Hyperglucagonemia (Glucagonoma Syndrome) Treatment & Management

Updated: Feb 18, 2019
  • Author: George T Griffing, MD; Chief Editor: George T Griffing, MD  more...
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Treatment

Medical Care

Medical treatment of glucagonoma syndrome includes therapy for NME, treatment of diabetes, treatment of hyperglucagonemia, and treatment of islet cell tumor.

NME has been documented to respond to surgical resection of the glucagonoma, to therapy with octreotide, and to chemotherapy, all of which lead to reduction in glucagon levels. [15, 16, 18, 19, 20, 1, 21, 22, 24]  Amino acid supplementation and total parenteral nutrition, even in the presence of elevated glucagon levels, are shown to lead to dramatic improvement of NME. NME is reported to respond to omega-3 triglyceride therapy. The response of NME to zinc supplementation and to topical zinc has been described, but the role of zinc deficiency in the etiology of NME remains unclear. Other agents used in the treatment of NME include tetracycline and hydrocortisone topical creams.

The control of diabetes in glucagonoma syndrome usually can be achieved with diet, oral hypoglycemic agents, or, in some cases, insulin.

Octreotide is the therapeutic agent of choice for hyperglucagonemia. It is used alone, in combination with chemotherapy, or in conjunction with hepatic artery embolization. A long-acting analogue of somatostatin, octreotide, which has a half-life of 3 hours, can be employed preoperatively prior to the surgical resection or debulking of large metastatic tumors. The drug acts by blocking the secretion and effects of glucagon [45] and is particularly effective in the treatment of NME and diarrhea.

The most commonly used treatment for islet cell tumor is combination chemotherapy with streptozocin and 5-fluorouracil, which is reported to cause tumor shrinkage in as many as 10% of patients. Other chemotherapeutic agents used in combination include doxorubicin, dacarbazine, cisplatin, etoposide, lomustine, cyclophosphamide, and interferon. Chemotherapeutic agents are occasionally used in combination with octreotide.

In rapidly progressive disease, a multimodality approach has been advocated, with the use of surgery or hepatic artery embolization, octreotide, and chemotherapy.

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Surgical Care

Surgery is the treatment of choice for glucagonoma syndrome. Surgical treatment includes the following:

  • Resection of a localized tumor, including, in selected cases, through laparoscopic surgery [21, 22, 23]

  • Cytoreduction or debulking of large and nonresectable metastatic tumors

  • Hepatic artery embolization - This procedure can be used alone or in combination with the somatostatin analogue octreotide or with chemotherapy for unresectable hepatic metastases. Hepatic artery embolization works on the principle that most of the blood supply to the tumor is derived from the hepatic artery, whereas the blood supply to the healthy liver parenchyma comes from the portal vein. Embolization of the hepatic artery leads to tumor necrosis.

  • Liver transplantation [46]

Preoperatively, patients may require the following:

  • Total parenteral nutrition with amino acid, fatty acid, and zinc supplementation

  • Blood transfusion - In cases of severe anemia

  • Proper treatment and control of diabetes

  • Heparin - For the prophylaxis of deep venous thrombosis

  • Treatment with octreotide

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