Primary pulmonary synovial sarcoma

Last revised by Rohit Sharma on 7 Mar 2018

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
  • biphasic
  • 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.

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.

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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