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Multiple Endocrine Neoplasia 1 in a Young Man With ...
Multiple Endocrine Neoplasia 1 in a Young Man With Seizures
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This case describes a 29-year-old man with a strong family history of MEN1 who presented with seizures and was later found to have classic manifestations of multiple endocrine neoplasia type 1. Initial labs showed hypercalcemia with elevated parathyroid hormone, consistent with primary hyperparathyroidism. Additional endocrine testing revealed elevated gastrin, chromogranin A, and pancreatic polypeptide levels, suggesting functional neuroendocrine tumors.<br /><br />Imaging demonstrated a pituitary microadenoma, multiple pancreatic lesions, and duodenal submucosal lesions. Endoscopic biopsy confirmed well-differentiated, low-grade neuroendocrine tumors in the duodenum and pancreas. A DOTATATE PET-CT showed multiple somatostatin receptor–avid pancreatic lesions, supporting multifocal neuroendocrine neoplasia.<br /><br />During hospitalization, the patient had several generalized tonic-clonic seizures, but EEG did not show evidence that the seizures were due to hypoglycemia or an insulin-secreting tumor. He was treated with antiepileptic medications and remained seizure-free after stabilization. Clinicians concluded that his epilepsy was likely unrelated to MEN1, since he was euglycemic with appropriately low insulin and C-peptide levels.<br /><br />For his hyperparathyroidism, he underwent outpatient subtotal parathyroidectomy with biochemical improvement. He was also started on lanreotide to help slow tumor growth, and further surgery for duodenal and pancreatic lesions was being considered. He continues to follow with endocrinology and gastroenterology for ongoing surveillance of calcium, PTH, prolactin, pituitary hormones, and repeat brain and gastrointestinal imaging.<br /><br />The authors emphasize that MEN1 should be suspected in patients with a compatible family history and endocrine abnormalities, and that diagnosis and management require multidisciplinary care.
Asset Subtitle
Nitya Batra
Meta Tag
Author List
Daniel Misra, Daniel Shelden, Nishant Aggarwal, Nitya Batra, Shailesh Niroula
Category
Clinical Vignettes
Concept
Multiple Endocrine Neoplasia Type 1
Concept
Primary Hyperparathyroidism
Concept
Pituitary Adenoma
Concept
Duodenopancreatic Neuroendocrine Tumor
Concept
Hypercalcemia
Distinguished
Non-Finalist
Presenter Organization
William Beaumont Hospital
Presenting Author
Nitya Batra
Track
Adult
Keywords
MEN1
multiple endocrine neoplasia type 1
primary hyperparathyroidism
neuroendocrine tumors
pituitary microadenoma
pancreatic lesions
duodenal lesions
hypercalcemia
lanreotide
subtotal parathyroidectomy
Multiple Endocrine Neoplasia Type 1
Primary Hyperparathyroidism
Pituitary Adenoma
Duodenopancreatic Neuroendocrine Tumor
Hypercalcemia
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