Clinical characteristics associated with primary cardiac angiosarcoma outcomes: a surveillance, epidemiology and end result analysis

Abstract

Background Primary cardiac angiosarcoma (PCAS) is a rare type of tumour. Furthermore, descriptions of the demographic features and prognostic factors of PCAS patients have been poorly reported. Methods A population cohort study was conducted using retrospectively extracted data from the SEER (Surveillance, Epidemiology and End Results) database for patients with histological diagnoses of PCAS; the extracted information included demographic, treatment and outcome data. Results A total of 168 cases of PCAS from 1973 to 2013 were included. The mean age at diagnosis was 44.4 ± 15.5 years. PCAS was more prevalent in men than in women. The majority of PCAS patients were white (67.3%), while the incidence of PCAS in black individuals was relatively infrequent (19.0%). In addition, 87 cases were classified as distant stage, 44 as regional stage, and 33 as localized stage. The median disease-specific survival (DSS) was 7.22 months, and the 1-, 2- and 5-year DSS rate for PCAS patients was 34.7%, 14.3% and 10.2%, respectively. Further multivariate analyses showed that an age at (greater than or equal to) 45 years (HR 2.165), no radiotherapy (HR 1.629), tumour size > 5 cm (HR 3.182), and the summary stage was associated with worse PCAS-related survival. Cancer-directed surgery and radiotherapy significantly improved the DSS for patients with PCAS ( P < 0.05). The C-index of the nomograms was 0.706 (95% CI 0.654–0.758), and the calibration curves showed good agreement between the nomogram prediction and actual observation. Conclusion PCAS is a rare cancer that is prone to have poor prognoses. To understand PCAS more thoroughly, more cases with adequate information are needed.

Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study

“…Conic et al analyzed the outcome of cutaneous AS patients and disclosed no significant impacts of RT on OS [ 3 ], same results were confirmed by Buehler et al and Zhang et al [ 11 , 26 ]. In contrast, Ogawa et al [ 19 ] reported that RT effectively improved OS in 48 patients with localized AS of the scalp and face.…”

“…The results of the earlier studies regarding the relation between tumor grade and patients’ survival time varied. A number of previous studies have documented no association of tumor grade with OS of AS [ 7 , 26 ]. Conversely, Kathryn et al [ 20 ] reported that higher tumor grade is predictive of greater risk of death in primary mediastinal sarcoma, conflicting with the conclusion reached by Brett et al and Manjari et al [ 21 , 27 ].…”

“…In contrast, a pooled analysis incorporating 75 articles involving 186 patients suggested that tumor size (< 10 cm) was the only significant favorable factor for OS of hepatic AS in adults [ 28 ]. Several other documents presented tumor size was a critical predictor [ 7 , 10 , 18 , 26 , 29 ]. Data from our large-scale investigation revealed that tumor size was an independent prognostic factor for this rare disease.…”

Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

“…A retrospective analysis on 168 primary cardiac angiosarcomas selected from the National Cancer Institute's SEER database confirmed data about outcomes associated with RT. The mean survival rate was higher in patients who underwent radiotherapy (63% higher than controls at univariate analysis) [73].…”

Prognostic Nomograms for Predicting Overall Survival and Cancer-Specific Survival in Patients with Angiosarcoma, a SEER population-based study

“…As for the histologic grade, a recent study concluded AS with grade III histology tended to experience a shorter survival which was consistent with our result 15 . The median survival time of AS was various in different primary sites [16][17][18] , but which site with the highest mortality has not been well documented. Our study elaborated AS from bone harbored the highest risk compared with soft tissue, heart mediastinum and peritoneum.…”