Epidemiology
A rare sarcoma of young adults, often related to the tendons or aponeuroses of the limbs. Its relationship to tendons and aponeuroses and its occasional melanin can lead to confusion with metastatic melanoma.
Basic features
It can mimic a melanoma because of its melanocytic markers, but it is distinguished by its characteristic EWSR1-ATF1 fusion.
Usual treatment
The main treatment is wide resection with lymph node evaluation and additional therapies according to risk.
Outcomes and prognosis
The prognosis tends to be unfavorable in the long term: it responds poorly to chemotherapy and has a marked tendency toward late metastases, including to the lymph nodes, so it requires prolonged follow-up.
