Medical Care
Medical treatment of glucagonoma syndrome includes the following:
" Therapy for the necrolytic migratory erythema
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o NME has been documented to respond to surgical resection of the glucagonoma or therapy with octreotide and chemotherapy, all of which lead to reduction in the levels of glucagon.
o Amino acid supplementation and total parenteral nutrition, even in the presence of elevated glucagon levels, are shown to lead to dramatic improvement of NME.
o NME is reported to respond to omega-3 triglyceride therapy.
o 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.
o Other agents used in the treatment of NME include tetracycline and hydrocortisone topical creams.
" Treatment for diabetes: The control of diabetes in glucagonoma syndrome usually can be achieved with diet, oral hypoglycemic agents, or, in some cases, insulin.
" Treatment for hyperglucagonemia
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o Octreotide is the therapeutic agent of choice used alone, in combination with chemotherapy, or in conjunction with hepatic artery embolization. This drug can be used preoperatively prior to surgical resection or debulking of large metastatic tumors.
o Octreotide is a long-acting analogue of somatostatin with a half-life of 3 hours. The drug acts by blocking the secretion and the effects of glucagon and is particularly effective in the treatment of NME and diarrhea.
" Treatment of islet cell tumor
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o The most commonly used treatment modality is combination chemotherapy with streptozocin and 5-fluorouracil, which is reported to cause tumor shrinkage in as many as 10% of patients. Other chemotherapy agents used in combination include doxorubicin, dacarbazine, cisplatin, etoposide, lomustine, cyclophosphamide, and interferon. Occasionally, chemotherapy agents are used in combination with octreotide.
o In rapidly progressive disease, a multimodality approach has been advocated, with use of surgery or hepatic artery embolization, octreotide, and chemotherapy.
Surgical Care
Surgery is the treatment of choice for glucagonoma syndrome. Surgical treatment includes the following:
" Resection of a localized tumor
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" Cytoreduction or debulking of large and nonresectable metastatic tumors
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" Hepatic artery embolization, either alone or in combination with somatostatin analogue (octreotide) or 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.
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" Liver transplantation
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" Preoperatively, patients may require the following:
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o Total parenteral nutrition with amino acid, fatty acid, and zinc supplementation
o Blood transfusion - In cases of severe anemia
o Proper treatment and control of diabetes
o Heparin - For prophylaxis of deep venous thrombosis
o Treatment with octreotide
DRUG TREATMENT :
1. SOMATOSTATIN ANALOGUES :
- OCTREOTIDE ACETATE