Adult parameningial alveolar rhabdomyosarcoma: Case report and literature review

Cancer Radiother. 2020 Dec;24(8):870-875. doi: 10.1016/j.canrad.2020.03.015. Epub 2020 Nov 7.


Alveolar rhabdomyosarcoma (ARMS) represents the most common childhood soft tissue sarcoma, but they are rarely seen among adults. Most of the protocols for adults are adapted from pediatric protocols. Here we report a case of a 53-year-old woman diagnosed with a nasal alveolar rhabdomyosarcoma, stage IV at diagnosis, treated by chemotherapy (a regimen inspired from the pediatric protocole pEpSSG RMS 2005) which led to partial response followed by chemo-radiotherapy. We performed a systematic review of adult head and neck ARMS and found 29 cases. Primary chemotherapy with different protocols (VAC, VAI or VIE) should be done followed by surgery and/or external beam radiotherapy (preferably with IMRT). EBRT seems beneficial to every ARMS with a dose around 50Gy in a conventional fractionation, eventually completed with a boost on residual tumor. The target volume must be defined on pre-chemotherapy imaging. Brachytherapy and proton therapy are under evaluation.

Keywords: Adult; Adulte; Alveolar rhabdomyosarcoma; Chemotherapy; Chimiothérapie; Radiation therapy; Radiothérapie; Rhabdomyosarcome alvéolaire.

Publication types

  • Case Reports
  • Systematic Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Chemoradiotherapy / methods*
  • Combined Modality Therapy / methods
  • Dactinomycin / administration & dosage
  • Doxorubicin / administration & dosage
  • Female
  • Humans
  • Ifosfamide / administration & dosage
  • Middle Aged
  • Nose Neoplasms / diagnostic imaging
  • Nose Neoplasms / therapy*
  • Radiotherapy, Intensity-Modulated / methods
  • Rhabdomyosarcoma, Alveolar / diagnostic imaging
  • Rhabdomyosarcoma, Alveolar / therapy*
  • Vincristine / administration & dosage


  • Dactinomycin
  • Vincristine
  • Doxorubicin
  • Ifosfamide