Chad G Rusthoven1, Arthur K Liu2, Marilyn M Bui3, Tracey E Schefter2, Anthony D Elias4, Xian Lu5, Ricardo J Gonzalez6. 1. Department of Radiation Oncology, University of Colorado-Denver, Aurora, CO. Electronic address: chad.rusthoven@ucdenver.edu. 2. Department of Radiation Oncology, University of Colorado-Denver, Aurora, CO. 3. Department of Anatomic Pathology, Moffitt Cancer Center, Tampa, FL. 4. Department of Medical Oncology, University of Colorado-Denver, Aurora, CO. 5. Department of Biostatistics and Informatics, University of Colorado-Denver, Aurora, CO. 6. Sarcoma Program, Moffitt Cancer Center, Tampa, FL.
Abstract
BACKGROUND: Aortic sarcomas are rare and aggressive tumors with a propensity for arterial embolization, disseminated metastases, and rapid clinical deterioration. Overall, little is known about the evaluation and management of this disease. METHODS: A systematic review and pooled analysis were performed from a comprehensive search of the MEDLINE database for reports of primary aortic sarcomas published in the English language. RESULTS: One hundred sixty-five cases were analyzed. The median age was 60 years, and the male:female ratio was 1.5:1. High tumor grade (87.3%), arterial embolization (46.7%), and metastatic disease at diagnosis (44.8%) were common. Typical histologies were undifferentiated (39.4%), angiosarcomatous (37%), leiomyosarcomatous (13.3%), and fibroblastic (7.3%). Management was diverse and included combinations of surgical resection (46.7%), palliative vascular surgeries (37.7%), chemotherapy (28.7%), and radiotherapy (14.7%). The median survival was 11 months, and the 1-, 3-, and 5-year survival rates were 46.7%, 17.1%, and 8.8%, respectively. On univariate analyses, metastatic disease at diagnoses, surgical resection, and chemotherapy were associated with survival. On multivariate analysis, only metastatic disease remained significant (P < 0.001). CONCLUSIONS: Aortic tumors are devastating malignancies with distinct clinical features from sarcomas at other sites. Although prognosis is poor overall, long-term survivors have been reported, and aggressive management with surgical resection and adjuvant therapy should be considered in medically suitable patients. High embolic rates suggest a potential role for prophylactic anticoagulation.
BACKGROUND:Aortic sarcomas are rare and aggressive tumors with a propensity for arterial embolization, disseminated metastases, and rapid clinical deterioration. Overall, little is known about the evaluation and management of this disease. METHODS: A systematic review and pooled analysis were performed from a comprehensive search of the MEDLINE database for reports of primary aortic sarcomas published in the English language. RESULTS: One hundred sixty-five cases were analyzed. The median age was 60 years, and the male:female ratio was 1.5:1. High tumor grade (87.3%), arterial embolization (46.7%), and metastatic disease at diagnosis (44.8%) were common. Typical histologies were undifferentiated (39.4%), angiosarcomatous (37%), leiomyosarcomatous (13.3%), and fibroblastic (7.3%). Management was diverse and included combinations of surgical resection (46.7%), palliative vascular surgeries (37.7%), chemotherapy (28.7%), and radiotherapy (14.7%). The median survival was 11 months, and the 1-, 3-, and 5-year survival rates were 46.7%, 17.1%, and 8.8%, respectively. On univariate analyses, metastatic disease at diagnoses, surgical resection, and chemotherapy were associated with survival. On multivariate analysis, only metastatic disease remained significant (P < 0.001). CONCLUSIONS: Aortic tumors are devastating malignancies with distinct clinical features from sarcomas at other sites. Although prognosis is poor overall, long-term survivors have been reported, and aggressive management with surgical resection and adjuvant therapy should be considered in medically suitable patients. High embolic rates suggest a potential role for prophylactic anticoagulation.
Authors: Gabriella A Conte; Marjan Alidoost; Mitchel S Devita; Jonathan S Harmon; Jake W Schuler; Fernando Brea; Taliya Farooq; Angelo A Chinnici Journal: Eur J Case Rep Intern Med Date: 2020-09-15
Authors: Giuseppe Deiana; Genadi Genadiev; Antonio Nicola Giordano; Mario Moro; Francesco Spanu; Flavia Urru; Stefano Camparini Journal: Vasc Specialist Int Date: 2021-03-31