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. 2019 Aug 19;24(1):29.
doi: 10.1186/s40001-019-0389-2.

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

Chanqiong Zhang  1 , Chongan Huang  2 , Xueke Zhang  1 , Liang Zhao  3 , Dan Pan  4
Affiliations

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

Chanqiong Zhang et al. Eur J Med Res. .

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.

Keywords: Primary cardiac angiosarcoma; Prognostic factors; SEER.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Analysis of incidence trend of primary cardiac angiosarcoma (incidence per 100,000) from 1976 to 2015
Fig. 2
Fig. 2
Analysis of mortality trend of primary cardiac angiosarcoma (incidence per 100,000) from 1976 to 2015
Fig. 3
Fig. 3
Kaplan–Meier estimated DSS in patients with primary cardiac angiosarcoma stratified by age (a), radiation (b), surgery (c), tumour size (d). DSS disease-specific survival
Fig. 4
Fig. 4
a Nomograms predicting 0.5-, 1-, 2- and 3-year DSS (b) of patients with primary cardiac angiosarcoma. b Calibration plots of the nomogram for 1-year DSS prediction. X-axis represents the nomogram-predicted probability of survival; Y-axis represents the actual OS probability. The blue circle overlaps the light blue line indicating near perfect calibration. Dots with bars represent nomogram-predicted probabilities along with 95% confidence interval
See this image and copyright information in PMC

References

    1. Fayette J, et al. Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases. Ann Oncol. 2007;18(12):2030–2036. - PubMed
    1. Fury MG, et al. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy. Cancer J. 2005;11(3):241–247. - PubMed
    1. Tran LM, et al. Sarcomas of the head and neck. Prognostic factors and treatment strategies. Cancer. 1992;70(1):169–177. - PubMed
    1. Kim CH, et al. Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience. Hum Pathol. 2008;39(6):933–938. - PubMed
    1. Zhang PJ, et al. Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol. 2008;39(9):1385–1395. - PMC - PubMed

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