Primary pulmonary synovial sarcoma is an extremely rare tumor and refers to a situation where a synovial sarcoma arises from the lung as a primary site. It together with a pleural synovial sarcoma comes under the broader category of pleuro-pulmonary synovial sarcomas 3.
It accounts for less than 0.5% of all lung tumors 1.
The diagnosis can only be established only after sarcoma like primary lung malignancies and metastatic sarcoma has been confidently excluded.
There are four recognized pathological subtypes
- monophasic fibrous
- monophasic epithelial
- poorly differentiated
They can occur anywhere ranging from peripheral to central.
Can occur in variable locations within the lung as described above. Typically seen a "well defined" mass 7. They can be heterogeneous enhancement post intravenous contrast 7.
Treatment and prognosis
The overall prognosis for primary pulmonary sarcomas are poor. In many cases a treatment of choice is surgical removal with view to achieve negative resection margins. Completeness of resection (but not size or grade) is thought to be a key factor associated with increased survival.
History and etymology
It was first described in 1995 by Zeren and colleagues as a distinctive primary lung sarcoma, but sharing histological and immunohistochemical features with monophasic synovial sarcomas of soft tissue 2.
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