MEN-1 Management in Clinical Practice: Navigating Complexity Through Multidisciplinary Collaboration

Multiple endocrine neoplasia type 1 (MEN-1) is a rare hereditary disorder characterized by the development of multifocal tumors in endocrine organs. This article presents a clinical case of a female patient with a confirmed MEN1 mutation (c.203delC p.T70fsX118), who exhibited manifestations of various syndrome components over more than 10 years, including parathyroid adenomas, pancreatic neuroendocrine tumors, and a pituitary microprolactinoma. The patient underwent multiple surgical interventions: duodenopancreatectomy, parathyroidectomy with autotransplantation, adrenal resection, and esophageal leiomyoma removal. The study discusses the challenges in diagnosing the primary source of parathyroid hormone secretion, the difficulties in achieving optimal glycemic control due to absolute insulin deficiency following fulminant diabetes mellitus development after total pancreatectomy, as well as aspects of reproductive health and quality of life. This case highlights the importance of a personalized approach and multidisciplinary management in patients with MEN-1 syndrome.

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