Li Jing, Zhang Weiling, Hu Huimin, Zhang Yi, Wen Yuan, Huang Dongsheng
Department of Pediatrics, Beijing Tongren Hospital of China Capital Medical University, Beijing, 100176, People's Republic of China.
Cancer Manag Res. 2021 Feb 11;13:1307-1314. doi: 10.2147/CMAR.S289191. eCollection 2021.
This study aimed to summarize the clinical characteristics, comprehensive treatment, and prognosis of adrenocortical carcinoma (ACC) in children.
The clinical data of eight children with definite diagnoses of ACC were retrospectively analyzed, and statistical methods were used to analyze the clinical characteristics, comprehensive treatment mode, and prognosis of these patients.
(1) Clinical characteristics: two were males and six were females with the median age of onset was six-years old were involved. Four patients had a rash and precocious puberty as the symptoms of onset. European Network for the Study of Adrenal Tumors (ENSAT) staging: stage II, two patients; stage IV, six patients. (2) Comprehensive treatment: all eight patients underwent surgical treatment and received six cycles of chemotherapy: the regimen was "etoposide + pirarubicin + cisplatin + mitotane." (3) Prognosis analysis: among these eight patients, two patients died, two patients achieved complete remission, the disease was stable in four patients, and the overall five-year survival rate was 75%. Prognosis analyzed according to ENSAT staging (stage II versus stage IV) revealed that two-year survival rates of the two groups were 100% versus 65%, respectively, without statistical significant ( = 1.066, P = 0.302). Prognosis analyzed according to Weiss score (Weiss score was <6, five patients;≥6, three patients) revealed That survival time of the two groups was 50±9.52 months versus 6±1.70 months, the two-year survival rates of the two groups were 100% versus 35%, and the difference in survival rates between these two groups was statistically significant (χ = 4.091, P = 0.043).
The Weiss score is an important prognostic factor for ACC. The chemotherapy regimen "mitotane + etoposide + adriamycin + cisplatin" is recommended.
本研究旨在总结儿童肾上腺皮质癌（ACC）的临床特征、综合治疗及预后情况。
回顾性分析8例确诊为ACC的儿童临床资料，采用统计学方法分析这些患者的临床特征、综合治疗方式及预后。
（1）临床特征：男性2例，女性6例，中位发病年龄为6岁。4例患者以皮疹和性早熟为首发症状。欧洲肾上腺肿瘤研究网络（ENSAT）分期：Ⅱ期2例；Ⅳ期6例。（2）综合治疗：8例患者均接受了手术治疗，并接受6周期化疗：方案为“依托泊苷+吡柔比星+顺铂+米托坦”。（3）预后分析：8例患者中，2例死亡，2例完全缓解，4例病情稳定，总体5年生存率为75%。根据ENSAT分期（Ⅱ期与Ⅳ期）分析预后显示，两组2年生存率分别为100%和65%，差异无统计学意义（χ² = 1.066，P = 0.302）。根据Weiss评分（Weiss评分<6分，5例；≥6分，3例）分析预后显示，两组生存时间分别为50±9.52个月和6±1.70个月，两组2年生存率分别为100%和35%，两组生存率差异有统计学意义（χ² = 4.091，P = 0.043）。
Weiss评分是ACC的重要预后因素。推荐化疗方案“米托坦+依托泊苷+阿霉素+顺铂”。
