Complexities of managing refractory hypoglycemia in a rare case of metastatic grade 2 pancreatic neuroendocrine tumor.

Abstract

e16361 Background: Pancreatic neuroendocrine tumors (pNETs) are often classified based on their ability to secrete hormones. While Insulinomas are the most common functional PNETs, malignant insulinomas are exceedingly rare accounting for only 10% of all insulinomas. we present a rare case of aggressive pancreatic neuroendocrine tumor grade 2 in a 63-year-old non-diabetic male who presented with recurrent hypoglycemic episodes despite optimized medical management including dextrose infusion, diazoxide, octreotide, and steroids, continues to experience persistent hypoglycemia. This article highlights the complexities of managing refractory hypoglycemia in the context of widespread metastatic disease and emphasizes the urgent need for effective therapeutic strategies to improve patient outcomes. Methods: Patient is a 63-year-old male with no significant past medical history presented to the ER for evaluation of hypoglycemia. he has no prior history of diabetes and admits to several similar episodes of hypoglycemia. his blood glucose was 31, which required several amps of D50. he had refractory hypoglycemia necessitating steady IV infusion of D10W. Labs were pertinent for POC glucose 31, AST 199, ALT 335, ALP 350, A1C 4.5. he had elevated c-peptide of 13.8 and free insulin 73. Ct scan abd / pelvis was consistent with Pancreatic-tail malignancy measuring approximately 3 cm with extensive/innumerable hepatic metastases as well as Area of hypo-enhancement in the spleen measuring 5.5 cm, with direct tumor invasion from the pancreatic tail tumor. Liver fine needle aspirate cytology was performed and was positive for neoplasia with well differentiated neuroendocrine tumor grade 2 consistent with pancreatic primary. Nest uniform cells positive for cytokeratin, CA19-9, Chromogranin, CD56 and Synaptophysin, KI67 Positive in 3-5 % of cells. Results: Despite maximal dose of diazoxide, he continued to have frequent hypoglycemia and was dependent on steady D10W IV infusions. Discharge home seemed impossible due to frequent hypoglycemic attacks and unfortunately, he was not a surgical candidates given extensive tumor burden and metastatic disease. He was eventually diagnosed as Stage IV (cT4 cNx pM1) well-differentiated neuroendocrine tumor, grade 2 of the pancreas with multiple hepatic metastases. Treatment is palliative, not curative, with a goal to control tumor growth, alleviate cancer-related symptoms, improve quality of life, and extend life. treatment options which include but not limited to lanreotide/octreotide LAR shots or everolimus. Conclusions: Ultimately, the case emphasizes the importance of a multidisciplinary approach to optimize outcomes for patients with stage 4 pNETs. Further research is needed to expand the therapeutic arsenal for these rare and aggressive tumors, aiming to improve survival and quality of life for affected individuals.