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. 2020 Aug 26;20(1):811.
doi: 10.1186/s12885-020-07323-0.

Epidemiological features and survival outcomes in patients with malignant pulmonary blastoma: a US population-based analysis

Xiang Bu  1   2 , Jing Liu  2   3 , Linyan Wei  2   3 , Xiqiang Wang  2   3 , Mingwei Chen  4
Affiliations

Epidemiological features and survival outcomes in patients with malignant pulmonary blastoma: a US population-based analysis

Xiang Bu et al. BMC Cancer. .

Abstract

Background: Pulmonary blastoma (PB) is a rare lung primary malignancy with poorly understood risk factors and prognosis. We sought to investigate the epidemiologic features and long-term outcomes of PB.

Methods: A population-based cohort study was conducted to quantify the death risk of PB patients. All subjects diagnosed with malignant PB from 1988 to 2016 were screened from the Surveillance, Epidemiology and End Results database. Cox regression model of all-cause death and competing risk analysis of cause-specific death were performed.

Results: We identified 177 PB patients with a median survival of 108 months. The 5 and 10-year survival rate in all PB patients were 58.2 and 48.5%, as well as the 5 and 10-year disease-specific mortality were 33.5 and 38.6%. No sex or race disparities in incidence and prognosis was observed. The death risk of PB was significantly associated with age at diagnosis, clinical stage, histologic subtype and surgery treatment (p<0.01). On multivariable regression analyses, older age, regional stage and no surgery predicted higher risk of both all-cause and disease-specific death in PB patients.

Conclusion: We described the epidemiological characteristics of PB and identified its prognostic factors that were independently associated with worse clinical outcome.

Keywords: Competing-risk model; Long-term prognosis; Pulmonary blastoma; SEER database.

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Conflict of interest statement

I would like to declare on behalf of my co-authors that this article was original and all the authors listed have approved the manuscript that is submitted. No conflict of interest exists in the submission of this manuscript.

Figures

Fig. 1
Fig. 1
Forest plot of HR for all-cause death in PB patients. A total of 177 patients with malignant PB were stratified by different factors. HRs and 95% CIs for all-cause death in different stratifications were calculated using Cox models, with the first subgroup as reference. The p values were for the difference between subgroups in each stratification. All tests were 2-sided, and statistical significance was set as p-value of < 0.05. *, ** and *** indicated p<0.05, p<0.01 and p<0.001 respectively (R program, Version 3.6.3, R core team). PB, pulmonary blastoma; PPB, pleuropulmonary blastoma; WDFA, well-differentiated fetal adenocarcinoma; CBPB, classic biphasic PB; HR, hazard ratio; CI, confidence interval
Fig. 2
Fig. 2
Kaplan–Meier survival plots for PB patients. Kaplan–Meier plots for overall survival of PB patients stratified by (a) age at diagnosis, (b) histologic subtype, (c) clinical stage and (d) surgery or not. The p values for comparison of the cumulative survival probability in different stratifications were calculated using Log Rank (Mantel-Cox) test (R program, Version 3.6.3, R core team). PB, pulmonary blastoma; PPB, pleuropulmonary blastoma; WDFA, well-differentiated fetal adenocarcinoma; CBPB, classic biphasic PB
Fig. 3
Fig. 3
Cumulative incidence of cause-specific death for PB patients. The cumulative incidence of cause-specific death for (a) overall patients and stratified patients by (b) age at diagnosis, (c) clinical stage, (d) histologic subtype, (e) surgery or not and (f) the number of primary malignancy. The p values for comparison of the cumulative incidence functions according to different stratification are based on Gray’s test (R program, Version 3.6.3, R core team). PB, pulmonary blastoma; PPB, pleuropulmonary blastoma; WDFA, well-differentiated fetal adenocarcinoma; CBPB, classic biphasic PB
See this image and copyright information in PMC

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