Primary 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. Pleuropulmonary synovial sarcoma can 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 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 gadoliniumbased contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both.
Mirzoyan
Michael
M
Department of Medicine, Fairview Hospital, Cleveland, OH 44111, USA.
Muslimani
Ala'a
A
Setrakian
Sebouh
S
Swedeh
Mohamed
M
Daw
Hamed A
HA
D006801:Humans; D008175:Lung Neoplasms / Q000473:pathology* / Q000628:therapy; D010997:Pleural Neoplasms / Q000473:pathology* / Q000628:therapy; D013584:Sarcoma, Synovial / Q000473:pathology* / Q000628:therapy