In 1953, for the first time, Paul Wermer described a family presenting endocrine gland neoplasms over several generations. The transmission was autosomal dominant and the penetrance was high. Forty years later in 1997, the multiple endocrine neoplasia type 1 (MEN1) gene was sequenced, thus enabling diagnosis and early optimal treatment. Patients carrying the MEN1 gene present endocrine but also non-endocrine tumors. Parathyroid, pancreatic and pituitary impairment are the three main types of endocrine involvement. The present article details therapeutic management of hyperparathyroidism, neuroendocrine pancreatic tumors and pituitary adenomas in patients carrying the MEN1 gene. Significant therapeutic progress has in fact been made in the last few years. As concerns the parathyroid glands, screening of family members and regular monitoring of affected subjects now raise the question of early management of parathyroid lesions and optimal timing of parathyroid surgery. As concerns the duodenum-pancreas, proton-pump inhibitors are able to control gastrin-secreting syndrome, reducing mortality in MEN1 patients. Mortality in MEN1 patients is no longer mainly secondary to uncontrolled hormonal secretion but to metastatic (mainly pancreatic) disease progression. Tumor risk requires regular monitoring of morphological assessment, leading to iterative pancreatic surgery in a large number of patients. Finally, pituitary adenomas in MEN1 patients are traditionally described as aggressive, invasive and resistant to medical treatment. However, regular pituitary screening showed them to be in fact infra-centimetric and non-secreting in the majority of patients. Consequently, it is necessary to regularly monitor MEN1 patients, with regular clinical, biological and morphological work-up. Several studies showed that this regular monitoring impairs quality of life. Building a relationship of trust between patients and care provider is therefore essential. It enables the patient to be referred for psychological or psychiatric care in difficult times, providing long-term support and preventing any breakdown in continuity of care.

1953 年，Paul Wermer 首次描述了一个家族出现了几代人的内分泌腺肿瘤。传播为常染色体显性遗传，外显率高。四十年后的 1997 年，对多发性内分泌肿瘤 1 型( MEN1 ) 基因进行了测序，从而实现了诊断和早期最佳治疗。携带MEN1基因的患者存在内分泌肿瘤，但也存在非内分泌肿瘤。甲状旁腺、胰腺和垂体受损是内分泌受累的三种主要类型。本文详细介绍了携带MEN1患者的甲状旁腺功能亢进、胰腺神经内分泌肿瘤和垂体腺瘤的治疗管理基因。事实上，在过去几年中，治疗取得了重大进展。就甲状旁腺而言，家庭成员的筛查和受影响受试者的定期监测现在提出了甲状旁腺病变的早期管理和甲状旁腺手术的最佳时机的问题。至于十二指肠胰腺，质子泵抑制剂能够控制胃泌素分泌综合征，降低 MEN1 患者的死亡率。MEN1 患者的死亡率不再主要继发于不受控制的激素分泌，而是继发于转移性（主要是胰腺）疾病进展。肿瘤风险需要定期监测形态学评估，导致大量患者进行反复胰腺手术。最后，MEN1 患者的垂体腺瘤传统上被描述为具有侵袭性，侵入性和抗药性。然而，定期垂体筛查显示它们在大多数患者中实际上是厘米以下且不分泌。因此，有必要定期监测 MEN1 患者，并定期进行临床、生物学和形态学检查。几项研究表明，这种定期监测会损害生活质量。因此，在患者和护理提供者之间建立信任关系至关重要。它使患者能够在困难时期被转介接受心理或精神科护理，提供长期支持并防止护理连续性出现任何故障。定期进行临床、生物学和形态学检查。几项研究表明，这种定期监测会损害生活质量。因此，在患者和护理提供者之间建立信任关系至关重要。它使患者能够在困难时期被转介接受心理或精神科护理，提供长期支持并防止护理连续性出现任何故障。定期进行临床、生物学和形态学检查。几项研究表明，这种定期监测会损害生活质量。因此，在患者和护理提供者之间建立信任关系至关重要。它使患者能够在困难时期被转介接受心理或精神科护理，提供长期支持并防止护理连续性出现任何故障。