PDF(Pubmed)
Abstract：
Alveolar soft part sarcoma (ASPS) is an extremely rare type of soft tissue sarcoma. The primary sites of ASPS are mostly located in the extremities and trunk. Primary pulmonary ASPS is extremely rare. A search of the PubMed® database identified only five cases of primary pulmonary ASPS. This current case report describes the sixth case of ASPS in a 15-year-old male that presented with recurrent headaches. Head computed tomography showed space-occupying lesions in the left parietal lobe. Positron emission tomography-computed tomography confirmed the space-occupying lesions in the left parietal lobe and showed multiple nodules and masses in the two lungs and pleura, which were considered to be low-grade malignant mesenchymal tumours. The case report presents the clinical characteristics, diagnosis and treatment process. Programmed cell death protein 1 monoclonal antibody (sintilimab) combined with a tyrosine kinase inhibitor (anlotinib hydrochloride) achieved a good therapeutic effect, indicating that this combination therapy is worth exploring further. Large-scale prospective studies are needed to explore and develop standardized treatments for ASPS.
摘要：
肺泡软组织肉瘤（ASPS）是一种极为罕见的软组织肉瘤。ASPS的主要部位主要位于四肢和躯干。原发性肺ASPS极为罕见。对PubMed®数据库的搜索仅发现5例原发性肺ASPS。当前的病例报告描述了一名15岁男性中的第六例ASPS，该病例表现为复发性头痛。头部计算机断层扫描显示左顶叶占位病变。正电子发射断层扫描-计算机断层扫描证实左顶叶占位性病变，并显示两肺和胸膜多发结节和肿块，被认为是低度恶性间充质肿瘤。病例报告介绍了临床特点，诊断和治疗过程。程序性细胞死亡蛋白1单克隆抗体（sintilimab）联合酪氨酸激酶抑制剂（盐酸安洛替尼）取得了良好的治疗效果，表明这种联合治疗值得进一步探索。需要大规模的前瞻性研究来探索和开发ASPS的标准化治疗方法。
