Endocrine Abstracts

Introduction: Primary neuroendocrine tumors can develop in any organ of the digestive system, most commonly in the small intestine, pancreas, or appendix, followed by the stomach or rectum. Appendiceal neuroendocrine tumors are often discovered incidentally during an appendicular presentation or as part of a carcinoid syndrome, without symptoms of hypoglycemic crises. The association between hypoglycemia and appendicular neuroendocrine tumors remains a subject of debate.

Observation: A 19-year-old patient was admitted for recurrent hypoglycemia, with no specific medical history except for a diabetic mother on metformin and sulfonylureas for the past year. The disease history dates back to one month before admission when a severe hypoglycemic episode occurred with a recorded blood glucose level of 0.5 g/l, accompanied by adrenergic symptoms complicated by a convulsive crisis, which resolved with glucose administration and anticonvulsant therapy. The course was marked by successive hypoglycemic episodes associated with a Whipple triad. Throughout this period, the patient experienced weight loss of 2 kg in one month. Upon admission, the patient presented with a generalized convulsive crisis coinciding with a blood glucose level of 1.03 g/l, leading to status epilepticus and transfer to the intensive care unit. Brain CT and MRI were normal, hile an abdominal CT scan revealed a swollen appendix with a small amount of peritoneal fluid but without infiltration of adjacent fat or pancreatic lesions. An appendectomy was performed, and the pathology report indicated a grade 1 neuroendocrine tumor according to the WHO classification, measuring 2x4 mm, with immunohistochemical expression of chromogranin A and CD56, a Ki-67 proliferation index of 2%, and clear resection margins. Additionally, exploration of thyroid, renal, and hepatic function, cortisol levels returned normal. During post-appendectomy monitoring, the patient normalized blood glucose levels and did not experience further hypoglycemia. As part of the postoperative extension assessment, thoracic-abdominal-pelvic and pancreatic CT scans were performed, and urinary Chromogranin A and 5HIAA levels were negative. An octreoscan conducted 6 months after surgery revealed very moderate hyperfixation in the right iliac fossa.

Conclusion: This observation illustrates the possible endocrine effects of a non-pancreatic neuroendocrine tumor (NET), such as hypoglycemia, which has been described in only one case in the literature. Curative surgery appears to be an effective treatment for these localized tumors.