Sarcomas of the aorta: a systematic review and pooled analysis of published reports.
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.
Department of Radiation Oncology, University of Colorado-Denver, Aurora, CO. Electronic address: chad.rusthoven@ucdenver.edu.
Chad G
CG
Rusthoven
Department of Radiation Oncology, University of Colorado-Denver, Aurora, CO.
Arthur K
AK
Liu
Department of Anatomic Pathology, Moffitt Cancer Center, Tampa, FL.
Marilyn M
MM
Bui
Department of Radiation Oncology, University of Colorado-Denver, Aurora, CO.
Tracey E
TE
Schefter
Department of Medical Oncology, University of Colorado-Denver, Aurora, CO.
Anthony D
AD
Elias
Department of Biostatistics and Informatics, University of Colorado-Denver, Aurora, CO.
Xian
X
Lu
Sarcoma Program, Moffitt Cancer Center, Tampa, FL.
Ricardo J
RJ
Gonzalez
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001011:Aorta / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D060787:Neoplasm Grading; D009361:Neoplasm Invasiveness; D016016:Proportional Hazards Models; D012307:Risk Factors; D012509:Sarcoma / Q000401:mortality / Q000556:secondary / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D055815:Young Adult