Abstract Clinical Case In 2015, a 35-year-old male was referred for preoperative evaluation of pituitary surgery after MRI revealed a 25×25×19 mm macroadenoma compressing the optic chiasm. Initial prolactin was ∼2000 µg/L and IGF-1 was markedly elevated (623 µg/L). During assessment, severe hypercalcemia (14 mg/dL) was noted with PTH also significantly elevated (up to 623 pg/mL). After correction, subtotal (3.5 gland) parathyroidectomy was performed, confirming multiglandular adenoma. Postoperatively, permanent hypoparathyroidism developed, requiring lifelong calcium and calcitriol. Despite residual macroadenoma, the patient declined pituitary surgery; cabergoline was selected, normalizing prolactin and IGF-1 with radiologic regression. The patient also initially refused thymic surgery despite a mediastinal mass, but underwent thymectomy in 2020. Histopathology revealed a well-differentiated thymic NET, Grade 2, Ki-67 3–4%, with pleural metastases. Laboratory results showed marked hypergastrinemia (417 ng/L), normal glucagon (173 pg/mL), elevated Chromogranin A (850 µg/L) and neuron-specific enolase. Ga-68 PET/CT demonstrated pancreatic, pleural, and mediastinal metastases. MEN1 genetic testing confirmed a pathogenic mutation. The patient underwent thymectomy and subtotal parathyroidectomy. Cabergoline achieved biochemical remission of pituitary disease. Sandostatin LAR (30 mg IM every 28 days) was initiated for hormonal and antiproliferative effects. Calcium and calcitriol continue for hypoparathyroidism. Multidisciplinary follow-up is ongoing. Conclusion This case illustrates an aggressive MEN1 variant with multihormonal secretion and metastatic thymic NET. Thymic involvement is rare but prognostically significant. Somatostatin analogs are valuable for hormonal and tumor control. In case of non-response, alternative systemic treatment options may also be used. Close, multidisciplinary surveillance is essential for high-risk MEN1 patients.
Mantar et al. (Thu,) studied this question.