eMedicine Specialties > Endocrinology > Multiple Endocrine Disease and Miscellaneous Endocrine Disease

Glucagonoma: Follow-up

Author: Luigi Santacroce, MD, Assistant Professor, Medical School, State University at Bari, Italy
Coauthor(s): Laura Diomede, University of Bari School of Medicine, Italy; Mini R Abraham, MD, Consulting Staff, Saint Luke's Medical Group
Contributor Information and Disclosures

Updated: Nov 20, 2008

Follow-up

Further Inpatient Care

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

Further Outpatient Care

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

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.

Complications

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

Prognosis

  • 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.

Miscellaneous

Medicolegal Pitfalls

  • 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.

Special Concerns

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


More on Glucagonoma

Overview: Glucagonoma
Differential Diagnoses & Workup: Glucagonoma
Treatment & Medication: Glucagonoma
Follow-up: Glucagonoma
Multimedia: Glucagonoma
References
Further Reading

References

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

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

  3. 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. Sep 2004;18(5):591-5. [Medline].

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

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

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

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

  8. 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. Jun 2006;1(2):237-54. [Medline].

  9. 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].

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

  28. 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].

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

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

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

  32. Moertel CG, Johnson CM, McKusick MA, et al. The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann Intern Med. Feb 15 1994;120(4):302-9. [Medline].

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

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

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

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

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

Keywords

glucagonoma, glucagon, glucagonoma syndrome, alpha-cell tumor, alpha-cell adenoma, alpha cell, alpha cells, alpha-2 cell, alpha-2 cells, diabetes, diabetes mellitus, diabetes mellitus type 2, pancreas, hyperglycemia, hyperglycemic, neuroendocrine, 4D syndrome, neuroendocrine tumor, islet cell, islet cells, islet cell pancreatic tumor, pancreatic tumor, pancreas tumor, insulin, insulinoma, glucagon overproduction, hypoaminoacidemia, weight loss, normochromic and normocytic anemia, necrolytic migratory erythema, NME, hyperglucagonemia, pancreas, octreotide, Sandostatin, peptide hormone, bioactive peptide, tumor of the pancreas, multiple endocrine neoplasia type1, MEN I, islet celltumor

Contributor Information and Disclosures

Author

Luigi Santacroce, MD, Assistant Professor, Medical School, State University at Bari, Italy
Disclosure: Nothing to disclose.

Coauthor(s)

Laura Diomede, University of Bari School of Medicine, Italy
Disclosure: Nothing to disclose.

Mini R Abraham, MD, Consulting Staff, Saint Luke's Medical Group
Mini R Abraham, MD is a member of the following medical societies: American Association of Clinical Endocrinologists and Endocrine Society
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Novo Nordisk Honoraria Speaking and teaching; Eli Lilly Honoraria Speaking and teaching

Medical Editor

Frederick H Ziel, MD, Associate Professor of Medicine, David Geffen School of Medicine at UCLA; 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, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Romesh Khardori, MD, Chief, Division of Endocrinology, Metabolism and Molecular Medicine, Professor, Department of Internal Medicine, Southern Illinois University School of Medicine
Romesh Khardori, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society of Andrology, Endocrine Society, and Illinois State Medical Society
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor 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, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.