Endocrine and Diabetic Unit, Lady Ridgeway Hospital for Children, Colombo, Sri Lanka.
Pathology Unit, Lady Ridgeway Hospital for Children, Colombo, Sri Lanka.
J Med Case Rep. 2023 Apr 13;17(1):137. doi: 10.1186/s13256-023-03890-5.
Pediatric adrenocortical tumors include both benign adenomas and highly virulent malignant tumors. However, they are very rare among children. The aim of this study is to evaluate the clinicopathological data of children presenting with adrenocortical tumors and assess their survival in a South Asian population.
This is a retrospective cohort study that includes patients diagnosed with adrenocortical tumors from August 2020 to August 2022 followed-up at Lady Ridgeway Hospital. Seven children were diagnosed with adrenal cortical tumors. Their ages ranged from 10 months to 6.5 years. Five of them were boys. All displayed signs of peripheral precocious puberty. One boy phenotypically had features of Beckwith-Wiedemann syndrome. The median time for diagnosis after the onset of symptoms was 4.4 months. The preoperative diagnosis was based on clinical manifestations, elevated dehydroepiandrosterone sulfate levels, and suprarenal masses on computed tomography. All five boys had right-sided suprarenal masses, while the two girls had them on the left side. All underwent surgery for tumor resection. The diagnosis was confirmed based on the histopathology of the adrenal specimens. Four children had a Wieneke score of 4 or more, suggesting the possibility of adrenocortical carcinoma; however, only two of them behaved as malignant tumors. To date, two children have developed local recurrences within a very short period.
Adrenocortical tumors are uncommon in children, and treatment options are limited. To identify early recurrences, routine clinical, radiological, and biochemical examinations at least once every 6-8 weeks is important.
儿科肾上腺皮质肿瘤包括良性腺瘤和高度恶性的肿瘤。然而，它们在儿童中非常罕见。本研究旨在评估南亚人群中患有肾上腺皮质肿瘤的儿童的临床病理数据，并评估他们的生存情况。
这是一项回顾性队列研究，纳入了 2020 年 8 月至 2022 年 8 月在 Lady Ridgeway 医院就诊的被诊断为肾上腺皮质肿瘤的患儿。共 7 例患儿被诊断为肾上腺皮质肿瘤。他们的年龄从 10 个月到 6.5 岁不等。其中 5 例为男性。所有患儿均表现出外周性早熟的迹象。一名男孩表型上具有 Beckwith-Wiedemann 综合征的特征。症状出现后确诊的中位时间为 4.4 个月。术前诊断基于临床表现、脱氢表雄酮硫酸酯水平升高和计算机断层扫描发现的肾上腺肿块。所有 5 名男孩均有右侧肾上腺肿块，而 2 名女孩则有左侧肿块。所有患儿均行肿瘤切除术。根据肾上腺标本的组织病理学诊断确诊。4 名儿童的 Wieneke 评分在 4 分或以上，提示有肾上腺皮质癌的可能；然而，只有其中 2 名表现为恶性肿瘤。迄今为止，2 名患儿在很短的时间内发生了局部复发。
肾上腺皮质肿瘤在儿童中并不常见，治疗选择有限。为了早期发现复发，重要的是至少每 6-8 周进行一次常规的临床、放射学和生化检查。
