催乳素瘤是临床实践中最常见的垂体腺瘤类型。像卡伯吉林这样的多巴胺受体激动剂（DA）通常能够为大多数患者提供症状控制、使催乳素水平正常化并减小肿瘤大小。DA耐药型催乳素瘤相对较少见，可能与MEN1等一些遗传原因和AIP基因（AIPvar）的致病性生殖细胞突变有关。我们将AIPvar相关性催乳素瘤（n = 13）与未选择住院治疗的催乳素瘤（“未选择”，n = 41）和无遗传因素相关、DA耐药的催乳素瘤（DA耐药，n = 39）的临床、放射学和治疗特征进行了比较。与未选择或DA耐药的催乳素瘤相比，AIPvar相关性催乳素瘤的发病年龄显著较小（p <0.01）。在AIPvar中，男性的比例较高（75.0%），而在DA耐药和未选择的催乳素瘤中，男性的比例较低（分别为49.7%和9.8%；p <0.001）。与其他组相比，AIPvar催乳素瘤的肿瘤侵袭更为频繁（p <0.001）且呈现出更大的肿瘤直径趋势。与AIPvar组相比，DA耐药组在诊断时的催乳素水平显著较高（p <0.001）。AIPvar和DA耐药组的最大DA剂量显著高于未选择组。AIPvar组中DA诱导的巨腺瘤缩小（>50%）发生率为58.3%，而DA耐药组为4.2%（p <0.01）。相较于未选择组（19.5%：p <0.01），AIPvar和DA耐药组的手术频率更高（分别为43.8%和61.5%）。放射治疗仅在AIPvar（18.8%）和DA耐药（25.6%）组中应用。 AIPvar在催乳素瘤中表现出侵袭性的表型，且在较小的年龄就出现侵袭性肿瘤。这些特征有助于将罕见的AIPvar相关催乳素瘤与DA耐药和无遗传因素相关的肿瘤区分开来。 Copyright © 2023 Vroonen, Beckers, Camby, Cuny, Beckers, Jaffrain-Rea, Cogne, Naves, Ferriere, Romanet, Elenkova, Karhu, Brue, Barlier, Pétrossians and Daly.

Prolactinomas are the most frequent type of pituitary adenoma encountered in clinical practice. Dopamine agonists (DA) like cabergoline typically provide sign/ symptom control, normalize prolactin levels and decrease tumor size in most patients. DA-resistant prolactinomas are infrequent and can occur in association with some genetic causes like MEN1 and pathogenic germline variants in the AIP gene (AIPvar).We compared the clinical, radiological, and therapeutic characteristics of AIPvar-related prolactinomas (n=13) with unselected hospital-treated prolactinomas ("unselected", n=41) and genetically-negative, DA-resistant prolactinomas (DA-resistant, n=39).AIPvar-related prolactinomas occurred at a significantly younger age than the unselected or DA-resistant prolactinomas (p<0.01). Males were more common in the AIPvar (75.0%) and DA- resistant (49.7%) versus unselected prolactinomas (9.8%; p<0.001). AIPvar prolactinomas exhibited significantly more frequent invasion than the other groups (p<0.001) and exhibited a trend to larger tumor diameter. The DA-resistant group had significantly higher prolactin levels at diagnosis than the AIPvar group (p<0.001). Maximum DA doses were significantly higher in the AIPvar and DA-resistant groups versus unselected. DA-induced macroadenoma shrinkage (>50%) occurred in 58.3% in the AIPvar group versus 4.2% in the DA-resistant group (p<0.01). Surgery was more frequent in the AIPvar and DA- resistant groups (43.8% and 61.5%, respectively) versus unselected (19.5%: p<0.01). Radiotherapy was used only in AIPvar (18.8%) and DA-resistant (25.6%) groups.AIPvar confer an aggressive phenotype in prolactinomas, with invasive tumors occurring at a younger age. These characteristics can help differentiate rare AIPvar related prolactinomas from DA-resistant, genetically-negative tumors.Copyright © 2023 Vroonen, Beckers, Camby, Cuny, Beckers, Jaffrain-Rea, Cogne, Naves, Ferriere, Romanet, Elenkova, Karhu, Brue, Barlier, Pétrossians and Daly.