The Journal of Respiratory Diseases.
No. 11
A CASE IN POINT
Primary synovial sarcoma presenting as an endobronchial mass
By SEEMA AGARWAL, MBBS, MD, BHAVNEESH SHARMA, MBBS, MD, VLADIMIR SABAYEV, MD, PAUL C. LEE, MD,
and RAMMOHAN GUMPENI, MD
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October 23, 2008
Dr Agarwal and Dr Sharma are postdoctoral fellows in pulmonary and critical care medicine, New York Hospital Queens, Weill Medical College of Cornell University, Flushing, New York. Dr Sabayev is assistant professor of pulmonary medicine, Weill Medical College of Cornell University. Dr Lee is assistant professor of cardiothoracic surgery and attending surgeon, NewYork-Presbyterian Hospital/Weill Cornell Medical Center, New York, and director of thoracic surgery, New York Hospital Queens. Dr Gumpeni is assistant professor of pulmonary medicine, Weill Medical College of Cornell University, and assistant program director, pulmonary and critical care medicine fellowship, New York Hospital Queens.
This tumor usually presents as a lung parenchymal mass. In the largest case series on thoracic sarcomas, most tumors presented as mediastinal, pulmonary, or pleural masses. Published cases of pulmonary parenchymal synovial sarcoma showed the tumor size to be between 2 and 16 cm.1-9 Most synovial sarcomas demonstrate epithelial markers, such as vimentin, cytokeratin, EMA, and CD99. Our patient's biopsy specimen also stained positive for BCL2. The biological significance of BCL2 protein expression in synovial sarcoma is that BCL2 is believed to be involved in regulating apoptosis in the tumor. Cytogenetic detection of the chimeric fusion gene SYT-SSX is a newer method of diagnosing synovial sarcoma. Reverse transcriptase polymerase chain reaction assays can be used for RNA extraction and gene amplification for identification of SYT-SSX gene.5 To the best of our knowledge, only 2 cases of endobronchial primary pulmonary sarcoma have been previously reported in the medical literature.10,11 The first reported case (published in German) described a 54-year-old man whopresented with a polypoidal endobronchial mass in the right upper lobe.10 Histological and immunohistochemical analysis showed a biphasic synovial sarcoma. Niwa and associates11 described a 42-year-old Japanese woman who presented with hemoptysis and was found to have a left-sided mainstem endobronchial mass. A final diagnosis of monophasic endobronchial pulmonary synovial sarcoma was made after histological and immunohistochemical testing. The patient underwent surgical resection of the tumor; this was followed by adjuvant chemotherapy. Patients with endobronchial lesions theoretically have a better prognosis because of early symptoms and presentation.8 Most pulmonary parenchymal sarcomas reported in the literature were treated with surgical resection. Lobectomy or pneumonectomy was done depending on the size of the tumor and the extent of involvement. Chemotherapy and/or radiotherapy was given postoperatively in few cases.1 The tumor is poorly responsive to chemotherapy, and recurrence is common.
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