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Primary pleuropulmonary sarcoma: what we know about it?


With the word ‘primary pleuropulmonary sarcoma’ we consider a heterogeneous group of tumors derived from cells of mesenchymal origin, which could arise from the lung and pleura. Primary chest wall sarcomas are rare, representing 0.2% of all lung cancers and 5% of all thoracic neoplasms [1]. As often happens with a rare disease, available data are derived from small cohorts of patients and often from single institution experiences and, frequently, from retrospectives analyses. Furthermore, unlike soft tissue and bone sarcomas, there are not specific guidelines dedicated for this disease, leaving to every center experience the patient’s charge. What are the major changes observed in the recent years and which are the open questions about this disease?

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