Distinct clinical characteristics and prognosis of pediatric-onset growth hormone-secreting pituitary adenoma (GHPA) patients compared to adult-onset patients.

IF 3.7 3区 医学 Q2 Medicine
Xue-Qing Zheng, Sheng-Min Yang, Tong-Xin Xiao, Ying-Ying Yang, Yu-Xin Sun, Ran Li, Jie Liu, Huai-Gu Huang, Yong Yao, Kan Deng, Xin Lian, Hui You, Lian Duan, Hui-Juan Zhu
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Abstract

Purpose: To explore the clinical characteristics, treatment, and prognosis of growth hormone-secreting pituitary adenoma (GHPA) patients with pediatric-onset, so as to facilitate clinical management.

Methods: A retrospective cohort study was carried out between 102 pediatric-onset GHPA patients admitted to our hospital from January 2013 to June 2022 and 204 adult-onset GHPA patients who were randomly matched.

Results: GHPA with pediatric-onset was predominantly male, associated with higher proportion of genetic syndromes, longer course, and delayed diagnosis. Clinical symptoms of visual field defects and menstrual abnormality were more common. The pediatric-onset group exhibited higher growth hormone (GH) nadir during oral glucose tolerance test (OGTT), higher rates of hyperprolactinemia, larger maximum diameter of adenoma, and higher rates of optic chiasm compression, suprasellar invasion, and pituitary apoplexy. Hypertension, diabetes, and obstructive sleep apnea-hypopnea syndrome (OSAHS) were more common in the adult-onset group. Echocardiography results were similar between the two groups. The pediatric-onset group owned significantly higher treatment scores and proportions of multimodal therapy modality, more surgical complications, and a higher proportion of ki67 ≥ 3%. There was no significant difference in the final cure rate, but male patients with adult-onset had a worse prognosis. The recurrence rate was also similar between two groups. Hypopituitarism was more prevalent in the pediatric-onset group, while the adult-onset group had a higher incidence of other tumors.

Conclusion: Pediatric-onset GHPA patients exhibit distinct clinical characteristics compared to adult-onset patients. Multimodal therapy modalities could help to achieve a cure rate comparable to that of adult-onset patients.

小儿生长激素分泌型垂体腺瘤(GHPA)患者的临床特征和预后与成人患者不同。
目的:探讨儿童型生长激素分泌性垂体腺瘤(GHPA)患者的临床特点、治疗方法及预后,以促进临床治疗:方法:对我院2013年1月至2022年6月收治的102例小儿型GHPA患者和随机匹配的204例成人型GHPA患者进行回顾性队列研究:结果:儿童型GHPA患者以男性为主,遗传综合征比例较高,病程较长,诊断延迟。视野缺损和月经异常的临床症状更为常见。小儿发病组在口服葡萄糖耐量试验(OGTT)中表现出更高的生长激素(GH)最低值,更高的高泌乳素血症发生率,腺瘤最大直径更大,视交叉受压、星状上侵犯和垂体中风的发生率更高。高血压、糖尿病和阻塞性睡眠呼吸暂停-低通气综合征(OSAHS)在成人发病组中更为常见。两组患者的超声心动图结果相似。儿童发病组的治疗评分和多模式治疗比例明显更高,手术并发症更多,ki67≥3%的比例更高。最终治愈率无明显差异,但成年男性患者的预后较差。两组患者的复发率也相似。儿童发病组的垂体功能减退症发病率更高,而成人发病组的其他肿瘤发病率更高:结论:与成人发病者相比,儿童发病型GHPA患者表现出不同的临床特征。结论:与成人发病型患者相比,小儿发病型GHPA患者表现出不同的临床特征,多模式治疗有助于实现与成人发病型患者相当的治愈率。
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来源期刊
Endocrine
Endocrine 医学-内分泌学与代谢
CiteScore
6.40
自引率
5.40%
发文量
0
期刊介绍: Well-established as a major journal in today’s rapidly advancing experimental and clinical research areas, Endocrine publishes original articles devoted to basic (including molecular, cellular and physiological studies), translational and clinical research in all the different fields of endocrinology and metabolism. Articles will be accepted based on peer-reviews, priority, and editorial decision. Invited reviews, mini-reviews and viewpoints on relevant pathophysiological and clinical topics, as well as Editorials on articles appearing in the Journal, are published. Unsolicited Editorials will be evaluated by the editorial team. Outcomes of scientific meetings, as well as guidelines and position statements, may be submitted. The Journal also considers special feature articles in the field of endocrine genetics and epigenetics, as well as articles devoted to novel methods and techniques in endocrinology. Endocrine covers controversial, clinical endocrine issues. Meta-analyses on endocrine and metabolic topics are also accepted. Descriptions of single clinical cases and/or small patients studies are not published unless of exceptional interest. However, reports of novel imaging studies and endocrine side effects in single patients may be considered. Research letters and letters to the editor related or unrelated to recently published articles can be submitted. Endocrine covers leading topics in endocrinology such as neuroendocrinology, pituitary and hypothalamic peptides, thyroid physiological and clinical aspects, bone and mineral metabolism and osteoporosis, obesity, lipid and energy metabolism and food intake control, insulin, Type 1 and Type 2 diabetes, hormones of male and female reproduction, adrenal diseases pediatric and geriatric endocrinology, endocrine hypertension and endocrine oncology.
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