Clinical Scenario

This protocol addresses vaginal rhabdomyosarcoma of the embryonal subtype presenting in children and adolescents. Among vaginal malignancies in this age group, RMS is the predominant histological type. Embryonal RMS at this site is highly chemosensitive, and a systemic-first approach is appropriate from initial diagnosis.

Treatment Approach — Partial Overview

Management follows a multimodal strategy in which systemic chemotherapy is the primary first-line intervention. Neoadjuvant and adjuvant combination chemotherapy including an alkylating agent forms the backbone of treatment, with the regimen tailored to individual risk factors. Importantly, initial surgical resection of the tumour is deliberately avoided; at diagnosis, surgery is limited to a diagnostic biopsy only.

Full sequencing, risk stratification criteria, maintenance considerations, and local therapy decision rules are detailed in the complete protocol.

Treatment Goals

The target is complete remission, confirmed by a negative pelvic MRI and negative vaginoscopy with biopsies of any suspicious areas, assessed after neoadjuvant chemotherapy courses.

References

The main histological types are RMS (70%) and GCT/YST (27%); clear cell carcinoma is less common (3%).
RMS and YST are highly chemosensitive. Therefore, a multimodal strategy starting with systemic chemotherapy should be always considered in the first line.
Neoadjuvant and adjuvant combination chemotherapy, including an alkylating (cyclophosphamide, ifosfamide) agent, is recommended.
The chemotherapy schedule should be adapted to risk factors (IRS stage, age, tumour size, nodal or distant spread, molecular and pathology findings).
In rare cases with regionally involved lymph nodes and/or with a fusion positive RMS subtype additional maintenance strategies, after adjuvant therapy, should be considered.
Local therapy is adapted to the tumour response and histological type, assessed by pelvic MRI and vaginoscopy after 3 and 6 courses of neoadjuvant chemotherapy.
In case of complete remission of an embryonal RMS after 6 courses of neoadjuvant chemotherapy confirmed by a negative pelvic MRI and a negative vaginoscopy (including biopsies of any suspicious areas), no local treatment is indicated.

DOI: 10.1016/j.radonc.2023.109662

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Treatment of Vaginal Cancer with Rhabdomyosarcoma (Embryonal Type) in Children and Adolescents

References