포상 연부 육종

Abstract

Alveolar soft part sarcoma, named by Christopherson in 1952, is a very rare soft tissue sarcoma. It reportedly accounts for 0.5 to 1.0% of all soft tissue sarcoma and no established treatment principle assists it. The relative lack of symptoms makes it easily overlooked and consequently early diagnosis is frequently impossible. Often, the metastasis to the lung or the brain is the first manifestation of the disease. In some cases wide excision is not performed because it is misdiagnosed as a benign tumor. Authors experienced 7 cases of alveolar soft part sarcoma in 20 years and found the ultimate prognosis grave and the treatment not promising. Therefore, the authors recommend the following; 1. Early diagnosis and early treatment are important for successful management of alveolar soft part sarcoma. 2. Preoperative incisional biopsy is necessary for pathologic diagnosis. 3. The recommended treatment modality is a combination of wide excision, chemotherapy and radiotherapy.