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Non-Rhabdomyosarcoma (Non-RMS)

 

 

Prognosis

 

 

The prognosis of non-RMS tumors varies greatly depending on the histologic grade, invasiveness, tumor size, resectability, use of radiation therapy, site of primary tumor, and presence of metastases.

 

Prognostic factors

POOR PROGNOSIS IMPROVED PROGNOSIS

Advanced tumor stage

  • High AJCC stage (Stage III)
Localized disease
High histopathological grade Low histopathological grade
Size Size

Anatomical depth

  • Deep tumor

Anatomical depth

  • Superficial tumor

Site

  • Extremity

Site

  • Trunk, pelvis
Positive margin of resection for disease Tumor widely excised
Poor tumor response to therapy 90% or more necrosis of tumor after therapy
No adjuvant radiation therapy Radiation therapy improves chance of local control
   

 

 

Results of a pooled analysis of results from United states and European groups:

  • Overall survival (OS):
    • 60.0% at 5 years
    • 51.5% at 10 years
    • Significantly associated with patient's age, histological subtype, tumour site and size, quality of delayed surgical resection, radiotherapy administration and response to induction chemotherapy.

MPNST associated to neurofibromatosis type 1 was the tumour type with the worst rate of response to chemotherapy and the worst outcome.

 

 

 

 

 

References

 

 

  

 

 

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