Braungart Sarah, Davenport Rachel
Department of Paediatric Surgery, Royal Hospital for Children Glasgow, Glasgow, UK.
School of Medicine, University of Glasgow, Glasgow, UK.
Pediatr Blood Cancer. 2025 Aug;72(8):e31786. doi: 10.1002/pbc.31786. Epub 2025 May 14.
Clear cell adenocarcinoma of the uterine cervix (CCAC) or vagina (CCAV) is rare and usually presents in postmenopausal women. Paediatric cases are rare, and have historically been associated with intrauterine exposure to diethylstilbestrol (DES). We aimed to summarise outcomes of CCAC and CCAV in children with no history of DES exposure.
Systematic review of the Pubmed/Medline/Ovid databases from inception to 2024 according to PRISMA guidelines. The initial search identified 127 articles, and 29 articles were included in the final analysis.
Forty-three cases of paediatric CCAC and CCAV were described. Median age at presentation was 10 years [interquartile range (IQR): 8-14 years]. Most patients presented with stage I tumours and symptoms of prolonged vaginal bleeding. Staging assessment included CT or MRI abdomen/pelvis and vaginoscopy with biopsy in most cases. Treatments consisted of variable combinations of chemotherapy, radiotherapy (external beam or brachytherapy) and surgery. Surgical procedures included localised resection only, radical trachelectomy or radical hysterectomy with pelvic lymph node clearance. Follow-up information was available for 88% patients and was overall very heterogeneous. Median duration of follow-up was 24 months [IQR: 14-82.5]. There were seven reported deaths, and two additional patients experienced recurrence during follow-up.
This is the first systematic review on the management and outcomes of children with CCAC and CCAV. The cases identified were few and heterogeneous, with limited information on longer term outcomes. Current evidence does not allow for the generation of paediatric-specific treatment guidelines. A cautious approach to the management of this rare and aggressive disease is essential, carefully balancing the desire of fertility preservation with the need for cure from disease.
子宫颈透明细胞腺癌（CCAC）或阴道透明细胞腺癌（CCAV）较为罕见，通常发生于绝经后女性。儿科病例罕见，既往一直与子宫内暴露于己烯雌酚（DES）有关。我们旨在总结无DES暴露史儿童的CCAC和CCAV的治疗结果。
根据PRISMA指南，对从数据库建立至2024年的Pubmed/Medline/Ovid数据库进行系统评价。初步检索识别出127篇文章，最终分析纳入29篇文章。
共描述了43例儿科CCAC和CCAV病例。就诊时的中位年龄为10岁[四分位间距（IQR）：8 - 14岁]。大多数患者表现为I期肿瘤及长期阴道出血症状。分期评估在大多数情况下包括腹部/盆腔CT或MRI以及阴道镜检查并活检。治疗包括化疗、放疗（外照射或近距离放疗）和手术的不同组合。手术操作包括仅局部切除、根治性宫颈切除术或根治性子宫切除术加盆腔淋巴结清扫。88%的患者有随访信息，总体差异很大。中位随访时间为24个月[IQR：14 - 82.5]。报告有7例死亡，另有2例患者在随访期间复发。
这是关于CCAC和CCAV患儿管理及治疗结果的首次系统评价。所识别的病例数量少且差异大，长期结果信息有限。目前的证据无法制定儿科特异性治疗指南。对于这种罕见且侵袭性疾病的管理，谨慎的方法至关重要，要仔细权衡保留生育能力的愿望与治愈疾病的需求。
