Abstract
Alveolar soft part sarcoma (ASPS) is a rare mesenchymal soft-tissue tumour. It commonly arises in the lower extremities in adults and the head and neck in children. Primary pulmonary involvement, without evidence of soft tissue tumour elsewhere, is very rare. We present the seventh case ever reported in the literature of primary ASPS of the lung, which was revealed by brain metastases in a 28-year-old male patient. The parietal brain tumour was resected. The radiological and histological study led to the diagnosis: metastasis of an alveolar sarcoma. The patient then had brain radiotherapy and chemotherapy. Using this case as a starting point, we reviewed the characteristics and the main therapeutic options in these uncommon neoplasms.