Synovial Sarcoma, Lung

Synonyms

Historical terms such as synoviosarcoma; Synovial cell sarcoma and tenosynovial sarcoma are obsolete

Definition

A mesenchymal tumor which morphologically shows a variable degree of epithelial differentiation including gland formation and which is characterized by the presence of a t(X;18)(p11;q11) translocation.

Clinical Features

• Incidence

  Primary pulmonary synovial sarcomas are rare accounting for less than 0.1% of lung cancers.

• Age

  Synovial sarcomas can present at any age, but around 75% are seen in patients under the age of 50 with approximately 60% presenting in teenagers and young adults.

• Sex

  Males and females are equally affected.

• Site

  Most synovial sarcomas arise in deep soft tissues of the upper and lower extremities. Pulmonary synovial sarcomas are more likely to represent a metastasis from a primary elsewhere rather than being a true lung primary. In the lung tumors are most often peripheral but can arise in the endobronchial tree.

• Treatment

  Surgical resection with...