GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma

Clin Transl Oncol. 2021 Dec;23(12):2460-2473. doi: 10.1007/s12094-021-02654-1. Epub 2021 Jul 1.


Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm.

Keywords: Alveolar rhabdomyosarcoma; Embryonal rhabdomyosarcoma; Prognostic factors; Rhabdomyosarcoma.

MeSH terms

  • Child
  • Combined Modality Therapy
  • Humans
  • Incidence
  • Positron Emission Tomography Computed Tomography / methods*
  • Practice Guidelines as Topic / standards*
  • Rhabdomyosarcoma / diagnostic imaging
  • Rhabdomyosarcoma / epidemiology
  • Rhabdomyosarcoma / pathology
  • Rhabdomyosarcoma / therapy*
  • Spain / epidemiology