A RARE ASSOCIATION OF ORGAN IMPLICATION FOR MULTIPLE ENDOCRINE NEOPLASIA TYPE 1.

Introduction: Multiple endocrine neoplasia type 1 (MEN1), an autosomal-dominantly inherited tumor syndrome, is classically defined by tumors arising from the "3 Ps": Parathyroids, Pituitary, and the endocrine Pancreas. The majority (>95%) of MEN1 patients will develop parathyroid tumors, 35% to 75% of patients will develop neuroendocrine tumors of the pancreas, <30% will have pituitary adenomas, and ... -- will develop other endocrine tumors such as adrenal adenomas and carcinomas. This case study seeks to emphasize the significance of accurately determining the underlying cause of basic signs such as hypoglycemia and conducting an in-depth examination of the patient to detect potential correlations between latent conditions. Case Report: This is the case of a 42-year-old female patient who presented at Akdeniz Hospital complaining of fatigue and hyperphagia. The patient's past medical history shows that she had surgical excision of an insulinoma in 2007 and had experienced weakness, fainting, and polyfagia over the previous three years. Blood testing showed low vitamin D levels, high calcium and PTH levels, and low glucose levels linked to high insulin and C-peptide levels. Computer tomography and parathyroid SPECT confirmed the results, which point to endocrine pancreas and parathyroid dysfunction. The patient was admitted for further investigations, including genetic testing for MEN1 syndrome. Discussions : Four smooth-bordered focal lesions were identified on the CT scan of the pancreatic tail section, the largest measuring 29x24 mm in size, compatible with Insulinoma. Additionally seen on the CT scan are bilateral nodular lesions on the adrenal glands, which raise the suspicion of pheochromocytoma. This led to the testing of catecholamine metabolites, which exhibited normal values for normetanephrine (37, 1 ug/24 h) and metanephrine (12, 7 ug/24 h). The Parathyroid SPECT detected lesions in the inferior commissure of both thyroid lobes, through an increased MIBI uptake in a hypo-isoechoic and heterogeneous area on the ultrasonography scan. A DEXA scan was conducted to assess bone density in the hip and lumbar spine due to the discovery of parathyroid dysfunction; both findings were conclusive for osteoporosis with a T-score of less than -2.5. Conclusions: The patient appears to have all the signs and symptoms of an uncommon case of MEN1 syndrome, which includes adrenal adenomas, pancreatic insulinoma, and parathyroid dysfunction. MEN1 is a complex disorder predisposing to more than 20 benign and malignant endocrine and nonendocrine neoplasms, still incompletely understood. [ABSTRACT FROM AUTHOR]