Endocrine Abstracts

Introduction: Glucagonoma is an extremely rare PNET which presenting symptoms can be varied but the most common include necrolytic migratory erythema, diabetes, diarrhea and weight loss. Treatment involves a multidisciplinary approach, as most of them are large and present with metastasis at diagnosis, and would need nutritional support, an expert management of pancreatic exocrine insuffiency and diabetes. The different therapeutic options could potentially also cause nutritional and metabolic disturbances that might need a close monitoring and constant adjusting. We present the case of a patient diagnosed with a stage IVB, glucagon producing GRADE 2 pNET who required a multidisciplinary approach to improve her overall survival and quality of life.

Case report: A 69 year old woman with a new diagnosis type 2 DM and no other previous relevant history, presented with chronic abdominal pain and weight loss. CT scan disclosed a 10×3 cm hypervascular mass in the pancreatic tail, with multiple hypervasular lesions in the liver. A 18FDG PET confirmed abnormal uptake (SUV 4,8) at the distal pancreas, correlating with the CT scan and the Octreo-scan as well (krening 4) Biopsy of the hepatic lesion confirmed metastatic grade 2 PNET. The laboratory values were normal except for an elevated serum glucagon level (830 pg/ml, Ref. 50–150), and increased levels of Cromogranin A 360 (0-108) and NSE 17,20 (0-16,3) accompanied by low levels of Cupper and Zinc. The patient was treated with octreotide lar, which lowered the serum glucagon levels by 73% for 10 months and the tumor size remained stable for 15 months of follow up, but soon after glucagon levels elevated and liver metastases where shown in the MRI. The patient underwent 19 cycles of Everolimus, until progression was assessed and treatment with ¹⁷⁷Lu-DOTATATE decided. 6 months after completing the treatment, glucagon levels had gone down by 50% and a 40% reduction in liver metastatic size and a 10% reduction in pancreatic tumor mass were shown by the MRI. The patient had not presented significant treatment-related toxicity and her insulin needs were cut by half. During the whole process the patient’s nutrtitional needs, gastric enzymes and insulin requirements were adjusted to ensure the best outcome and improve her quality of life.

Conclusions: Glucagonoma is difficult to diagnose and once diagnosed a challenge to manage. An interprofessional team must work together to monitor the patient as needs as they navigate through the wide range of treatment modalities and their side effects.