Kuhlen Michaela, Wudy Stefan A, Baumann Clara, Vokuhl Christian, Hartmann Michaela F, Kunstreich Marina, Claus Rainer, Redlich Antje
Pediatrics and Adolescent Medicine, Faculty of Medicine, 531257 University of Augsburg , Augsburg, Germany.
Bavarian Cancer Research Center (BZKF), Augsburg, Germany.
J Pediatr Endocrinol Metab. 2025 Jun 30;38(9):931-938. doi: 10.1515/jpem-2025-0198. Print 2025 Sep 25.
To explore the clinical spectrum and symptom duration in pediatric adrenocortical tumors (pACTs), with a focus on identifying cases that may reflect gradual tumor progression.
We retrospectively analyzed data from 110 pediatric patients with pACTs enrolled in the German Pediatric Oncology Hematology-Malignant Endocrine Tumor (GPOH-MET) studies (1997-2022). Endocrine symptom duration, histopathological classification, and clinical outcomes were assessed. Patients with symptom durations ≥2 standard deviations (SDs) from the mean were defined as outliers and evaluated for potential progression.
The cohort included 31 patients with adrenocortical adenomas (ACAs), 12 with tumors of uncertain malignant potential (ACx), and 67 with adrenocortical carcinomas (ACCs). Seven patients (6.4 %) showed markedly prolonged symptom duration, including four with ACC. One representative case demonstrated a nearly 5-year course from initial androgen excess to metastatic ACC, with evolving biochemical features and a diagnostic urinary steroid profile indicative of adrenal tumor activity.
A small subset of pACTs may present with prolonged endocrine symptoms, possibly reflecting gradual tumor evolution. While molecular validation is lacking, these findings support the need for early recognition and further research into the natural history of pACTs.
探讨儿童肾上腺皮质肿瘤（pACTs）的临床谱和症状持续时间，重点是识别可能反映肿瘤逐渐进展的病例。
我们回顾性分析了110例纳入德国儿科肿瘤血液学 - 恶性内分泌肿瘤（GPOH - MET）研究（1997 - 2022年）的儿童pACTs患者的数据。评估内分泌症状持续时间、组织病理学分类和临床结局。症状持续时间≥平均水平2个标准差（SDs）的患者被定义为异常值，并评估其潜在进展情况。
该队列包括31例肾上腺皮质腺瘤（ACAs）患者、12例恶性潜能不确定肿瘤（ACx）患者和67例肾上腺皮质癌（ACCs）患者。7例患者（6.4%）症状持续时间明显延长，其中4例为ACC。1例代表性病例展示了从最初雄激素过多到转移性ACC的近5年病程，伴有不断演变的生化特征以及诊断性尿类固醇谱，提示肾上腺肿瘤活动。
一小部分pACTs可能表现为内分泌症状延长，这可能反映肿瘤逐渐演变。虽然缺乏分子验证，但这些发现支持早期识别以及对pACTs自然史进行进一步研究的必要性。
