Primary pulmonary sarcomas are a rare group of pulmonary malignancies, outnumbered by bronchogenic carcinomas by a factor of 500:1. These tumors are often difficult to differentiate and characterize on the basis of morphology alone, and diagnosis frequently depends upon immunohistochemical and cytogenetic analysis. Primary pulmonary sarcomas are typically very advanced at the time of diagnosis, with symptoms related to tumor invasion or postobstructive pneumonia commonly prompting evaluation. Some patients, however, are asymptomatic at the time of diagnosis, and primary pulmonary sarcoma is an incidental finding on an imaging study. The clinical symptoms and radiographic findings of primary pulmonary sarcoma are myriad and can resemble many other disease entities, so primary pulmonary sarcoma needs to be entertained in the differential diagnosis during evaluation of other diseases. Complete surgical resection remains the only therapeutic approach for cure but due to advanced stage at diagnosis, many patients are not eligible for resection. Chemotherapy and radiation therapy are important for treatment but newer, more efficacious therapeutic agents are needed.
- Epithelioid hemangioendothelioma
- Kaposi sarcoma
- Pulmonary sarcoma
- Solitary fibrous tumor/hemangiopericytoma
- Synovial sarcoma
ASJC Scopus subject areas
- General Medicine