7213 Severe IGF-2 Mediated Hypoglycemia In A Patient With Adrenocortical Carcinoma: A Case Report

Abstract

Abstract Disclosure: M. Camelo Pereira: None. P. Itersky: None. S. Sternlieb: None. N. Manuel: None. J. Dendy: None. Background: Hypoglycemia has been rarely described as a complication of adrenocortical carcinoma (ACC). Tumor cells produce insulin-like growth factor II, causing a paraneoplastic syndrome with severe refractory hypoglycemia. We report the unusual case of a patient with known ACC presented with seizures and severe IGF-2 mediated hypoglycemia. Clinical case: A 21-year-old man presented to a regional hospital due to new onset seizures in September 2023. He had been diagnosed with ACC in July 2022, when he presented to the hospital with frank Cushing’s syndrome. CT scan of his chest and abdomen at that time revealed a large left suprarenal mass and multiple lung and liver masses; a biopsy from the liver lesion confirmed the diagnosis of ACC and he was started on treatment with mitotane, levoketoconazole and hydrocortisone. Oncology attempted treatment with 3 cycles of cisplatin and etoposide, with no significant response; subsequently, treatment with Keytruda initially decreased tumor size, but ultimately his CT scan showed progression of disease and mitotane was stopped in August 2023. When brought to the hospital in September 2023 due to seizures and altered mental status, the patient’s blood glucose (BG) was 25 mg/dL (70-110 mg/dL) on point of care testing, so he was started on IV 10% dextrose with improvement in BG and mental status. He underwent endotracheal intubation and was taken to the intensive care unit, showed improvement and was eventually transferred to the general medical floor, where the dextrose infusion was discontinued. However, the following day, he had another hypoglycemic episode complicated with a seizure and had to be reintubated, returning to intensive care. The patient was subsequently transferred to a large tertiary hospital. Evaluation showed undetectable point-of-care BG (< 5 mg/dL) as well as hypokalemia. Fasting labs showed undetectable BHB, undetectable insulin, C-peptide of 0.32 ng/mL (0.78 - 5.19 ng/mL), and negative insulin antibodies. IGF-2/IGF-1 ratio was elevated at 15.6, suggesting IGF-2 mediated hypoglycemia. He was started on IV glucose 20% and glucagon infusions. Mitotane was restarted aiming to decrease tumor burden and consequently IGF-2 levels. Given his hypokalemia, his levoketoconazole dose had to be increased; he was also treated with stress dose steroids. His BG levels improved, and he had partial neurologic recovery, however ultimately curative treatment was not possible given the extent of his disease, so he was transitioned to comfort-focused care. Conclusion: This case illustrates the importance of appropriate workup of IGF-2 mediated hypoglycemia, allowing for the best possible therapeutic approach. In our case, reducing tumor burden was not feasible since the patient had widely metastatic disease, leading to a poor outcome. Presentation: 6/3/2024