Vaginal Germ Cell Tumour (Yolk Sac Tumour) in Children and Adolescents — What to Do After Neoadjuvant Chemotherapy Has Not Achieved Complete Remission

Clinical scenario

This protocol addresses children and adolescents with a vaginal germ cell tumour — specifically yolk sac tumour (YST), the most common and only relevant malignant germ cell tumour arising in the vagina in this age group. Elevated serum alpha-foetoprotein (AFP) is a defining feature of this presentation and is used as both a diagnostic marker and a treatment-response indicator throughout management.

Previous line — failure condition

The standard first-line approach for paediatric vaginal YST is neoadjuvant chemotherapy — including platinum-based regimens — with the number of cycles and drug selection adapted to the extent of disease, dissemination pattern, and patient age. Initial surgical resection is avoided, as vaginal GCT are highly chemosensitive.

This protocol applies when that chemotherapy has not achieved its required endpoints: normalisation of serum AFP, complete remission on pelvic MRI, and negative vaginoscopy.

Next-step approach (partial overview)

When chemotherapy alone has not produced complete remission, the subsequent strategy involves surgical and/or radiotherapy-based interventions — selected and sequenced according to the anatomical site and the extent of residual disease, with organ preservation as a guiding principle where achievable.

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References

DOI: 10.1016/j.radonc.2023.109662

Vaginal YST is frequently associated with elevated serum AFP (alpha-foetoprotein), which should always be measured at diagnosis and during treatment.

YST is by far the most common GCT and the only relevant malignant GCT occurring in the vagina.

Surgery should be reserved for situations where there is still persistent disease after completion of neoadjuvant chemotherapy.

If conservative surgery is not feasible, brachytherapy can be proposed as local treatment.

Radiotherapy is only indicated if no complete response can be achieved by chemotherapy ± organ sparing surgery. In case of radiotherapy, vaginal brachytherapy is preferred over EBRT to minimize long term morbidity. If EBRT is needed, proton beam therapy is preferred [IV, B].

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