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
- Embryonal rhabdomyosarcoma
- Alveolar rhabdomyosarcoma
- Pleomorphic rhabdomyosarcoma
- Anaplastic rhabdomyosarcoma
- Sclerosing rhabdomyosarcoma
- Mixed type
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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