TriangleHEALTH PROFESSIONAL · SOURCE READING
Surgery with or without radiation therapy and chemotherapy
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
The standard treatment approach is complete resection of the primary lesion.[72] If complete excision is not feasible, radiation therapy is administered.
Source links and citations
Evidence (surgery with or without chemotherapy):
A study from China reported on 18 patients with alveolar soft part sarcoma of the oral and maxillofacial region. Fifteen patients were younger than 30 years. Surgical removal with negative margins was the primary treatment.[65][Level of evidence C2]
Source links and citations
A study from China reported on 18 patients with alveolar soft part sarcoma of the oral and maxillofacial region. Fifteen patients were younger than 30 years. Surgical removal with negative margins was the primary treatment.[65][Level of evidence C2]
All patients survived, and only one patient had metastatic disease recurrence.
In a series of patients treated on consecutive studies from Germany, the following was reported:[67]
Source links and citations
In a series of patients treated on consecutive studies from Germany, the following was reported:[67]
Progression-free survival (PFS) for patients without metastases on presentation appeared to improve with complete resection of the primary tumor.
In a series of patients treated on consecutive studies from Germany, the following was reported:[67]
The 5-year EFS rate was 100% for patients with completely resected tumors, compared with 50% for patients with microscopic or gross residual disease.
In a series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma, the following was reported:[63][Level of evidence C1]
Source links and citations
In a series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma, the following was reported:[63][Level of evidence C1]
The OS rate was 78% at 10 years, and the EFS rate was about 63%.
In a series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma, the following was reported:[63][Level of evidence C1]
Patients with localized disease (n = 37) had a 10-year OS rate of 87%.
In a series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma, the following was reported:[63][Level of evidence C1]
The 14 patients with metastases at diagnosis had a 10-year OS rate of 44%, partly resulting from the surgical removal of the primary tumor and lung metastases in some patients.
In a series of 51 pediatric patients aged 0 to 21 years with alveolar soft part sarcoma, the following was reported:[63][Level of evidence C1]
Only 3 of 18 patients (17%) with measurable disease had a response to conventional antisarcoma chemotherapy, but two of four patients treated with sunitinib had a partial response.
Publication references
Read the original reference and check its publication notices.
Preserved source evidence · Not medical advice