Sarah Braungart

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

ABSTRACTAimClear 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.MethodsSystematic 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 ResultsForty‐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.ConclusionThis 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.

Reply to “Comment on: Standardizing the surgical management of benign ovarian tumours in children and adolescents: A best practice Delphi consensus statement”

Standardizing the surgical management of benign ovarian tumors in children and adolescents: A best practice Delphi consensus statement

AbstractAimNo widely agreed consensus protocols exist for the management of benign ovarian tumors (BOT) in children. This presents a substantial risk for suboptimal management. We aimed to generate multispecialty consensus guidance to standardize surgical management and provide a clear follow‐up protocol for children with BOTs.MethodsProspective two‐round confidential e‐Delphi consensus survey distributed among multispecialty expert panel; concluded by two semistructured videoconferences.Main resultsConsensus was generated on these core outcome sets: preoperative/intraoperative management; follow‐up; adolescent gynecology referral. (1) Children with BOTs should receive the same management as other patients with potentially neoplastic lesions: Preoperative discussion at a pediatric oncology multidisciplinary meeting to risk stratify tumors, and management by health professionals with expertise in ovarian‐sparing surgery and laparoscopy. (2) Ovarian‐sparing surgery for BOTs should be performed wherever possible to maximize fertility preservation. (3) Ovarian masses detected during emergency laparoscopy/laparotomy should be left in situ wherever feasible and investigated appropriately (imaging/tumor markers) before resection. (4) Follow‐up should be undertaken for all patients after BOT resection. Patients should be offered referral to adolescent gynecology to discuss fertility implications.ConclusionThis best practice Delphi consensus statement emphasizes the importance of managing children with BOTs through a well‐defined oncological MDT strategy, in order to optimize risk stratification and allow fertility preservation by ovarian‐sparing surgery wherever possible.

Recurrence and Metachronous Disease in Children with Benign Ovarian Tumors: A Systematic Review of the Literature

Abstract Aim The majority of ovarian tumors in children are benign, with good prognosis following complete resection. Little is published on the incidence of tumor recurrence and metachronous disease, and follow-up management of children with benign ovarian tumors (BOTs) remains a matter of debate. This systematic review aimed to evaluate the incidence and timing of recurrence and metachronous disease in children with BOTs in pediatric literature. Methods Comprehensive literature searches of the English literature (PubMed, OVID, EMBASE databases) from inception to present according to the Preferred Reporting Items for Systematic Review and Meta-analysis guidelines. Outcomes for tumor recurrence and metachronous disease were synthesized. Results Nineteen studies comprising 1,069 patients with BOTs were included in the analysis. All studies were retrospective cohort studies of children less than 18 years old. A total of 56 events of recurrence or metachronous disease were reported in these patients. The overall risk of recurrence/metachronous event occurrence was 5.2%/2.9%. Seventy-five percent of events occurred within the first 4 years following resection. Conclusion Although the studies identified are few and heterogeneous, they demonstrate a significant risk of tumor recurrence and metachronous disease for children following resection of a BOT.Especially following total unilateral oophorectomy, these children are at risk of losing the contralateral ovary in case of metachronous disease.Immediate discharge from follow-up, therefore, does not appear safe. The majority of events occurred within the first 4 years following resection. Follow-up for children following resection of a BOT should, therefore, be continued for a minimum of 4 years following surgery. Larger, long-term prospective studies are required to more accurately determine the true incidence and long-term outcomes for children and adolescents with these tumors.