Key result
Intravascular low-grade myofibroblastic sarcoma was the only subtype of primary pulmonary artery sarcoma associated with long-term cure without evidence of disease (66.7% vs 0%).
Population
43 patients with primary pulmonary artery sarcomas with primary intraluminal growth
Design
Case_series
Follow-up
long-term (>3 years)
Authors
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May prompt sarcoma consideration in recurrent pulmonary embolism; extends limited histologic-outcome data but leaves management questions open.
Observational (n=43)
Absolute Event Rate: 66.7% vs 0%
Survival beyond 3 years is possible for primary pulmonary artery sarcoma, but cure without evidence of disease is currently possible only for the select subtype of intravascular low-grade myofibroblastic sarcoma.
Távora et al. (2008) conducted an observational in Primary pulmonary artery sarcoma (n=43). Low-grade myofibroblastic sarcoma subtype vs. Other histologic subtypes was evaluated on Cure with no evidence of disease at long-term follow-up. Intravascular low-grade myofibroblastic sarcoma was the only subtype of primary pulmonary artery sarcoma associated with long-term cure without evidence of disease (66.7% vs 0%).
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