About 50% of all soft tissue malignomas Annually new diagnosis in 4.

3 in 1 million children less than 16 years old Sixty-seven percent of children are less than 10 years old at diagnosis Ratio of boys to girls 1.15:1

Age distribution
Age (years) Frequency (%) <1 year 7 l4 years 35 59 years 25 1014 years 20 >15 years 13 Location Frequency (%) Head and neck (without orbit) 26 Orbital 9 Genitourinary 22 Extremities 18 Trunk 7 Retroperitoneal 7 Perineal and anal 2 Others 9

Etiology and Pathogenesis Mostly sporadic forms Some with genetic predisposition as in Li-Fraumeni syndrome with mutation of the p53 suppressor gene (TP53): high incidence of brain and breast tumors and adrenocortical carcinoma in families with a child with rhabdomyosarcoma Increased risk in fetal alcohol syndrome; also in mothers using marijuana or cocaine during pregnancy Cytogenetics: NRAS oncogene abnormalities in 35% of patients Mouse models with inactivated p53 or pRB demonstrate disturbance of muscle differentiation and neoplastic development Histopathology Muscle-specific proteins are histologically detectable, such as actin, myosin, desmin, and myoglobulin Four subtypes of rhabdomyosarcoma Embryonal: Frequency: 5364% of all rhabdomyosarcomas in childhood Location: orbit, head and neck, abdomen, genitourinary tract Microscopically resemblance to embryonic muscle tissue; mainly primitive round cells, some spindle cells with central nucleus and eosinophilic cytoplasm; cross striations characteristic of skeletal muscle in about 30% of cases Subtype: Sarcoma botryoides (6% of all rhabdomyosarcomas in children); in vagina, bladder, uterus; microscopically as embryonal type with polypoid mass and presence of a dense subepithelial cell layer Alveolar: Frequency: 21% of all rhabdomyosarcomas in children

 Location: mainly extremities Histology: round cells with eosinophilic cytoplasm, occasionally with vacuoles; multinucleated giant cells; rarely cross-striations; groups of tumor cells separated by fibrotic septation (alveolar structure) Pleomorphic: Frequency: 1% of all rhabdomyosarcoma in children Occurrence: mainly in adulthood Histology: undifferentiated muscle tissue; spindle cells with variable eosinophilic cytoplasm and pleomorphic nuclei, frequently mitotic cells, often cross-striations, structured in rows and bundles Undifferentiated subtype: Frequency: 8% without muscle-specific gene proteins