Epidemiology
A rare neoplasm of children and young adults that, within this site, is considered when it appears in the pelvis, limbs, or chest wall. Some cases have ALK alterations or other fusions, information that can open up targeted treatment options.
Basic features
It can cause a mass, pain, or inflammatory symptoms; some lesions have ALK alterations or other treatable fusions.
Usual treatment
Resection is preferred when possible; in unresectable or recurrent disease with an ALK rearrangement, the ALK inhibitor crizotinib is an approved targeted option, and ROS1 or NTRK fusions open up other therapies.
Outcomes and prognosis
It can recur and rarely metastasize, so it needs follow-up based on its location and molecular biology.
