Rhabdomyosarcoma

Rhabdomyosarcoma 

Updated: 08/18/2021

© Jun Wang, MD, PhD

General features
  • Malignant soft tissue tumor with skeletal muscle differentiation
  • Most common pediatric soft tissue sarcoma
Clinical presentations
  • Nontender mass
  • Occasionally with overlying skin erythema
  • Most common location: Head and neck, genitourinary tract, and the extremities
Subtypes
Key morphological features
  • Rhabdomyoblast: eccentric nuclei, granular cytoplasms, tadpole cells
Markers
  • Positive for myogenin, desmin, myoD1
Treatment
  • Surgery
  • Radiation
  • Chemotherapy
Prognosis
  • Superior prognosis: botryoid, spindle cell
  • Intermediate prognosis: embryonal
  • Poor prognosis: alveolar, undifferentiated sarcoma
  • Poor with FOXO1 gene fusion


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