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.
Pheochromocytomas are rare neuroendocrine tumors that frequently have genetic involvement and can present a particular risk in terms of the anesthetic-surgical procedure. Current worldwide medical recommendations state that laparoscopic adrenalectomy is the conventional treatment for pheochromocytoma, however, the ideal surgical strategy is still being developed as specialized surgeons gain experience with these rare tumors. The study's purpose was to thoroughly evaluate our film library and identify the best surgical steps that represent our standardized procedure. We developed a comprehensive set of surgical critical strategies for either a left or right adrenalectomy, providing helpful advice on patient positioning, port placement, the adrenal gland approach, as well as other surgical methods specifically designed for tumor mobilization and dissection. The current paper aims to synthesize our clinical experience in terms of preoperative preparation, patient positioning, intraoperative complications, causes of conversion, and specimen extraction. The main conclusions concern both the best surgical approach and the importance of surgical experience in the clinical management of these rare tumor types.
