- Most common soft tissue sarcoma in children
- 3% to 4% of all cases of childhood cancer
- More common in males and Caucasians
- Two-thirds of cases occur in patients under the age of 10 years
- Median age at diagnosis of 5 years
- Germ-line P53 mutations
- Costello syndrome
- Beckwith-Wiedemann syndrome
- Neurofibromatosis type I
- Germ-line DICER1 mutations
- Parental use of cocaine and marijuana
- Birthing order and accelerated in utero growth
Site of involvement
- Head and neck (including the orbit and parameningeal areas [35%])
- Genitourinary tract (including the bladder, prostate, vagina, vulva, uterus, and paratesticular area [26%]), and
- Extremities (19%)
- 20% of children present with disseminated disease - commonly involves the lung
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