Multiple endocrine neoplasias (MEN) represent a rare and intricate group of disorders with diverse endocrine and extra- endocrine manifestations. Among the five identified types of MEN syndromes, MEN 2 stands out due to its complexity, featuring medullary thyroid carcinoma (MTC), pheochromocytoma, and primary hyperparathyroidism (PHPT). MEN 2 is inherited in an autosomal dominant manner, with nearly a 100% lifetime risk of developing MTC, which can be fatal if not detected early. Comprehensive diagnostic protocols and timely interventions significantly mitigate morbidity. To guide surgical decisions, management strategies include regular imaging and biomarker monitoring (calcitonin, CEA, metanephrines/normetanephrines, and parathyroid hormone). Genetic screening of at-risk families and prophylactic thyroidectomy remain cornerstone preventive measures. Although tyrosine kinase inhibitors are generally reserved for specific cases of MTC and are not first-line therapies for MEN2-associated tumors, they hold the potential for mitigating tumor progression and recurrence despite the risk of endocrine dysfunction. This paper presents a narrative review focusing on multiple endocrine neoplasia type 2 (MEN2) syndrome, encompassing its genetic underpinnings, clinical presentations, diagnostic modalities, and therapeutic approaches. A total of 48 articles were included in this review.
