Glucagonoma Treatment & Management

  • Author: Luigi Santacroce, MD; Chief Editor: George T Griffing, MD   more...
 
Updated: May 23, 2011
 

Medical Care

Currently, active drugs used to treat glucagonoma do not exist, although some drugs can cause partial regression of a neoplastic mass or improvements in the symptoms of necrolytic migratory erythema (NME).[10]

  • In the literature, good results have been obtained with doxorubicin and streptozotocin (5-fluorouracil [5-FU] and streptozotocin), via selective damage of islets cells.[9]
  • Long-acting octreotide, analogous to human somatostatin, causes NME symptom regression in some, but not all, patients.[8, 9, 11, 12]
  • The remission of glucagonoma through treatment with dacarbazine has been described in a single patient.
  • Everolimus was approved by the US Food and Drug Administration (FDA) in May 2011, for progressive neuroendocrine tumors located in the pancreas (PNET) that are metastatic or are not surgically resectable. The approval was based on a study that showed an increased median progression-free survival of 11.0 months with everolimus compared with 4.6 months with placebo.[13]
  • Sunitinib was also approved by the FDA in May 2011, for PNET. The study was discontinued early, after the independent data and safety monitoring committee observed more serious adverse events and deaths in the placebo group but an improvement in progression-free survival in the sunitinib group. The sunitinib group showed a median progression-free survival of 11.4 months compared with the placebo group of 5.5 months.[14]
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Surgical Care

Once a glucagonoma is identified, the optimal management is surgical resection, which is the only curative therapy.[10, 15, 16]

  • In some patients, removal of the tumor may reverse symptoms.
  • Patients with liver metastases and severe symptoms caused by tumor bulk or hormone-release syndromes may benefit from procedures that reduce hepatic arterial blood flow to metastases, including hepatic arterial occlusion with embolization or chemoembolization that causes a necrosis of the metastases without damaging the healthy hepatic parenchyma, which is supplied from the portal circulation.[17, 18] This treatment may also be combined with systemic chemotherapy in selected patients.
  • Multimodal therapeutic interventions including liver transplantation are reported, but the results need further studies to confirm and validate such time and cost expensive procedure.[19]
  • Beyond neoplasm removal, resect healthy surrounding parenchyma and locoregional lymph nodes, which can occasionally be metastatic or, more rarely, the primary site of the tumor.
  • Several authors have reported the clinical palliation of symptoms from surgical debulking of the tumor.[20]
  • Although first performed in 1996, laparoscopic resection of pancreatic endocrine tumors has seen only limited use, because there has not been a great deal of data about the safety, feasibility, indications for, and outcomes after such intervention. In 2008, however, Fernández-Cruz and colleagues reported on 49 patients who underwent laparoscopic pancreatic surgery.[21] The authors concluded that such surgery is a safe, feasible means of treating benign endocrine pancreatic tumors.
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Consultations

The occurrence of mucocutaneous lesions, endocrine disturbances, and optic and psychic disturbances may be very helpful for differential diagnosis, therapy, and needed consultations (from dermatologists, neurologists, endocrinologists, ophthalmologists).

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Diet

In patients with glucagonoma, providing a supplemental protein supply in order to furnish amino acids is useful. In more severe cases, such supplementation can be administered intravenously. The administration of essential fatty acids (ie, olive oil), zinc, vitamins, and minerals is also helpful.

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Activity

Mild exercise is usually not harmful in patients with glucagonoma.

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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)

Laura Diomede  University of Bari School of Medicine, Italy

Disclosure: Nothing to disclose.

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 and Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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

Disclosure: eMedicine Salary Employment

Romesh Khardori, MD, PhD, FACP  Former Professor, Department of Medicine, Former Chief, Division of Endocrinology, Metabolism, and Molecular Medicine, Southern Illinois University School of Medicine

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, and Endocrine Society

Disclosure: Nothing to disclose.

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.

References

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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.
 
 
 
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