Le Bras Maelle, Leclerc Hélène, Rousseau Olivia, Goudet Pierre, Cuny Thomas, Castinetti Frederic, Bauters Catherine, Chanson Philippe, Tabarin Antoine, Gaujoux Sebastien, Christin-Maitre Sophie, Ruszniewski Philippe, Borson-Chazot Francoise, Guilhem Isabelle, Caron Philippe, Goichot Bernard, Beckers Albert, Delemer Brigitte, Raingeard Isabelle, Vergès Bruno, Smati Sarra, Wargny Matthieu, Cariou Bertrand, Hadjadj Samy
CHU de Nantes PHU2 Institut du Thorax et du Système Nerveux, Service d'Endocrinologie, Diabétologie et Nutrition, Nantes, France.
Centre Hospitalier de La Roche sur Yon, Department of Endocrinology and Diabetology, La Roche sur Yon, France.
Eur J Endocrinol. 2021 Nov 26;185(6):863-873. doi: 10.1530/EJE-21-0630.
Pituitary adenoma (PA) is one of the three major components of multiple endocrine neoplasia type 1 (MEN1). Recent studies have suggested that MEN1-associated PAs are less aggressive than initially estimated. We propose an analysis of the outcome of PAs with a standard of care treatment in a nationwide cohort of MEN1 patients.
Retrospective observational nationwide cohort study using the MEN1 patient registry from the French Group of Endocrine Tumours (GTE).
The GTE database population consists of 1435 patients with MEN1. This analysis focused on 551 patients recruited after 2000 with at least 3 years of follow-up. The study outcome was tumour progression of PA defined by an increase in Hardy classification (HC) during follow-up according to referring physician regular reports.
Among 551 MEN1 patients (index and related), 202 (36.7%) had PA, with 114 (56.4%) diagnosed by MEN1-related screening. PAs were defined according to HC as microadenoma (grade I) in 117 cases (57.9%), macroadenoma in 59 (29.2%) with 20 HC grade II and 39 HC grades III-IV and unspecified in 26 (12.8%). They were prolactinomas in 92 cases (45.5%) and non-secreting in 73 (36.1%). After a median follow-up of 3 years among the 137 patients with HC grades I-II, 4 patients (2.9%) presented tumour progression.
PAs in patients with MEN1 are less aggressive than previously thought. Tumour progression is rare with a standard of care monitoring and treatment, especially in related patients who mostly present non-secreting microadenoma. MRI monitoring for asymptomatic MEN1 patients should be reduced accordingly.
垂体腺瘤（PA）是1型多发性内分泌腺瘤病（MEN1）的三大主要组成部分之一。最近的研究表明，与MEN1相关的垂体腺瘤的侵袭性低于最初估计。我们建议在全国范围内的MEN1患者队列中，按照标准治疗方案分析垂体腺瘤的治疗结果。
使用法国内分泌肿瘤研究组（GTE）的MEN1患者登记系统进行全国性回顾性观察队列研究。
GTE数据库人群包括1435例MEN1患者。本分析聚焦于2000年后招募的、至少随访3年的551例患者。研究结果是根据转诊医生的定期报告，随访期间Hardy分级（HC）增加所定义的垂体腺瘤肿瘤进展。
在551例MEN1患者（索引病例及相关病例）中，202例（36.7%）患有垂体腺瘤，其中114例（56.4%）通过与MEN1相关的筛查确诊。根据HC，垂体腺瘤被定义为微腺瘤（I级）117例（57.9%），大腺瘤59例（29.2%），其中20例为HC II级，39例为HC III-IV级，26例（12.8%）未明确分级。92例（45.5%）为催乳素瘤，73例（36.1%）为无功能性垂体腺瘤。在137例HC I-II级患者中，中位随访3年后，4例（2.9%）出现肿瘤进展。
MEN1患者的垂体腺瘤侵袭性低于此前认为的情况。在标准的监测和治疗方案下，肿瘤进展很少见，尤其是在大多数为无功能性微腺瘤的相关患者中。因此，对于无症状的MEN1患者，应相应减少MRI监测。
