Department of Endocrinology, Centre Hospitalier Universitaire de Liège, University of Liège, Liège, Belgium.
Department of Otorhinolaryngology, Centre Hospitalier Universitaire de Liège, University of Liège, Liège, Belgium.
Front Endocrinol (Lausanne). 2023 Aug 29;14:1242588. doi: 10.3389/fendo.2023.1242588. eCollection 2023.
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 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.
催乳素瘤是临床实践中最常见的垂体腺瘤类型。多巴胺激动剂（DA）如卡麦角林通常可提供症状/体征控制，使大多数患者的催乳素水平正常化并缩小肿瘤体积。DA 耐药性催乳素瘤并不常见，并且可能与一些遗传原因有关，如 MEN1 和 基因（AIPvar）中的致病性种系变异。
我们比较了 AIPvar 相关催乳素瘤（n=13）与未经选择的医院治疗催乳素瘤（“未经选择”，n=41）和遗传阴性、DA 耐药性催乳素瘤（DA-耐药，n=39）的临床、放射学和治疗特征。
AIPvar 相关催乳素瘤的发病年龄明显低于未经选择或 DA 耐药性催乳素瘤（p<0.01）。男性在 AIPvar（75.0%）和 DA-耐药（49.7%）中比未经选择的催乳素瘤（9.8%）更常见（p<0.001）。AIPvar 催乳素瘤的侵袭性明显高于其他组（p<0.001），且肿瘤直径较大。DA-耐药组在诊断时的催乳素水平明显高于 AIPvar 组（p<0.001）。AIPvar 和 DA-耐药组的最大 DA 剂量明显高于未经选择的组。AIPvar 组中有 58.3%的患者出现 DA 诱导的大腺瘤缩小（>50%），而 DA-耐药组中仅有 4.2%（p<0.01）。AIPvar 和 DA-耐药组的手术频率（分别为 43.8%和 61.5%）明显高于未经选择的组（19.5%：p<0.01）。仅在 AIPvar（18.8%）和 DA-耐药（25.6%）组中使用了放疗。
AIPvar 在催乳素瘤中表现出侵袭性表型，年轻患者发生侵袭性肿瘤。这些特征有助于将罕见的 AIPvar 相关催乳素瘤与 DA 耐药、遗传阴性的肿瘤区分开来。
