From the archives of the AFIP: Pleuropulmonary synovial sarcoma.
Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities; affects young and middle-aged adults, with no difference in distribution between the sexes; and has well-documented radiologic manifestations. PPSS may arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based either in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadolinium-based contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both.
Frazier
Aletta Ann
AA
Department of Radiologic Pathology, Armed Forces Institute of Pathology, 14th St and Alaska Ave NW, Washington, DC 20306-6000, USA. frazier@afip.osd.mil
Franks
Teri J
TJ
Pugatch
Robert D
RD
Galvin
Jeffrey R
JR
D003287:Contrast Media; D006801:Humans; D007089:Image Enhancement / Q000379:methods*; D008175:Lung Neoplasms / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D010997:Pleural Neoplasms / Q000175:diagnosis*; D017410:Practice Guidelines as Topic; D010818:Practice Patterns, Physicians'; D013584:Sarcoma, Synovial / Q000175:diagnosis*
D003287:Contrast Media