Prognosis

In a series of 19 treated patients with alveolar soft part sarcoma, one study reported a 5-year OS rate of 80%. The OS rate was 91% for patients with localized disease, 100% for patients with tumors 5 cm or smaller, and 31% for patients with tumors larger than 5 cm.[72]

In another series of 33 patients, the OS rate was 68% at 5 years from diagnosis and 53% at 10 years from diagnosis. Survival was better for patients with smaller tumors (≤5 cm) and completely resected tumors.[73][Level of evidence C1]

A retrospective review of children and young adults younger than 30 years (median age, 17 years; range, 1.5–30 years) from four institutions identified 69 patients treated primarily with surgery between 1980 and 2014.[68][Level of evidence C1] The ASPSCR1::TFE3 translocation was present in all 26 patients tested. There were 19 patients with IRS group I tumors (28%), 7 patients with IRS group II tumors (10%), 5 patients with IRS group III tumors (7%), and 38 patients with IRS group IV tumors (55%). The 5-year EFS rate was 80%, and the OS rate was 87% for the 31 patients with localized tumors (IRS postsurgical groups I, II, and III). The 5-year EFS rate was 7%, and the OS rate was 61% for the 38 patients with metastatic tumors (IRS group IV).

In a series of patients treated on consecutive studies from Germany, 15 of 61 patients (25%) presented with metastases, often miliary in nature. Despite lack of response to chemotherapy, the 5-year OS rate was 61%, with an EFS rate of 20%.[67]