Department of Pediatric Endocrinology, School of Medicine, Kocaeli University, Kocaeli, Turkey.
Department of Academic Writing, Kocaeli University, Kocaeli, Turkey.
Endocrine. 2024 Jan;83(1):160-170. doi: 10.1007/s12020-023-03523-3. Epub 2023 Oct 2.
Pediatric pituitary adenomas (PPA) are rare. Although PPAs are mostly benign, they can be challenging to manage. Most studies evaluating PPA are based on surgical series. We aimed to present the clinical features, hormonal status and treatment outcomes of children with PPA managed in a joint neuroendocrine setting.
In this single-center study, demographic, clinical and endocrinological data of patients under 19 years old who were followed up with the diagnosis of PPA between 2002-2022 were retrospectively reviewed. A total of 21 studies published in the past 20 years were also systematically reviewed.
There were 79 patients (52 girls, 27 boys) with a median age of 15.8 years. Median follow-up time was 30 months. The most common adenoma subtype was non-functioning adenoma (NFA) (35.5%), followed by prolactinoma (29.1%), corticotropinoma (21.5%), and somatotropinoma (13.9%), respectively. The frequency of micro and macroadenomas was almost equal while 38% of all adenomas were invasive. Headache, visual impairment and menstrual irregularity were the most common complaints, while the most common hormonal deficiency at diagnosis was central hypothyroidism (31.6%), followed by hypogonadotropic hypogonadism (22.7%), growth hormone deficiency (15.2%) and central adrenal insufficiency (11.4%), respectively. Fifty patients (63.2%) underwent endoscopic endonasal transsphenoidal surgery (EETS). Following the surgery, impaired endocrine functions recovered at a rate of 62% while permanent central diabetes insipidus was observed in 6%, and new onset hypopituitarism developed in 4%.
NFA was more common in this cohort than in previous reports, which is one of the largest PPA series in the literature. Hormonal disorders, which were common at the time of diagnosis, were largely resolved with appropriate endocrinological and surgical approaches, while the rate of pituitary hormonal deficiencies after EETS was relatively low. Therefore, we recommend that children with PPA be managed in the setting of a high-volume pituitary center to provide long-term low morbidity.
儿童垂体腺瘤(PPA)较为罕见。尽管 PPA 大多为良性,但治疗颇具挑战性。大多数评估 PPA 的研究都基于手术系列。我们旨在介绍在联合神经内分泌环境下管理的儿童 PPA 的临床特征、激素状态和治疗结果。
在这项单中心研究中,回顾了 2002 年至 2022 年间在诊断为 PPA 后接受随访的 19 岁以下患者的人口统计学、临床和内分泌数据。还系统地回顾了过去 20 年发表的 21 项研究。
共有 79 名患者(52 名女孩,27 名男孩),中位年龄为 15.8 岁。中位随访时间为 30 个月。最常见的腺瘤亚型是非功能性腺瘤(NFA)(35.5%),其次是催乳素瘤(29.1%)、促肾上腺皮质激素瘤(21.5%)和生长激素瘤(13.9%)。微腺瘤和大腺瘤的频率几乎相等,而所有腺瘤的 38%为侵袭性。头痛、视力障碍和月经不规律是最常见的症状,而诊断时最常见的激素缺乏是中枢性甲状腺功能减退症(31.6%),其次是促性腺激素低下性性腺功能减退症(22.7%)、生长激素缺乏症(15.2%)和中枢性肾上腺功能不全(11.4%)。50 名患者(63.2%)接受了内镜经鼻蝶窦手术(EETS)。手术后,受损的内分泌功能恢复率为 62%,而永久性中枢性尿崩症为 6%,新发垂体激素缺乏症为 4%。
与以往报告相比,该队列中 NFA 更为常见,这是文献中最大的 PPA 系列之一。在诊断时常见的激素紊乱通过适当的内分泌和手术方法得到了很大程度的解决,而 EETS 后垂体激素缺乏的发生率相对较低。因此,我们建议在高容量垂体中心管理 PPA 患儿,以提供长期低发病率。