Local treatment of rhabdomyosarcoma of the female genital tract: Expert
consensus from the Children’s Oncology Group (COG), European Soft Tissue
Sarcoma Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe
(CWS)
Abstract
Background The International Soft Tissue Sarcoma Consortium (INSTRuCT)
was founded as an international collaboration between different
pediatric soft tissue sarcoma cooperative groups (COG, EpSSG, CWS).
Besides other tasks, a major goal of the INSTRuCT is to develop
consensus expert opinions for best clinical treatment. This consensus
paper for patients with rhabdomyosarcoma of the female genital tract
(FGU-RMS) provides treatment recommendations for local treatment, long
term follow up and fertility preservation. Methods Review of the current
literature was combined with recommendations of the treatment protocols
of the appropriate clinical trials. Additionally, opinions of
international FGU-RMS experts were incorporated into recommendations.
Results The prognosis of FGU-RMS is favorable with an excellent response
to chemotherapy. Initial complete surgical resection is not indicated,
but diagnosis should be established properly. In patients with tumors
localized at the vagina or cervix demonstrating incomplete response
after induction chemotherapy, local radiotherapy (brachytherapy) should
be carried out. In patients with persistent tumors at the corpus uteri,
hysterectomy should be performed. Fertility preservation should be
considered in all patients. Conclusion For the first time, an
international consensus for the treatment of FGU-RMS patients could be
achieved, which will help to harmonize the treatment in different study
groups.