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RMS causes swelling in the affected body part (usually painless). 

  • Nearly 50% have alveolar histology and in this case nodal involvement is more likely.  Overall there is a 20% risk of nodal metastases.
  • The preferred operative procedure is wide local excision with negative margins (even to convert a patient to group II with no gross residual disease). 
  • Adjacent lymph node sampling (inguinal or axillary) should always be performed - even when there is no palpable abnormality
  •  It is important not to give RT to the entire limb circumference and a longitudinal strip of normal tissue should always be spared.
  • Tumor tends to spread along fascial planes, to recur locally and to metastasize to distant sites.
  • Tumors at this site have a relatively poor prognosis.


The T1 TSE coronal MR below shows an extremity RMS (#1) in a 6 year old boy:

Below is a MR (Post gad transverse T1 FS) of the same tumor (#1):

Below is the PET scan of the same tumor (#1) showing increased FDG uptake:



The prognosis is similar to extremity tumors.

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