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.

Primary Pulmonary Synovial Sarcoma: A Very Rare Presentation

“…The presented case has a well-delineated peripheral mass in the chest X-ray which was compatible with the earlier reported series done by Frazier et al in which 78% of the cases have lesions with well-delineated borders [6]. Also the lesion has no calcification or cavitations which were consistent with the same series of results and the other hemithorax was normal.…”

“…CT demonstrates a well-defined homogeneous or heterogeneous enhancing mass containing necrotic areas and soft tissue components [4]. In the CT evaluation of the case, a wide pleural based localization of the mass was reported to be seen as in 73% of the cases by Frazier et al [6]. Ipsilateral pleural effusion is common, which was not seen in our case.…”

“…In our case, no bone destruction was detected but the tumor located intrathoracically and extrapulmonary has extension to the chest wall. No chest wall invasion was documented in the series by Frazier et al while Tateishi et al reported that infiltration to the adjacent tissue could occur [6, 7]. Radiologically, compared with soft tissue SS, primary pulmonary and mediastinal SS shows less vascularity, hemorrhage, and necrosis on MR [4].…”

Synovial sarcoma: CT imaging of a rare primary malignant tumour of the thorax

“…Radiological descriptions of the tumour have generally been rare [7,8] and mostly contained in reports regarding immunohistochemical diagnosis. Only recently, Frazier et al [9] reviewed the imaging features of 12 cases of PSSL.…”

“…La descrizione radiologica della neoplasia è stata saltuaria [7,8], per lo più presente in lavori relativi alla sua diagnosi immunoistochimica. Solo recentemente Frazier et al [9] hanno revisionato l'imaging di 12 pazienti con PPSS. In questo lavoro, la radiografi a standard del torace dimostra principalmente una massa parenchimale a margini sia irregolari che netti, a base pleurica.…”