Glucagonoma Treatment & Management

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

Medical Care

Some drugs can cause partial regression of a neoplastic mass or improvements in the symptoms of necrolytic migratory erythema (NME). [13]

In the literature, good results have been obtained with doxorubicin and streptozotocin (5-fluorouracil [5-FU] and streptozotocin), via selective damage of islets cells. [12]

Long-acting octreotide, analogous to human somatostatin, causes NME symptom regression in some, but not all, patients. [11, 12, 14, 15]

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. [16]

Sunitinib was also approved by the FDA in May 2011 for PNET. The study was discontinued 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, whereas the placebo group showed a median progression-free survival of 5.5 months. [17]

Lanreotide (Somatuline Depot) is approved by the FDA as a once monthly deep subcutaneous injection for unresectable, well-or moderately-differentiated, locally advanced or metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs) to improve progression-free survival. Its approval was based on the multicenter, international CLARINET trial (n=204). The trial demonstrated a significantly prolonged progression-free survival in those who received lanreotide (hazard ratio, 0.47; 95% confidence interval [CI], 0.30 - 0.73); P < 0.001; log-rank test. [18]

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Surgical Care

Resection

Once a glucagonoma is identified, the optimal management is surgical resection, which is the only curative therapy. [13, 19, 20]

In some patients, removal of the tumor may reverse symptoms. Several authors have reported the clinical palliation of symptoms from surgical debulking of the tumor. [21]

Beyond neoplasm removal, healthy surrounding parenchyma and locoregional lymph nodes can be resected because they may be metastatic or, more rarely, the primary site of the tumor.

Laparoscopic resection of pancreatic endocrine tumors has seen only limited use because of a lack of data about the safety, feasibility, indications for, and outcomes after such an intervention. In 2008, however, Fernández-Cruz and colleagues reported on 49 patients who underwent laparoscopic pancreatic surgery. [22] The authors concluded that such surgery is a safe, feasible means of treating benign endocrine pancreatic tumors.

Embolization

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. The videos below demonstrate bland embolization of the right hepatic artery in a patient with metastatic neuroendocrine tumors.

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.

Hepatic arterial occlusion with embolization or chemoembolization should cause necrosis of the metastases without damaging the healthy hepatic parenchyma, which is supplied from the portal circulation. [23, 24] This treatment may also be combined with systemic chemotherapy in selected patients.

Transplantation

Multimodal therapeutic interventions including liver transplantation have been reported, but further studies are needed to validate such time-consuming and expensive procedures. [25]

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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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