Clinical presentation

The median age at presentation is 25 years for patients with alveolar soft part sarcoma. This tumor most commonly arises in the extremities but can occur in the oral and maxillofacial region.[63-65] Alveolar soft part sarcoma in children can present with evidence of metastatic disease.[66] Delayed metastases to the brain and lung are uncommon.[63]

In a series of 61 patients with alveolar soft part sarcoma who were treated in four consecutive CWS trials and the Soft Tissue Sarcoma Registry (SoTiSaR), 46 patients presented with localized disease and 15 patients had evidence of metastasis at diagnosis.[67]

Sixty-nine patients younger than 30 years with alveolar soft part sarcoma were treated between 1980 and 2014 at four major institutions. The median age at diagnosis was 17 years, and 64% of patients were female. The most common site of disease was the lower extremity, and 26 patients had an ASPSCR1::TFE3 gene translocation.[68]