Primary pulmonary synovial sarcoma

Primary pulmonary synovial sarcoma is an extremely rare tumour 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 tumours 1.

The diagnosis can only be established only after sarcoma like primary lung malignancies and metastatic sarcoma has been confidently excluded.

Subtypes

There are four recognised pathological subtypes

  • monophasic fibrous
  • monophasic epithelial
  • biphasic
  • poorly differentiated
General

They can occur anywhere ranging from peripheral to central.

CT chest

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 co-workers as a distinctive primary lung sarcoma, but sharing histological and immunohistochemical features with monophasic synovial sarcomas of soft tissue 2.

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Article Information

rID: 41011
System: Chest
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Primary pulmonary synovial sarcomas
  • Primary synovial sarcoma of lung
  • Primary synovial sarcoma of the lung

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