title: From the archives of the AFIP: Pleuropulmonary synovial sarcoma.
abstract: 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.
authors.affiliation: Department of Radiologic Pathology, Armed Forces Institute of Pathology, 14th St and Alaska Ave NW, Washington, DC 20306-6000, USA. frazier@afip.osd.mil
authors.forename: Aletta Ann
authors.initials: AA
authors.lastname: Frazier
authors.forename: Teri J
authors.initials: TJ
authors.lastname: Franks
authors.forename: Robert D
authors.initials: RD
authors.lastname: Pugatch
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Galvin
mesh_terms: 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*
chemical_list: D003287:Contrast Media