Summary of the expert consensus
The prognosis of RMS arising from the female genital tract is favorable and there is an excellent response to chemotherapy. Initial complete surgical resection is not the goal in RMS of the vagina or uterus. The initial goal of surgery is the establishment of the diagnosis. Vaginal RMS do not require an upfront surgical resection except in very small, localized and well circumscribed tumors that can be completely resected with complete organ preservation. Primary re-excision is usually not indicated in this patient population unless the tumor can be completely resected with complete organ preservation. In tumors that do not completely respond to induction therapy, or who have confirmation of persistent viable tumor identified by screening vaginoscopy with biopsies and are not amenable to surgical resection, local therapy with RT, preferably BT applied as intracavitary and/or interstitial, is helpful. There is a role for surgical resection of gross disease provided the tenets of complete gross resection with organ preservation utilizing conservative procedures can be maintained.
RMS of the cervix uteri is treated similarly to RMS of the vagina. The diagnosis can be established by transvaginal biopsy or polypectomy. Partial excision of the cervix as primary surgery or PRE is feasible for small pedunculated tumors with preservation of organ function. In patients with evidence of tumor after induction chemotherapy local control is attempted using resection or RT. Brachytherapy is usually carried out with a vaginal mold and an additional brachytherapy tube which is placed into the cervix uteri. Residual tumors at the cervix uteri may be treated by partial or total resection of the cervix. Trachelectomy is an alternative option.
Rhabdomyosarcoma of the corpus uteri is treated following the above-mentioned principles regarding local therapy decision. For patients with persistent tumors after induction chemotherapy, abdominal hysterectomy should be performed.
Fertility preservation should be considered in all patients. The modality depends on the local treatment approach.