Department of Hypothalamic and Pituitary Surgery, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan.
Department of Pathology, Toranomon Hospital, Tokyo, Japan.
Pituitary. 2018 Feb;21(1):1-9. doi: 10.1007/s11102-017-0836-4.
Growth hormone (GH)-producing pituitary adenomas (PAs) in childhood or young adulthood are rare, and the details surrounding these tumors remain enigmatic. We present the clinical, pathological and genetic features of this disease.
We identified 25 patients aged 20 years or younger with GH-producing PAs who underwent surgery between 2003 and 2016 at Toranomon Hospital in Tokyo. We retrospectively reviewed the clinical data, treatment outcomes and pathological features of these patients to shed light on childhood acromegaly.
The cohort comprised 14 male and 11 female patients whose average age at the time of surgery was 17.3 years. Germline AIP mutations were present in 5 of 13 patients examined, and Carney complex was identified in 2 of 25 patients. The mean maximum tumor diameter was 26.7 mm, and total resection assessed during surgery was achieved in 17 patients. Based on their respective pathological findings, patients were divided into the following 4 groups: sparsely granulated adenomas (5), densely granulated (DG) adenomas (6), plurihormonal adenomas (9), and silent subtype 3 (SS3) adenomas (5). During the mean follow-up period of 50.3 months, complete endocrinological remission was achieved in 14 of 25 patients (56%) by surgery alone and in 19 patients (76%) after postoperative adjuvant therapy.
GH-producing PAs in young patients are intriguing and difficult to treat due to their distinct tumor characteristics, including a lower incidence of the DG subtype and a higher incidence of SS3 adenomas and genetic abnormalities. Therefore, multi-modal therapies are essential to achieve optimal clinical outcomes.
儿童或青年期生长激素（GH）分泌型垂体腺瘤（PA）较为罕见，其肿瘤特征仍不清楚。本研究旨在报道此类疾病的临床、病理和遗传学特征。
我们回顾性分析了 2003 年至 2016 年在东京 Toranomon 医院接受手术治疗的 25 例年龄 20 岁及以下的 GH 分泌型 PA 患者的临床资料、治疗效果和病理特征。
该队列包括 14 例男性和 11 例女性患者，手术时的平均年龄为 17.3 岁。在 13 例接受检查的患者中，有 5 例存在 AIP 种系突变，25 例患者中有 2 例患有卡尼综合征。平均最大肿瘤直径为 26.7mm，17 例患者在术中实现了全切除。根据各自的病理发现，患者被分为以下 4 组：稀疏颗粒腺瘤（5 例）、致密颗粒腺瘤（6 例）、多激素腺瘤（9 例）和静默型 3 型腺瘤（5 例）。在平均 50.3 个月的随访期间，14 例（56%）患者仅通过手术实现了完全内分泌缓解，19 例（76%）患者在术后辅助治疗后达到了完全缓解。
由于 GH 分泌型 PA 具有独特的肿瘤特征，包括 DG 型发生率较低、SS3 腺瘤和遗传异常发生率较高，因此，对于年轻患者，此类肿瘤具有挑战性且难以治疗。多模态治疗对于获得最佳临床结果至关重要。
