Alveolar rhabdomyosarcoma

Alveolar rhabdomyosarcoma 

Updated: 08/18/2021

© Jun Wang, MD, PhD

General features
  • High grade tumor
  • Rapid growth
  • Early to mid-teens
  • Classified as alveolar type if alveolar components present
  • Poor prognosis, worse with N-myc or PAX3 abnormalities
Clinical presentations
  • Painless mass
  • Most common location: deep muscles
Key morphological features
Markers
  • Positive for myogenin, desmin, myoD1
Genetic abnormalities
  • N-myc amplification
  • PAX3-FKHR(PAX3-FOXO1)
  • PAX7-FKHR(PAX7-FOXO1)
Treatment
  • Combination of surgery, radiation and chemotherapy


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