A Pediatric Radiology Textbook and Pediatric Radiology Digital Library
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
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).