Abstract:

Objective To review the clinical manifestations, pathological morphology, imaging findings, treatment and prognosis of thoracic soft tissue angiosarcoma. Methods The clinical data, diagnosis, treatment and follow-up of a patients with pleural soft tissue angiosarcoma admitted to Shaanxi Tuberculosis Control Hospital on May 8, 2020 were retrospectively analyzed with review of literatures. Taking “angiosarcoma” as the search term, the Chinese literature was searched through the CNKI and Wanfang, and “lung angiosarcoma” and “hemangiosarcoma” as the search terms, 56 domestic and 21 foreign related literatures were retrieved through the PubMed. Fifteen patients with complete data in 12 case reports were selected and summarized. The clinical characteristics, diagnosis and treatment of them were analyzed with the reported case. Results The patient was 34 years old, male, and the main symptom was “hemoptysis for 1 month”. The hemostatic effect was poor after interventional embolization on May 9 and 14, 2020. On June 12, 2020, the left thoracic focus was removed and the left lower lobe of lung was resected, the thoracic cavity continued to bleed after operation. On June 24, 2020, the thoracic exploration, hemostasis and pleural biopsy were performed. There was still a lot of bleeding in the thoracic cavity after operation. On February 1, 2021, thoracotomy exploration and thoracoplasty were performed. After operation, immunohistochemistry platelet-endothelial cell adhesion molecule, artificial hematopoietic progenitor cell antigen, vimentin, cell proliferation marker index-67 and ETS-related genes were positive, and the patient was diagnosed as Angiosarcoma and transferred to tumor hospital for blood transfusion. Half a month later, the patient died. Of the 16 patients (including 15 obtained from the literature), 10 were male and 6 were female, and the average age was 54 years. Clinical symptoms: hemoptysis in 8 cases, chest tightness and shortness of breath in 6 cases, cough in 7 cases and chest pain in 4 cases; 1 case was complicated with pulmonary tuberculosis and tuberculous empyema, 1 case was complicated with lung cancer. Of the 7 unilateral pulmonary cases: 3 in left lung and 4 in right lung.Bilateral lung lesions were found in 9 cases, platelet-endothelial cell adhesion molecule was positive in 16 cases, artificial hematopoietic progenitor cell antigen was positive in 9 cases, vimentin was positive in 4 cases, cell proliferation marker index-67 was positive in 3 cases, plasma coagulation factor Ⅷ was positive in 7 cases, ETS related gene was positive in 2 cases and Fried leukemia virus insertion site 1 was positive in 2 cases. All the 16 patients were confirmed by pathological examination, 3 were puncture biopsy and 13 were thoracoscopic biopsy; 10 cases received surgical treatment, 3 cases received chemotherapy, 2 cases received surgery+chemotherapy, and 1 case gave up treatment; 13 cases died and 3 cases were lost in follow-up. The average survival time was (7.2±2.1) months. Conclusion Thoracic soft tissue angiosarcoma is a very rare malignant tumor from soft tissue with poor prognosis. The treatment methods include surgery, radiotherapy and chemotherapy, and molecular targeted therapy.

Key words: Angiosarcoma, Disease attributes, Review literature as topic