Zhang Ning, White Eleanor, Weir Tessa, Dexter Mark, Varikatt Winny, Glastras Sarah J
Endocrinol Diabetes Metab Case Rep. 2024 Dec 23;2024(4). doi: 10.1530/EDM-24-0033. Print 2024 Oct 1.
Paediatric pituitary adenomas are rare in children and adolescents and differ from adults in both clinical presentation and management. We present the case of a 14-year-old female with primary amenorrhoea secondary to a macroprolactinoma, showing a modest radiological and biochemical response to dopamine agonist (DA) therapy. Despite a 10-month duration of increasing DA therapy, initial symptoms of primary amenorrhoea and hyperprolactinaemia persisted, with new symptoms of weight gain, lethargy and low mood. A transsphenoidal resection of the macroprolactinoma was successfully performed, followed by the initiation of additional hormonal therapy. This case explores the unique challenges of treating a macroprolactinoma refractory to medical management in adolescence.
Management of macroprolactinomas in childhood and adolescence can bring unique challenges, including a delay in sexual development, often presenting with primary or secondary amenorrhoea in girls.DA therapy is typically the first-line therapy in treating macroprolactinomas; however, resistance in paediatric and adolescent patients is associated with tumour size and initial prolactin levels.Surgical resection should be considered as a second-line therapy for adolescents unable to tolerate high-dose DA therapy or have inadequate response to DA therapy.There are a range of potential surgical complications, including permanent or transient diabetes insipidus, meningitis, cerebrospinal fluid leakage and hypopituitarism.Timely management of macroprolactinomas is important for secondary sex characteristics, bone development and psychological well-being.
小儿垂体腺瘤在儿童和青少年中较为罕见，其临床表现和治疗方法与成人不同。我们报告一例14岁女性，因大泌乳素瘤继发原发性闭经，对多巴胺激动剂（DA）治疗有适度的影像学和生化反应。尽管增加DA治疗长达10个月，但原发性闭经和高泌乳素血症的初始症状持续存在，还出现了体重增加、嗜睡和情绪低落等新症状。成功实施了经蝶窦大泌乳素瘤切除术，随后开始了额外的激素治疗。本病例探讨了治疗青春期对药物治疗难治的大泌乳素瘤所面临的独特挑战。
儿童和青少年大泌乳素瘤的治疗可能带来独特挑战，包括性发育延迟，女孩常表现为原发性或继发性闭经。DA治疗通常是治疗大泌乳素瘤的一线疗法；然而，儿科和青少年患者的耐药性与肿瘤大小和初始泌乳素水平有关。对于无法耐受高剂量DA治疗或对DA治疗反应不佳的青少年，应考虑手术切除作为二线治疗。存在一系列潜在的手术并发症，包括永久性或暂时性尿崩症、脑膜炎、脑脊液漏和垂体功能减退。及时治疗大泌乳素瘤对第二性征、骨骼发育和心理健康很重要。
