Sarcomas characterized by gene alterations, are a distinct clinico-pathological group of high-grade tumors, that represent 5% of small round cell tumors without or fusion. Diverse genetic alterations characterize this group, including gene fusion being the most common alteration and less frequently internal tandem duplications (ITDs). We present a compelling case of a 3-year-old girl diagnosed with a high-grade nasoethmoidal sarcoma exhibiting -ITD.
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