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Non-Rhabdomyosarcoma (Non-RMS)



General Management



The investigations and management of any non-RMS should be organized by a multidisciplinary team. This team should include a surgeon, a medical oncologist and a radiation oncologist together guided by an expert pathologist and radiologist.

The information necessary to make a treatment plan includes:

  • Clinical assessment
    • Specific to evaluate primary tumor extent
    • General health
  • Imaging studies
  • Planned biopsy


Discussion of these results in the setting of a multidisciplinary conference is essential to assess:

  • Staging
    • Local extent of disease
      • Tumor size
      • Tumor depth
      • Relationship to neurovascular bundle
    • Distant extent of disease
      • Presence of metastatic disease or not
  • Pathology
    • Tumor type
    • Grade
  • How each treatment modality can be optimally used for effective treatment of that particular patient
    • Chemotherapy
    • Radiation therapy
    • Surgery


Conventional treatment of soft tissue sarcomas of the extremities involves surgery aiming to achieve widely negative margins around the tumor.

Radiation therapy can be used pre-operatively with the aim of sterilizing the tumor edges if wide negative margins would be impossible to achieve.

Post-operative radiation therapy is indicated if margins are positive for microscopic disease to reduce the risk of local recurrence.

This multimodality approach has replaced amputation as the primary surgical treatment of choice.

Recommended guidelines for the management for these "adult type" soft tissue sarcoma exist and are very relevant to the treatment of children who have these tumors.  A multidisciplinary team is essential for effective therapy of these tumors.


ARST1321 is the current open COG study:

This study uses a tyrosine kinase inhibitor called pazopanib.  Tyrosine kinases have been found to be expressed and dysregulated in different non-RMS subtypes. Multi-targeted tyrosine kinase inhibitors (TKIs), such as pazopanib are able to target multiple signaling pathways that may be disrupted in many non-RMS. This study builds on ARST0332 by adding pazopanib to standard chemotherapy and radiation therapy in pediatric (and adult patients) who have newly diagnosed intermediate and high risk non-RMS.




National Cancer Institute: Childhood soft tissue sarcoma

Guidelines for the management of soft tissue sarcomas (Sarcoma. 2010; 2010: 506182.Robert GrimerIan JudsonDavid Peake, & Beatrice Seddon)




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