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Literature concerning primary pulmonary sarcomas (PPS) is limited to small case series. This study examines, in a large cohort, the clinical characteristics and therapeutic strategies of PPS and their impact on overall survival (OS).
This was a retrospective analysis from the Surveillance, Epidemiology, and End Results database (1988–2008). Eligible patients had primary PPS and underwent local therapy. Survival estimates were obtained using the Kaplan–Meier method and the Cox regression model. OS of PPS patients were compared with a cohort of 10,909 patients with extremity soft-tissue sarcomas.
The cohort included 365 PPS patients with a median follow-up of 21 months. Fifty-five percent of the patients had large tumors (>5 cm), 76% were high-grade, and 16% had node-positive disease. Seventy-five percent of the cohort underwent surgery alone, 14% underwent surgery and radiation therapy, and 11% underwent radiation therapy alone. Multivariate analysis showed reduced OS for patients with tumors more than 5 cm (hazard ratio [HR] 1.6, 95% confidence interval [CI] 1.25–2.19), high tumor grade (HR 3.1, 95% CI 1.26–3.62), and unresectable disease (HR 2.6, 95% CI 1.76–3.88. The 5-year OS for the cohort of pulmonary sarcomas versus sarcomas of the extremities was 35% versus 71% (p < 0.0001).
This large study examining PPS patients reveals a high rate of nodal involvement and a markedly worse OS than patients with extremity soft-tissue sarcomas. Thus, given the poor overall prognosis, it is recommended that PPS patients undergo a thorough mediastinal nodal evaluation to rule out locoregional metastasis and proceed with aggressive treatment.