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Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Insulinoma

Curative surgical excision, by open laparotomy or laparoscopy, is the treatment of choice when possible. The open surgical approach is used if the tumor is suspected to be malignant, since en bloc lymphadenectomy is performed for malignant tumors without distant metastases. Intraoperative ultrasound aids the localization of tumor extent and the relationship to other anatomic structures.[1]

Standard treatment options:

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  1. Single small lesion in head or tail of pancreas:[1,2,3,4]
    • Enucleation, if feasible.
  2. Large lesion in the head of the pancreas that is not amenable to enucleation:[1,2,3,4]
    • Pancreaticoduodenectomy.
  3. Single large lesion in body/tail:[1,2,3,4]
    • Distal pancreatectomy.
  4. Multiple lesions: occur in 10%, often in association with multiple endocrine neoplasia syndrome type 1 (MEN-1):[1,2,3,4]
    • Distal pancreatectomy with enucleation of tumors in the head of the pancreas.
  5. Metastatic lesions: lymph nodes or distant sites:[5,6,7,8,9,10,11,12]
    • Resect when possible.
    • Consider radiofrequency or cryosurgical ablation, if not resectable.
  6. Unresectable:[13,14,15,16,17,18,19,20,21,22]
    • Combination chemotherapy.
    • Pharmacologic palliation: diazoxide 300 to 500 mg/day.
    • Somatostatin analogue therapy.

Patients with hepatic-dominant disease and substantial symptoms caused by tumor bulk or hormone-release syndromes may benefit from procedures that reduce hepatic arterial blood flow to metastases (hepatic arterial occlusion with embolization or with chemoembolization).[6,8,9,10,11,12] Such treatment may also be combined with systemic chemotherapy in selected patients.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with insulinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Davies K, Conlon KC: Neuroendocrine tumors of the pancreas. Curr Gastroenterol Rep 11 (2): 119-27, 2009.
  2. Phan GQ, Yeo CJ, Hruban RH, et al.: Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: Review of 125 patients. J Gastrointest Surg 2 (5): 473-82, 1998 Sep-Oct.
  3. Kazanjian KK, Reber HA, Hines OJ: Resection of pancreatic neuroendocrine tumors: results of 70 cases. Arch Surg 141 (8): 765-9; discussion 769-70, 2006.
  4. Hochwald SN, Zee S, Conlon KC, et al.: Prognostic factors in pancreatic endocrine neoplasms: an analysis of 136 cases with a proposal for low-grade and intermediate-grade groups. J Clin Oncol 20 (11): 2633-42, 2002.
  5. Sarmiento JM, Que FG: Hepatic surgery for metastases from neuroendocrine tumors. Surg Oncol Clin N Am 12 (1): 231-42, 2003.
  6. Gupta S, Johnson MM, Murthy R, et al.: Hepatic arterial embolization and chemoembolization for the treatment of patients with metastatic neuroendocrine tumors: variables affecting response rates and survival. Cancer 104 (8): 1590-602, 2005.
  7. Nguyen C, Faraggi M, Giraudet AL, et al.: Long-term efficacy of radionuclide therapy in patients with disseminated neuroendocrine tumors uncontrolled by conventional therapy. J Nucl Med 45 (10): 1660-8, 2004.
  8. Kennedy AS, Dezarn WA, McNeillie P, et al.: Radioembolization for unresectable neuroendocrine hepatic metastases using resin 90Y-microspheres: early results in 148 patients. Am J Clin Oncol 31 (3): 271-9, 2008.
  9. King J, Quinn R, Glenn DM, et al.: Radioembolization with selective internal radiation microspheres for neuroendocrine liver metastases. Cancer 113 (5): 921-9, 2008.
  10. Siperstein AE, Berber E: Cryoablation, percutaneous alcohol injection, and radiofrequency ablation for treatment of neuroendocrine liver metastases. World J Surg 25 (6): 693-6, 2001.
  11. Gurusamy KS, Ramamoorthy R, Sharma D, et al.: Liver resection versus other treatments for neuroendocrine tumours in patients with resectable liver metastases. Cochrane Database Syst Rev (2): CD007060, 2009.
  12. Gurusamy KS, Pamecha V, Sharma D, et al.: Palliative cytoreductive surgery versus other palliative treatments in patients with unresectable liver metastases from gastro-entero-pancreatic neuroendocrine tumours. Cochrane Database Syst Rev (1): CD007118, 2009.
  13. di Bartolomeo M, Bajetta E, Buzzoni R, et al.: Clinical efficacy of octreotide in the treatment of metastatic neuroendocrine tumors. A study by the Italian Trials in Medical Oncology Group. Cancer 77 (2): 402-8, 1996.
  14. 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 326 (8): 519-23, 1992.
  15. Kouvaraki MA, Ajani JA, Hoff P, et al.: Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas. J Clin Oncol 22 (23): 4762-71, 2004.
  16. Kulke MH, Hornick JL, Frauenhoffer C, et al.: O6-methylguanine DNA methyltransferase deficiency and response to temozolomide-based therapy in patients with neuroendocrine tumors. Clin Cancer Res 15 (1): 338-45, 2009.
  17. Yao JC, Lombard-Bohas C, Baudin E, et al.: Daily oral everolimus activity in patients with metastatic pancreatic neuroendocrine tumors after failure of cytotoxic chemotherapy: a phase II trial. J Clin Oncol 28 (1): 69-76, 2010.
  18. Yao JC, Shah MH, Ito T, et al.: A randomized, double-blind, placebo-controlled multicenter phase III trial of everolimus in patients with advanced pancreatic neuroendocrine tumors (PNET) (RADIANT-3). [Abstract] Ann Oncol 21 (Suppl 8): A-LBA9, viii4-5, 2010.
  19. Raymond E, Niccoli-Sire P, Bang Y: Updated results of the phase III trial of sunitinib (SU) versus placebo (PBO) for treatment of advanced pancreatic neuroendocrine tumors (NET). [Abstract] American Society of Clinical Oncology 2010 Gastrointestinal Cancers Symposium, 22–24 January 2010, Orlando, Florida. A-127, 2010.
  20. Teunissen JJ, Kwekkeboom DJ, de Jong M, et al.: Endocrine tumours of the gastrointestinal tract. Peptide receptor radionuclide therapy. Best Pract Res Clin Gastroenterol 19 (4): 595-616, 2005.
  21. Kwekkeboom DJ, de Herder WW, Kam BL, et al.: Treatment with the radiolabeled somatostatin analog [177 Lu-DOTA 0,Tyr3]octreotate: toxicity, efficacy, and survival. J Clin Oncol 26 (13): 2124-30, 2008.
  22. Bushnell DL Jr, O'Dorisio TM, O'Dorisio MS, et al.: 90Y-edotreotide for metastatic carcinoid refractory to octreotide. J Clin Oncol 28 (10): 1652-9, 2010.
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Last Updated: February 25, 2014
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