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.