Clear Cell Adenocarcinoma of the Uterine Cervix and Vagina in Children Without Exposure to Diethylstilbestrol.

AIM

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.

METHODS

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.

MAIN RESULTS

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.

CONCLUSION

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.