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Introduction: Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor that shows skeletal muscle differentiation. There is no evidence it arises from skeletal muscle cells. Many cases occur at sites with scant or no striated muscle tissue (e.g. common bile duct, urinary bladder, nasal cavity, middle ear, and vagina etc).

Associations: RMS may occur in association with congenital retinoblastoma, familial adenomatous polyposis, multiple lentigines syndrome, type I neurofibromatosis, Costello syndrome, Beckwith-Wiedemann syndrome, and many other congenital anomalies.

Locations: RMS can occur anywhere in the body, but affect three regions more commonly - the head and neck, genitourinary tract and retroperitoneum, and upper and lower extremities. This photograph shows a large embryonal RMS involving the foot of an infant. More detailed discussion of locations is in the next image.
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