Pediatric Head and Neck Rhabdomyosarcoma

  • Etiology: Malignancy of mesenchymal origin which does not necessarily arise in muscle
  • Imaging Orbital:
    — Unilateral
    — Extraconal
    — Usually well-circumscribed
    — Most often in medial-superior quadrant of orbit
    — If parameningeal in location look hard for CNS involvement
    — May have aggressive or non-aggressive or mixed effects on bone
  • Imaging MRI:
    — T1WI: Hypointense to intermediate
    — T1WI post contrast: Enhances heterogeneously
    — T2WI: Intermediate to hyperintense
    — DWI: Restricted diffusion
  • DDX: Infantile hemangioma
  • Complications: Metastasis to lung
  • Treatment:
  • Clinical:
    — Most common sarcoma in children
    — Most common extraoccular orbital malignancy in childhood
    — In all ages with mean age 6-8 years old,
    — Orbital rhabdomyosarcoma has best prognosis of all head and neck rhabdomyosarcomas
    — 50% head and neck and 30% genitourinary and 20% musculoskeletal
    — Embryonal cell type seen in infants is most common
    — Alveolar cell type seen in older children and affects musculoskeletal system and is more aggressive
    — Orbital has best prognosis

Radiology Cases of Head and Neck Rhabdomyosarcoma

MRI of rhabdomyosarcoma
Axial T1 MRI without (above left) and with contrast (above right) of the orbits show a solid homogenously enhancing mass arising superior and medially to the right orbit which shows restricted diffusion on diffusion weighted imaging (below right).