Table 1 Management Options for IGF-2oma-mediated Hypoglycemia
Therapy | Comment |
|---|---|
Non-pharmacologic | |
Glucose pushes/ infusion | Initial temporizing management in hospital setting |
Dietary modifications | May be sufficient alone for mild hypoglycemia. Corn starch given at bedtime delays onset of overnight hypoglycemia and may be employed as adjunctive therapy in cases of more severe hypoglycemia. Tube feeds may have utility in the hospital setting/ while preparing for definitive management. |
- Frequent small meals | |
- Complex carbohydrates (corn starch) | |
- Enteral nutrition | |
Continuous glucose monitoring system | Useful for alerting patient to hypoglycemic events (particularly overnight) and for titrating efficacy of therapy. Tylenol interferes with glucose sensor; therefore, should be excluded form pain management regimen |
Pharmacologic | |
Glucocorticoids | Current mainstay of therapy. Inexpensive and effective. Must consider many short and long-term side effects. |
Recombinant growth hormone | Possible adjunct to glucocorticoids; occasional efficacy as monotherapy. Theoretical risk for increased tumor growth. |
mTOR inhibitors | Good efficacy in insulinoma. Not extensively investigated in IGF-2oma, however, successful in this case. Must consider immunosuppressive side effects. |
Glucagon infusion | Effective in preventing overnight hypoglycemia. Must ensure adequate carbohydrate intake to replete hepatic glycogen stores during waking hours. Commercially available glucagon preparations not designed for subcutaneous infusion via pump; therefore, concentration in reservoir must be sufficiently low to prevent line occlusion. Best utilized in monitored setting—inpatient or with home CGMS. Notably, side effects including venous thromboembolism, necrolytic migratory erythema, and angular cheilitis, have been reported in patients receiving intravenous glucagon infusion. |
Octreotide and diazoxide | No role in management of hypoglycemia caused by IGF-2oma |