Transplant Institute, Department of Surgery, George Washington University School of Medicine and Health Science Washington, DC
Mohamed Abdou, MD1, Abdelrhman Refaey, MD2, Ahmed Attia, MD1, Ahmed Ebeid, MD1, Mamoun Younes, MD3, Ameer Abutaleb, MD4 1Transplant Institute, Department of Surgery, George Washington University School of Medicine and Health Science, Washington, DC; 2Division of Gastroenterology and Liver Disease, Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC; 3Department of Pathology, The George Washington University Hospital, Washington DC, DC; 4The George Washington Transplant Institute, Department of Surgery, The George Washington University Hospital, Washington, DC Introduction: Non-islet cell tumor hypoglycemia (NICTH) is a paraneoplastic syndrome characterized by hypoglycemia from excessive production of insulin-like growth factor-2 (IGF-2) by tumors. We present a case of a 31-year-old female who was found to be severely and persistently hypoglycemic secondary to a liver mass.
Case Description/
Methods: A 31-year-old female with a history of endometrial stromal sarcoma status post total hysterectomy with bilateral salpingectomy 5 years ago, followed by chemotherapy, presented with a week-long history of lethargy, dizziness, and recurrent falls. Her blood glucose ranged between 26-28 mg/dl. Despite receiving multiple doses of IV dextrose in the emergency room, she was persistently hypoglycemic, which necessitated a transfer to the ICU for management of refractory hypoglycemia. She was started on a dextrose drip, and workup revealed low insulin and C-peptide levels (0.4 mc Intl units/ml and 0.1 ng/ml), beta-hydroxybutyrate of 0.7 mg/dl, normal IGF-2 level of 515 ng/ml. ACTH stimulation test was normal, and sulphonylurea levels were negative. The patient remained hypoglycemic despite continuous replacement. MRI abdomen/pelvis revealed a 15 cm right hepatic mass, raising a concern for a tumoral production of an insulin-like factor or a glucose-consumptive tumor with reduced hepatic gluconeogenesis contributing to her presentation. A right hepatic lobe resection was done with a dramatic increase in blood glucose level to normal limits. Pathology examination showed high-grade sarcoma positive for CD56, CyclinD1, CD99, and BCOR, consistent with metastatic high-grade endometrial stromal sarcoma. Discussion: NICTH diagnosis can be confirmed by an elevated IGF2 level or IGF-2/IGF-1 ratio. However, in critically ill patients in the setting of an underlying tumor, the mechanism of hypoglycemia could be explained by the extensive tumor burden in the liver. The presence of hypoinsulinemic hypoglycemia with low C-peptide levels can support this conclusion. Although the IGF2 level was normal and IGF-2/IGF-1 ratio was not documented, the improvement in her clinical status and biochemical profile with tumor resection and after exclusion of other causes of hypoglycemia support the diagnosis of NICTH. This case highlights a unique presentation of recurrent high-grade endometrial stromal sarcoma and emphasizes the importance of post-treatment cancer surveillance.
Figure: (1a) MRI abdomen with and without contrast showed 12.8*10.1*15.2 cm T2 hyperintense T1 hypointense mass with central stellate T1 hyperintense/T2 hypointense structures and arterial hyperenhancement with persistent hyperenhancement on more delayed sequences. (1b) Gross picture of right hepatic lobe mass post-resection.
Figure: Representative images of the metastatic tumor to the liver showing an H&E-stained section of a poorly differentiated high-grade sarcoma (left) with positive nuclear staining for BCOR (right). H&E (left) and immunohistochemical staining (right), 20X microscope objective.
Disclosures: Mohamed Abdou indicated no relevant financial relationships. Abdelrhman Refaey indicated no relevant financial relationships. Ahmed Attia indicated no relevant financial relationships. Ahmed Ebeid indicated no relevant financial relationships. Mamoun Younes indicated no relevant financial relationships. Ameer Abutaleb indicated no relevant financial relationships.
Mohamed Abdou, MD1, Abdelrhman Refaey, MD2, Ahmed Attia, MD1, Ahmed Ebeid, MD1, Mamoun Younes, MD3, Ameer Abutaleb, MD4. P3866 - Refractory Hypoglycemia in a Young Female With a Large Hepatic Mass: A Case of Non-Islet Cell Tumor Hypoglycemia, ACG 2025 Annual Scientific Meeting Abstracts. Phoenix, AZ: American College of Gastroenterology.
