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Glucagonoma Follow-up

  • Author: Luigi Santacroce, MD; Chief Editor: George T Griffing, MD  more...
 
Updated: May 14, 2015
 

Further Outpatient Care

Psychotherapeutic intervention may be very helpful when comorbid depression is present.

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Further Inpatient Care

For inpatients, psychotherapeutic intervention may be very helpful when comorbid depression is present.

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Inpatient & Outpatient Medications

Because of severe weight loss, a period of total parenteral nutrition may be needed as part of the preoperative preparation for patients with glucagonoma. Antibiotics, steroids, amino acids, and zinc supplementation may improve severe skin rash. Octreotide is also useful to help improve the perioperative condition of these patients. Prophylaxis for venous thrombosis and the subcutaneous administration of low-dose heparin are mandatory treatments for all patients during the perioperative period.

In patients for whom surgery is not feasible, consider administering streptozotocin and doxorubicin or streptozotocin and 5-FU.

In patients with widespread metastases, consider hepatic artery catheterism for doxorubicin + cisplatin + mitomycin-C administration. Such treatment can determine colliquative necrosis and mass reduction in large number of patients. In addition, octreotide administration before, during, and after locoregional therapy may stop the crisis due to the massive release of glucagon.

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Complications

The main complication of glucagonoma is hepatic metastasis or metastasis of the locoregional lymph nodes.

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Prognosis

The skin changes observed in patients with glucagonoma usually improve with resection of the tumor.

The prognosis for glucagonoma is poor. The rate of survival after 5 years is difficult to determine because of the small number of patients who develop the disease. One study, however, reported an average survival time of 3.7 years in a group of 12 patients and an average survival period of 4.9 years in another group, consisting of 9 patients.

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Patient Education

The main medicolegal problems related to the treatment of glucagonoma are the toxic effects of antiproliferative drugs and the consequences of surgical procedures. For this reason, always obtain an informed consent for any procedure or treatment, explaining all procedures and their possible complications.

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Contributor Information and Disclosures
Author

Luigi Santacroce, MD Assistant Professor, Medical School, State University at Bari, Italy

Disclosure: Nothing to disclose.

Coauthor(s)

Mini R Abraham, MD Consulting Staff, Overland Park Medical Specialists

Mini R Abraham, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Endocrine Society

Disclosure: Nothing to disclose.

Laura Diomede University of Bari School of Medicine, Italy

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Romesh Khardori, MD, PhD, FACP Professor of Endocrinology, Director of Training Program, Division of Endocrinology, Diabetes and Metabolism, Strelitz Diabetes and Endocrine Disorders Institute, Department of Internal Medicine, Eastern Virginia Medical School

Romesh Khardori, MD, PhD, FACP is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD Professor Emeritus of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, International Society for Clinical Densitometry, Southern Society for Clinical Investigation, American College of Medical Practice Executives, American Association for Physician Leadership, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical and Translational Research, Endocrine Society

Disclosure: Nothing to disclose.

Additional Contributors

Frederick H Ziel, MD Associate Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group

Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

References
  1. McGavran MH, Unger RH, Recant L, et al. A glucagon-secreting alpha-cell carcinoma of the pancreas. N Engl J Med. 1966 Jun 23. 274(25):1408-13. [Medline].

  2. Yabe D, Rokutan M, Miura Y, Komoto I, Usui R, Kuwata H, et al. Enhanced glucagon-like peptide-1 secretion in a patient with glucagonoma: implications for glucagon-like peptide-1 secretion from pancreatic a cells in vivo. Diabetes Res Clin Pract. 2013 Oct. 102(1):e1-4. [Medline].

  3. Pujol RM, Wang CY, el-Azhary RA, et al. Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol. 2004 Jan. 43(1):12-8. [Medline].

  4. Remes-Troche JM, Garcia-de-Acevedo B, Zuniga-Varga J, et al. Necrolytic migratory erythema: a cutaneous clue to glucagonoma syndrome. J Eur Acad Dermatol Venereol. 2004 Sep. 18(5):591-5. [Medline].

  5. Georgiou GK, Gizas I, Katopodis KP, Katsios CS. Non-secreting benign glucagonoma diagnosed incidentally in a patient with refractory thrombocytopenic thrombotic purpura: report of a case. Surg Today. 2014 Nov 6. [Medline].

  6. el Darouty M, Abu el Ela M. Necrolytic migratory erythema without glucagonoma in patients with liver disease. J Am Acad Dermatol. 1996 Jun. 34(6):1092-3. [Medline].

  7. Nakashima H, Komine M, Sasaki K, et al. Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. J Dermatol. 2006 Aug. 33(8):557-62. [Medline].

  8. Yao JC, Eisner MP, Leary C, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol. 2007 Dec. 14(12):3492-500. [Medline]. [Full Text].

  9. Fang S, Li S, Cai T. Glucagonoma syndrome: a case report with focus on skin disorders. Onco Targets Ther. 2014. 7:1449-53. [Medline]. [Full Text].

  10. Xu Q, Chen WH, Huang QJ. Spiral CT localization of pancreatic functioning islet cell tumors. Hepatobiliary Pancreat Dis Int. 2004 Nov. 3(4):616-9. [Medline].

  11. Melen-Mucha G, Lawnicka H, Kierszniewska-Stepien D, et al. The place of somatostatin analogs in the diagnosis and treatment of the neuoroendocrine glands tumors. Recent Patents Anticancer Drug Discov. 2006 Jun. 1(2):237-54. [Medline].

  12. Kindmark H, Sundin A, Granberg D, et al. Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years. Med Oncol. 2007. 24(3):330-7. [Medline].

  13. O'Grady HL, Conlon KC. Pancreatic neuroendocrine tumours. Eur J Surg Oncol. 2008 Mar. 34(3):324-32. [Medline].

  14. Moattari AR, Cho K, Vinik AI. Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses. Surgery. 1990 Sep. 108(3):581-7. [Medline].

  15. Tomassetti P, Migliori M, Corinaldesi R, et al. Treatment of gastroenteropancreatic neuroendocrine tumours with octreotide LAR. Aliment Pharmacol Ther. 2000 May. 14(5):557-60. [Medline].

  16. Yao JC, Shah MH, Ito T, Bohas CL, Wolin EM, Van Cutsem E, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011 Feb 10. 364(6):514-23. [Medline].

  17. Raymond E, Dahan L, Raoul JL, Bang YJ, Borbath I, Lombard-Bohas C, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011 Feb 10. 364(6):501-13. [Medline].

  18. Caplin ME, Pavel M, Cwikla JB, Phan AT, Raderer M, Sedlácková E, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014 Jul 17. 371(3):224-33. [Medline]. [Full Text].

  19. Pautrat K, Bretagnol F, de Muret A, et al. [Recurrent glucagonoma 20 years after surgical resection]. Gastroenterol Clin Biol. 2003 Dec. 27(12):1163-5. [Medline].

  20. Akerstrom G, Hellman P, Hessman O, et al. Surgical treatment of endocrine pancreatic tumours. Neuroendocrinology. 2004. 80 Suppl 1:62-6. [Medline].

  21. Montenegro F, Lawrence GD, Macon W, et al. Metastatic glucagonoma. Improvement after surgical debulking. Am J Surg. 1980 Mar. 139(3):424-7. [Medline].

  22. Fernandez-Cruz L, Blanco L, Cosa R, Rendon H. Is laparoscopic resection adequate in patients with neuroendocrine pancreatic tumors?. World J Surg. 2008 May. 32(5):904-17. [Medline].

  23. Clouse ME, Perry L, Stuart K, et al. Hepatic arterial chemoembolization for metastatic neuroendocrine tumors. Digestion. 1994. 55 Suppl 3:92-7. [Medline].

  24. King J, Quinn R, Glenn DM, et al. Radioembolization with selective internal radiation microspheres for neuroendocrine liver metastases. Cancer. 2008 Sep 1. 113(5):921-9. [Medline].

  25. Radny P, Eigentler TK, Soennichsen K, et al. Metastatic glucagonoma: treatment with liver transplantation. J Am Acad Dermatol. 2006 Feb. 54(2):344-7. [Medline].

  26. Adam DN, Cohen PD, Ghazarian D. Necrolytic migratory erythema: case report and clinical review. J Cutan Med Surg. 2003 Jul-Aug. 7(4):333-8. [Medline].

  27. Baton O, Eggenspieller P, Bechade D, et al. [Median pancreatectomy for early glucagonoma]. Gastroenterol Clin Biol. 2005 Mar. 29(3):308-10. [Medline].

  28. Bhathena SJ, Higgins GA, Recant L. Glucagonoma and glucagonoma syndrome. Unger RH, Orci L, eds. Glucagon. New York, NY: Elsevier Science; 1981. 413.

  29. Cruz-Bautista I, Lerman I, Perez-Enriquez B, et al. Diagnostic challenge of glucagonoma: case report and literature review. Endocr Pract. 2006 Jul-Aug. 12(4):422-6. [Medline].

  30. Du Jardin P, Cools P, Van der Stighelen Y. Necrolytic migratory erythema: first symptom of a glucagonoma. A case report. Acta Chir Belg. 2004 Aug. 104(4):468-70. [Medline].

  31. Echenique-Elizondo M, Elorza JL, De Delas JS. Migratory necrolytic erythema and glucagonoma. Surgery. 2003 Apr. 133(4):449-50. [Medline].

  32. Grant CS. Surgical management of malignant islet cell tumors. World J Surg. 1993 Jul-Aug. 17(4):498-503. [Medline].

  33. Jabbour SA, Davidovici BB, Wolf R. Rare syndromes. Clin Dermatol. 2006 Jul-Aug. 24(4):299-316. [Medline].

  34. Koike N, Hatori T, Imaizumi T, et al. Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus. J Hepatobiliary Pancreat Surg. 2003. 10(1):101-5. [Medline].

  35. Kovacs RK, Korom I, Dobozy A, et al. Necrolytic migratory erythema. J Cutan Pathol. 2006 Mar. 33(3):242-5. [Medline].

  36. Krause W. Skin diseases in consequence of endocrine alterations. Aging Male. 2006 Jun. 9(2):81-95. [Medline].

  37. Marko PB, Miljkovic J, Zemljic TG. Necrolytic migratory erythema associated with hyperglucagonemia and neuroendocrine hepatic tumors. Acta Dermatovenerol Alp Panonica Adriat. 2005 Dec. 14(4):161-4, 166. [Medline]. [Full Text].

  38. 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. [Medline].

  39. Schanz S, Schaefer J, Fierlbeck G. Glucagonoma presenting with necrolytic migratory erythema: the glucagonoma syndrome. Gastroenterology. 2005 Dec. 129(6):1816, 2131. [Medline].

  40. Tomita T, Masuzaki H, Noguchi M, et al. GPR40 gene expression in human pancreas and insulinoma. Biochem Biophys Res Commun. 2005 Dec 30. 338(4):1788-90. [Medline].

  41. Vinik AI, Perry RR. Neoplasms of the gastroenteropancreatic endocrine system. Holland JF, Frei E III, Bast RC Jr, et al, eds. Cancer Medicine. 4th ed. Baltimore, Md: William & Wilkins; 1997.

  42. Wang L, Zhao YP, Lee CI, et al. Diagnosis and treatment of malignant pancreatic endocrine tumour. Chin Med Sci J. 2004 Jun. 19(2):130-3. [Medline].

  43. Zhang M, Xu X, Shen Y, et al. Clinical experience in diagnosis and treatment of glucagonoma syndrome. Hepatobiliary Pancreat Dis Int. 2004 Aug. 3(3):473-5. [Medline].

 
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A section of a glucagonoma mass with several fiber bundles and solid cellular strands (125 X). Courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
A section of a glucagonoma mass with irregular aspects of fiber bundles and cellular strands (400 X). Courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
Bland embolization of the right hepatic artery in a patient with metastatic neuroendocrine tumors: Part 1. Courtesy of Memorial Sloan-Kettering Cancer Center.
Bland embolization of the right hepatic artery in a patient with metastatic neuroendocrine tumors: Part 2. Courtesy of Memorial Sloan-Kettering Cancer Center.
Postprocedure computed tompgraphy scans after bland embolization of the right hepatic artery in a patient with metastatic neuroendocrine tumors. Courtesy of Memorial Sloan-Kettering Cancer Center.
 
 
 
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