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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)
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  • Timothy Lautz,
  • Helene Martelli,
  • Joerg Fuchs,
  • Cyrus Chargari,
  • Naima Smeulders,
  • Candace Granberg,
  • Suzanne Wolden,
  • Monika Sparber-Sauer,
  • Douglas Hawkins,
  • Gianni Bisogno,
  • Ewa Koscielniak,
  • David A. Rodeberg,
  • Guido Seitz
Timothy Lautz
Ann and Robert H Lurie Children's Hospital of Chicago
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Helene Martelli
Hopital Bicetre
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Joerg Fuchs
University of Tuebingen
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Cyrus Chargari
Institut de Cancerologie Gustave Roussy
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Naima Smeulders
Great Ormond Street Hospital for Children
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Candace Granberg
Mayo Clinic
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Suzanne Wolden
Memorial Sloan Kettering Cancer Center
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Monika Sparber-Sauer
Klinikum Stuttgart Olgahospital Frauenklinik
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Douglas Hawkins
Seattle Children's Hospital
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Gianni Bisogno
Division of Hematology/Oncology
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Ewa Koscielniak
Klinikum Stuttgart Olgahospital Frauenklinik
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David A. Rodeberg
East Carolina University
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Guido Seitz
University Children's Hospital Marburg
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Peer review status:ACCEPTED

30 Jun 2020Submitted to Pediatric Blood & Cancer
30 Jun 2020Assigned to Editor
30 Jun 2020Submission Checks Completed
30 Jun 2020Reviewer(s) Assigned
01 Jul 2020Review(s) Completed, Editorial Evaluation Pending
02 Jul 2020Editorial Decision: Accept

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.