62 year-old female with a history of intracranial hemangiopericytoma admitted to the ICU with altered mental status due to profound hypoglycemia. She was previously diagnosed six years prior with intracranial hemangiopericytoma and treated with resection and stereotactic radiosurgery. The tumor recurred locally three years later and she underwent additional resection. She denied a history of diabetes or treatment with insulin or hypoglycemic agents. Over the past year, she developed intermittent weakness that resolved with oral intake of sugary sodas. Exam revealed a neurologically intact female with hepatomegaly. Abdominal CAT scan demonstrated multiple hepatic masses, the largest measuring 12 cm in diameter.
Initial hypoglycemia (blood glucose <40 mg/dL) recurred despite repeated 25 g dextrose boluses and she required continuous dextrose 10% infusion. Abdominal MRI showed peripherally enhancing solid hepatic masses. Hepatic mass biopsy was morphologically similar to her prior intracranial hemangiopericytoma. Laboratory data revealed low levels of insulin, C-peptide, IGF-1, and proinsulin; negative sulfonylurea screen; and high-normal levels of IGF-2 768 ng/mL (reference range 333-967). She was transitioned to oral prednisone 10 mg three times daily to maintain normal blood glucose levels and discharged with endocrinology and oncology follow up.
Discussion:
Intracranial hemangiopericytoma is a rare neoplasm with potential for recurrence and metastasis. Severe hypoglycemia is a paraneoplastic complication of some malignancies, mediated most commonly by nonislet cell tumor production of insulin-like growth factor 2 (IGF-2) that activates insulin receptors and increases glucose cell entry. Hemangiopericytomas have been shown to cause hypoglycemia by production of IGF-2. In our patient, diagnostic results showed recurrence of metastatic hemangiopericytoma, high-normal IGF-2, and no other endogenous or exogenous etiology of hypoglycemia. We hypothesized that IGF-2, albeit within normal testing range, was elevated enough in our patient to induced hypoglycemia. Symptomatic hypoglycemia resolved through prednisone-mediated euglycemia until further definitive therapy.
Conclusions:
Our case demonstrates the potential of metastatic hemangiopericytoma to induce refractory nonislet cell tumor hypoglycemia via elaborated IGF-2. Recurrence of disease should be suspected in those patients with a prior diagnosis of hemangiopericytoma presenting with hypoglycemia.