Glucagonoma : Treatment & Prognosis

Comments:
Treatment of Glucagonoma: The definitive treatment is surgical resection; however, uncontrolled diabetes and severe anemia make glucagonoma patients poor surgical candidates. The initial therapeutic steps are medical and aimed at symptomatic relief, restoring nutritional status (correcting deficiencies) and controlling hyperglycemia. Severely anemic patients may need blood transfusions.

Surgical resection is the treatment of choice for glucagonomas that have not yet metastasized. Unfortunately, 50-70% of patients present with liver metastases. However, even with metastases or recurrent tumor, surgical debulking can offer several years of disease-free survival. In some cases, tumor resection with normalization of glucagon levels does not restore glucose tolerance.

Unresectable tumors are treated with somatostatin analogs such as octreotide and lanreotide or mTOR inhibitors such as everolimus for symptomatic relief.

Prognosis: The 5-yr survival rate after surgical resection is 70% for non-metastatic tumors. The overall cure rate is only about 20%.

The image shows an interesting pattern of stromal calcification in a glucagonoma (same case as the previous two images).