Clinical Characteristics and Long-Term Outcomes of Adrenal Tumors in Children and Adolescents.

IF 1.6 4区 医学 Q4 ENDOCRINOLOGY & METABOLISM
Ja Hye Kim, Yunha Choi, Soojin Hwang, Ji-Hee Yoon, Gu-Hwan Kim, Han-Wook Yoo, Jin-Ho Choi
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Abstract

Objective: Adrenal tumors are generally rare in children and can be a part of familial cancer syndrome. This research was conducted to examine the clinical outcomes, histopathological results, and genetic etiologies of adrenal tumors in children and adolescents.

Methods: Thirty-one children and adolescents with adrenal tumors were included. Data on clinical outcomes and endocrine and radiologic results were retrospectively analyzed. Molecular analysis was conducted in select patients according to their phenotype and family history.

Results: The median age at diagnosis was 7.9 years (range: 0.8-17.8 years) with 5.1±1.8 cm of maximum tumor diameter. Adrenal adenoma (n=7), carcinoma (n=5), borderline (n=2), isolated micronodular adrenocortical disease (n=2), pheochromocytoma (n=8), paraganglioma (n=3), and ganglioneuroma (n=4) are all pathological diagnoses. The most common presenting symptom was excess production of adrenocortical hormones (n=15), including virilization and Cushing syndrome. Non-functioning adrenocortical tumors were found in a patient with congenital adrenal hyperplasia. Genetic etiologies were identified in TP53 (n=5), VHL (n=4), and PRKACA (n=1). Patients with mutations in TP53 were young (1.5±0.5 years) and had large masses (6.1±2.3 cm).

Conclusions: This study describes clinical outcomes and the pathological spectrum of adrenal tumors in children and adolescents. Adrenocortical tumors mostly presented with an excess of the adrenocortical hormone. Patients with genetic defects presented at a young age and large size of tumors, necessitating genetic testing in patients at a young age.

儿童和青少年肾上腺肿瘤的临床特征和长期疗效。
目的:肾上腺肿瘤在儿童中普遍罕见,可能是家族性癌症综合征的一部分。本研究旨在检查儿童和青少年肾上腺肿瘤的临床结果、组织病理学结果和遗传病因。方法:纳入31例儿童和青少年肾上腺肿瘤患者。对临床结果、内分泌和放射学结果的数据进行回顾性分析。根据表型和家族史对选定的患者进行分子分析。结果:诊断时的中位年龄为7.9岁(范围:0.8-17.8岁),为5.1±1.8岁 肿瘤最大直径cm。肾上腺腺瘤(n=7)、癌(n=5)、交界性(n=2)、孤立性小结节性肾上腺皮质疾病(n=2、嗜铬细胞瘤(n=8)、副神经节瘤(n=3)和神经节神经瘤(n=4)均为病理诊断。最常见的症状是肾上腺皮质激素分泌过多(n=15),包括男性化和库欣综合征。在一名先天性肾上腺增生患者中发现了功能性肾上腺皮质肿瘤。在TP53(n=5)、VHL(n=4)和PRKACA(n=1)中确定了遗传病因。TP53突变的患者年龄较小(1.5±0.5岁),肿块较大(6.1±2.3 结论:本研究描述了儿童和青少年肾上腺肿瘤的临床结果和病理谱。肾上腺皮质肿瘤主要表现为肾上腺皮质激素过量。遗传缺陷患者在年轻时就出现,肿瘤体积大,因此需要在年轻时对患者进行基因检测。
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来源期刊
CiteScore
4.10
自引率
5.60%
发文量
72
审稿时长
3 months
期刊介绍: Publishing outstanding articles from all fields of endocrinology and diabetology, from molecular biology to clinical research, this journal is a brilliant resource. Since being published in English in 1983, the popularity of this journal has grown steadily, reflecting the importance of this publication within its field. Original contributions and short communications appear in each issue along with reviews addressing current topics. In addition, supplementary issues are published each year presenting abstracts or proceedings of national and international scientific meetings. The journal was initially published in German and is still the oldest endocrinological periodical in the German-language market!
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