pathology, pathophysiology, molecular genetics and differential diagnosis. In: Bilezikian JP, Levine MA, Marcus R (eds), The Parathyroids. New York, Raven Press, 1994, p 647.

15. Burke AO, Sobin LH, Federspiel BH, et al. Carcinoid tumors of the duodenum. Arch Pathol Lab Med 1990; 114:700.

16. Burke AP, Sobin LH, Shekitka KM, et al. Somatostatin-producing duodenal carcinoids in patients with von Recklinghausen's neurofibromatosis: A predilection for black patients. Cancer 1990;65:1591.

17. Chagnon JP, Barge J, Hienin D, Blanc D. Recklinghausen's disease with digestive localizations associated with gastric acid hypersecretion suggesting Zollinger-Ellison syndrome. Gastroenterol Clin Biol 1985;9:65.

18. Binkovitz LA, Johnson CD, Stephens DH. Islet cell tumors in Hippel-Lindau disease, increased prevalence and relationship to multiple endocrine neoplasia. AJR Am J Roentgenol 1990:155:501.

19. Davoren PM, Epstein MT. Insulinoma complicating tuberous sclerosis. J Neurol Neurosurg Psychiatry 1992;55:1509.

20. Oberg K, Skogseid B, Ericksson N. Multiple endocrine neoplasia type I. Acta Oncol 1989:28:383.

21. Bale AE, Norton JA, Wong EL, et al. Allelic loss on chromosome 11 in hereditary and sporadic tumors related to familial multiple endocrine neoplasia type 1. Cancer Res 1991:51:1154.

22. Knudson AG. Mutation and cancer: Statistical study of retinoblastoma. Proc Natl Acad Sei USA 1971;68:820.

23. Brandi Ml, Aurbach GD, Fitzpatrick LA, et al. Parathyroid mitogenic activity in plasma from patients with familial multiple endocrine neoplasia type I. N Engl J Med 1986:314:1287.

24. Zimmering MB, Brandi MI, de Grange DA, et al. Circulating fibroblast growth factor-like substance in familial multiple endocrine neoplasia-type I. J Clin Endocrinol Metab 1990;70:149.

25. Sheppard BC, Norton JA, Doppman JL, et al. Management of islet cell tumors in patients with multiple endocrine neoplasia: A prospective study. Surgery 1989;106:1108.

26. Thompson NW, Lloyd RV, Nishiyama RH, et al. MEN-1 pancreas: A histological and immunohistochemical study. World J Surg 1984; 8:561.

27. Klöppel G, Willemar S, Stamm B, et al. Pancreatic lesions and hormonal profile in pancreatic tumors in multiple endocrine neoplasia type I. Cancer 1986;57:1820.

28. Legaspi A, Brennan MF. Management of islet cell carcinoma. Surgery 1988;104:I018.

29. Harris G, Tio F, Cruz A. Somatostatinoma: A case report and a review of the literature. J Surg Oncol I987;36:8.

30. Higgins GA. The glucagonoma syndrome: Surgically curable diabetes. Am J Surg 1979; 137:142.

31. Chastain MA. The glucagonoma syndrome: A review of its features and discussion of new perspectives. Am J Med 2001;321:306.

32. Mozell E, Stenzel P, Woltering EA, et al. Functional endocrine tumors of the pancreas: Clinical presentation, diagnosis, and treatment. Curr Probl Surg 1990;27:303.

33. Wermers RA, Fatourechi V, Wynne AG, et al. The glucagonoma syndrome: Clinical and pathological features in 21 patients. Medicine (Baltimore) 1996:75:53.

34. Norton JA. Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg 1994;31:77.

35. Alexander EK, Robinson M, Staniec M, Dluhy R. Peripheral amino acid and fatty acid infusion for the treatment of necrolytic migratory erythema in the glucagonoma syndrome. Clin Endocrinol (Oxf) 2002;57:827.

36. Maton PN. The use of the long-acting somatostatin analogue, octreotide acetate, in patients with islet cell tumors. Gastroenterol Clin North Am 1989;I8:897.

37. Fraker DL, Norton JA. The role of surgery in the management of islet cell tumors. Gastroenterol Clin North Am 1989;18:805.

38. Carty S, Jensen RT, Norton JA. Prospective study of aggressive resection of metastatic pancreatic endocrine tumors. Surgery 1992;112:1024.

39. Ganda PO, Weir GC, Soeldner JS, et al. Somatostatinoma: A somato-statin-containing tumor of the endocrine pancreas. N Engl J Med 1977:296:963.

40. Larsson LI, Hirsch MA, Holst J, et al. Pancreatic somatostatinoma: Clinical features and physiologic implications. Lancet 1977; 1:666.

41. Krejs GJ, Orci L, Conlon M, et al. Somatostatinoma syndrome (biochemical, morphological, and clinical features). N Engl J Med 1979;301:285.

42. Verner JV, Morrison AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med 1958:29:529.

43. Marks IN, Bank S, Louw JH. Islet cell tumor of the pancreas with reversible watery diarrhea and achlorhydria. Gastroenterology 1967;52:695.

44. O'Dorisio TM, Mehkjian HS. VIPoma syndrome. In: Cohen S. Soloway RD (eds), Hormone Producing Tumors of the Pancreas. New York, Churchill Livingstone, 1985, p 101.

45. O'Dorisio TM, Mehkjian HS, Gaginella TS. Medical therapy of VIPomas. Endocrinol Metab Clin North Am 1989;18:545.

46. Bloom SR, Long RG, Bryant MG, et al. Clinical, biochemical and pathological studies on 62 VIPomas. Gastroenterology 1980;78:1143.

47. Verner JV, Morrison AB. Non-B islet tumors and the syndrome of watery diarrhea, hypokalemia and hypochlorhydria. Clin Gastroenterol 1974;3:595.

48. Maton PN, O'Dorisio TM, Howe BA, et al. Effect of a long-acting somatostatin analogue (SMS 201-995) in a patient with pancreatic cholera. N Engl J Med 1985;312:17.

49. Maton PN, Gardner JD, Jensen RT. The use of the long acting somatostatin analogue 201-995 in patients with pancreatic endocrine tumors. Dig Dis Sci 1989;34:29.

50. Sofka CM, Semelka RC, Marcos HB, Woosley JT. MR imaging of metastatic pancreatic VIPoma. Magn Reson Imaging 1997:15:1205.

51. Mortele KJ, Oei A, Bauters W, et al. Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome. Eur Radiol 2001; 11:1952.

52. Nagomey DM, Bloom SR, Polak JM, Blumgart LM. Resolution of recurrent Verner-Morrison syndrome by resection of metastatic VIPoma. Surgery 1983:93:348.

53. Maton PN. Octreotide acetate and islet cell tumors. Med Clin North Am 1989:18:897.

54. Maton PN, Gardner JD, Jensen RT. The incidence and etiology of Cushing's syndrome in patients with the Zollinger-Ellison syndrome. N Engl J Med 1986;315:1.

55. Schoevaerdts D, Favet L, Zekry D, et al. VIPOMA: Effective treatment with octreotide in the oldest old. J Am Geriatric Soc 2001:49:496.

56. McGavran MH, Unger RH, Recant L, et al. A glucagon-secreting alpha-cell carcinoma of the pancreas. N Engl J Med 1966; 274:1408.

57. Mallison CN, Bloom SR, Warin AP, et al. A glucagonoma syndrome. Lancet 1974;2:1.

58. Norton JA, Kahn CR, Schieberger R, et al. Amino acid deficiency and skin rash associated with glucagonoma. Ann Intern Med 1979; 91:213.

59. Wilkinson DS. Necrolytic migratory erythema with carcinoma of the pancreas. Trans St John's Hosp Dermatol Soc 1973;59:244.

60. Kahan RS, Perez-Figaredo RA, Neimanis A. Necrolytic migratory erythema: Distinctive dermatosis of the glucagonoma syndrome. Arch Dermatol 1977:113:792.

61. Vinik Al, Moattari AR. Treatment of endocrine tumors. Endocrinol Metab Clin North Am 1989; 18:483.

62. Stacpoole PW. The glucagonoma syndrome: Clinical features, diagnosis, and treatment. Endocr Rev 1981;2:347.

63. Chang-Cretien K, Chew JT, Judge DP. Reversible dilated cardiomyopathy associated with glucagonoma. Heart 2004;90:1.

64. Aldridge MC, Williamson RCN. Surgery of endocrine tumours of the pancreas. In: Lynn J, Bloom SR (eds), Surgical Endocrinology. New York, Butterworth, 1993, p 503.

65. Carty SE, Jensen RT, Norton JA. Prospective study of aggressive resection of metastatic pancreatic endocrine tumors. Surgery 1992;112:1024.

66. Rivier J, Spiess J, Thorner M, Vale W. Characterization of a growth hormone-releasing factor from a human pancreatic islet cell tumour. Nature 1982;300:276.

67. Thomer MO, Perryman RL, Cronin MJ, et al. Somatotroph hyperplasia. J Clin Invest 1982:70:965.

68. Sano T, Asa SL, Kovacs K. Growth hormone releasing-producing tumors: Clinical, biochemical and morphological manifestations. Endocr Rev 1988;9:357.

69. Zeiger MA, Pass HI, Doppman JD, et al. Surgical strategy in the management of non-small cell ectopic adrenocorticotropic hormone syndrome. Surgery 1992;112:994.

70. Bresley L, Borssel P, Conroy T, Grosdidier J. Pancreatic islet cell carcinoma with hypercalcemia: Complete remission 5 years after surgical excision and chemotherapy. Am J Gastroenterol 1991;86:635.

71. Arps H, Dietel M, Schulz A, et al. Pancreatic endocrine carcinoma with ectopic PTH-production and paraneoplasia hypercalcaemia. Virchows Arch A Pathol Anat Histopathol 1986;408:497.

72. Blackburn AM, Bryant MG, Adrian TE, Bloom SR. Pancreatic tumors produce neurotensin. J Clin Endocrinol Metab 1981:52:820.

73. Shulkes A, Boden R, Cook I, et al. Characterization of a pancreatic tumor containing vasoactive intestinal peptide, neurotensin and pancreatic polypeptide. J Clin Endocrinol Metab 1984;58:41.

74. Corbetta S, Peracchi M, Capiello V, et al. Circulating ghrelin levels in patients with pancreatic and gastrointestinal neuroendocrine tumors; identification of one pancreatic ghrelinoma. J Clin Endocrinol Metab 2003:88:3117.

75. Pathak RD, Tran TH, Burshell AL. A case of dopamine agonists inhibiting pancreatic polypeptide secretion from an islet cell tumor. J Clin Endocrinol Metab 2004;89:581.

76. Grino P, Martinez J, Grino E, et al. Acute pancreatitis secondary to pancreatic neuroendocrine tumors. JOP 2003;4:104.

77. Ramsay D, Gibson P, Edmunds S, Mendelson R. Pancreatic islet cell tumours presenting as recurrent acute pancreatitis: Imaging features in three cases. Australas Radiol 2001 ;45:520.

78. Iguchi H, Yasuda D, Yamada Y, et al. 7B2, a possible marker for nonfunctioning pancreatic islet cell tumor. Horm Metab Res 1991;23;486.

79. Kent RB, van Heerden JA, Weiland LH. Nonfunctioning islet cell tumors. Ann Surg 1981:193:185.

80. Eckhauser FE, Cheung PS, Vinik Al, et al. Nonfunctioning malignant neuroendocrine tumors of the pancreas. Surgery 1986;100:978.

81. VonKatesh S, Ordonoz NG, Ajani J, et al. Islet cell carcinoma of the pancreas: A study of 98 patients. Cancer 1990;65:354.

82. Lin TH, Zhu Y, Lui QI, et al. Nonfunctioning pancreatic endocrine tumors: An immunohistochemical and electron microscopic analysis of 26 cases. Pathol Res Pract 1992:188:191.

83. Danforth DN, Gorden P, Brennan MF. Metastatic insulin secreting carcinoma of the pancreas: Clinical course and the role of surgery. Surgery 1984;96:1027.

84. Norton JA, Sugarbaker PH, Doppman JL, et al. Aggressive resection of metastatic disease in selected patients with malignant gastrinoma. Ann Surg 1986:203:352.

85. Bolden G. Insulinoma and glucagonoma. Semin Oncol 1987;14:253.

10 Ways To Fight Off Cancer

10 Ways To Fight Off Cancer

Learning About 10 Ways Fight Off Cancer Can Have Amazing Benefits For Your Life The Best Tips On How To Keep This Killer At Bay Discovering that you or a loved one has cancer can be utterly terrifying. All the same, once you comprehend the causes of cancer and learn how to reverse those causes, you or your loved one may have more than a fighting chance of beating out cancer.

Get My Free Ebook

Post a comment