Abstract
Alveolar soft part sarcoma (ASPS) is a rare form of soft-tissue sarcoma arising from connective tissue. It is most often seen in adolescents and young adults and has high propensity for recurrence and metastasis. Clinically, it mimics haemangioma or arteriovenous malformations. In our case report, an 18-year-old female presented with markedly vascular tumour in the left forearm, for which excision biopsy was done. Histopathological report revealed ASPS. The patient was screened for metastasis. Ultrasound abdomen, computed tomography (CT) chest, CT brain and whole-body skeletal survey was done. The patient was found to have bilateral pulmonary metastasis. The patient was given 6 cycles of chemotherapy with adriamycin, cyclophosphamide and vincristine. There was no locoregional and pulmonary recurrence for 11 months after being treated by excision of the tumour followed by chemotherapy.