Department of Paediatrics and Child Health, Haematology-Oncology Service, Red Cross War Memorial Children's Hospital.
Division of Epidemiology and Biostatistics, School of Public Health and Family Medicine, University of Cape Town.
J Pediatr Hematol Oncol. 2021 Jul 1;43(5):e619-e624. doi: 10.1097/MPH.0000000000002076.
Pediatric sex cord stromal tumors (SCSTs) are extremely rare and there are no reported data from Africa. The authors evaluated the outcomes of children and adolescents with biopsy-proven SCSTs in preparation for the introduction of a national protocol.
Retrospective data were collated from 9 South African pediatric oncology units from January 1990 to December 2015. Kaplan-Meier analysis was performed to estimate overall survival (OS) and event-free survival.
Twenty-three patients were diagnosed with SCSTs, 3 male and 20 female individuals, during the study period. Histologies included 1 thecoma, 9 Sertoli-Leydig cell tumors, and 13 juvenile granulosa cell tumors. Stage I tumors predominated (n=14; 60.9%), with 2 stage II (8.7%), 5 stage III (21.7%), and 2 stage IV tumors (8.7%). The upfront resection rate was 91.3% with no reported surgical morbidity or mortality and an OS of 82.1%. Chemotherapy approaches were not standardized. Most children (81.8%), except 2, had recognized platinum-based regimens. Chemotherapy-related toxicity was minimal and acceptable. Assessment of glomerular filtration rate and audiology assessments were infrequent and not standardized. Three patients were lost to follow-up.
Although the numbers in this cohort are small, this study represents the first national cohort in Africa. The 5-year OS of 82.1% was encouraging. Standardized management of rare tumors like SCSTs is critical to improve ensure OS and address potential long-term sequelae.
儿科性索间质肿瘤（SCST）极为罕见，目前尚无来自非洲的报道数据。作者评估了经活检证实的 SCST 患儿和青少年的结局，为引入国家方案做准备。
本研究回顾性分析了 1990 年 1 月至 2015 年 12 月期间来自南非 9 个儿科肿瘤单位的患者数据。采用 Kaplan-Meier 分析评估总生存率（OS）和无事件生存率。
研究期间共诊断出 23 例 SCST 患儿，男 3 例，女 20 例。组织学类型包括 1 例细胞瘤、9 例 Sertoli-Leydig 细胞瘤和 13 例幼年型颗粒细胞瘤。Ⅰ期肿瘤占多数（n=14；60.9%），Ⅱ期 2 例（8.7%），Ⅲ期 5 例（21.7%），Ⅳ期 2 例（8.7%）。初次手术切除率为 91.3%，无手术相关发病率或死亡率，OS 为 82.1%。化疗方案未标准化。大多数患儿（81.8%），除 2 例外，均接受了公认的含铂方案。化疗相关毒性较小，可接受。肾小球滤过率评估和听力评估不频繁且未标准化。3 例患儿失访。
尽管本队列中的病例数较少，但这是非洲首例全国性队列研究。5 年 OS 为 82.1%，结果令人鼓舞。对 SCST 等罕见肿瘤进行标准化管理对于提高 OS 和解决潜在长期后遗症至关重要。
