Kempf Julia, Schmitz Alexander, Meier Aline, Delfs Neele, Mueller Beat, Fandino Javier, Schuetz Philipp, Berkmann Sven
Division of Endocrinology, University Department of Medicine, Kantonsspital Aarau, Switzerland.
Department of Neurosurgery, Kantonsspital Aarau, Switzerland.
Swiss Med Wkly. 2018 Aug 22;148:w14653. doi: 10.4414/smw.2018.14653. eCollection 2018 Aug 13.
Acromegaly due to a growth hormone-secreting pituitary adenoma is a rare disease with high morbidity if not treated adequately. Using data of the Swiss Pituitary Registry (SwissPit), we studied initial presentation and predictors for adverse clinical outcomes in acromegalic patients treated during the last 10 years in our institution.
We evaluated 21 patients from the SwissPit registry with a final diagnosis of acromegaly confirmed by laboratory results (insulin-like growth factor-1 [IGF-1] and growth hormone suppression tests) and magnetic resonance imaging. Our main endpoint was clinical cure defined as complete remission, remission with need for medical treatment and uncontrolled disease defined by non-normalisation of IGF-1 and growth hormone levels.
The most prevalent clinical symptoms at presentation were acral enlargement (81%), headache (29%), macroglossia (29%) and visual field defects (19%). Arterial hypertension was present in 67%, carpal tunnel syndrome in 38% and diabetes in 24%. A total of 19 of the 21 patients underwent initial surgical treatment. Eight patients had complete remission and 13 patients had active disease, with 7 having remission with need for medical treatment and 6 uncontrolled disease. Larger initial adenoma size (odds ratio [OR] 12.0, 95% confidence interval [CI] 1.02-141.3; p = 0.048) and high post-operative IGF-1 levels (OR 4.5, 95% CI 1.1-19.2; p = 0.040) were predictors for non-full remission and uncontrolled disease, respectively.
This small, observational registry study showed a relevant success rate of initial pituitary surgery in patients with confirmed acromegaly. Initial tumour size and postoperative IGF-1 levels help to risk stratify patients regarding expected outcomes. In the case of disease persistence, a multimodal approach using drug and radiotherapy is mandatory.
由分泌生长激素的垂体腺瘤引起的肢端肥大症是一种罕见疾病，若治疗不当，发病率很高。利用瑞士垂体登记处（SwissPit）的数据，我们研究了过去10年在我们机构接受治疗的肢端肥大症患者的初始表现和不良临床结局的预测因素。
我们评估了SwissPit登记处的21例患者，其最终诊断为肢端肥大症，经实验室检查结果（胰岛素样生长因子-1[IGF-1]和生长激素抑制试验）及磁共振成像确诊。我们的主要终点是临床治愈，定义为完全缓解、缓解但需要药物治疗以及由IGF-1和生长激素水平未恢复正常定义的疾病未得到控制。
就诊时最常见的临床症状是手足增大（81%）、头痛（29%）、巨舌（29%）和视野缺损（19%）。67%的患者有动脉高血压，38%有腕管综合征，24%有糖尿病。21例患者中有19例接受了初始手术治疗。8例患者完全缓解，13例患者疾病活跃，其中7例缓解但需要药物治疗，6例疾病未得到控制。初始腺瘤较大（优势比[OR]12.0，95%置信区间[CI]1.02-141.3；p = 0.048）和术后IGF-1水平高（OR 4.5，95%CI 1.1-19.2；p = 0.040）分别是非完全缓解和疾病未得到控制的预测因素。
这项小型观察性登记研究表明，确诊肢端肥大症患者初始垂体手术有一定成功率。初始肿瘤大小和术后IGF-1水平有助于对患者预期结局进行风险分层。在疾病持续存在的情况下，必须采用药物和放疗的多模式方法。
