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Mediastinal Synovial Sarcoma Presenting as SVC Syndrome and Airway Obstruction FREE TO VIEW

Jonathan Lawson, MD; Jeremy Anthony, MD; Adil Degani, MD; Thomas Dillard, MD
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Georgia Regents University, Augusta, GA

Chest. 2013;144(4_MeetingAbstracts):602A. doi:10.1378/chest.1704021
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SESSION TITLE: Cancer Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: : Synovial sarcoma (SS) is a rare malignant neoplasm that arises from primitive mesenchymal tissue that differentiates into epithelial or spindle cell types. These comprise 10% of soft tissue sarcomas, and occur most often in young adult males. They typically present as a slow growing mass affecting the lower extremities, shoulder or elbow. We report a rare mediastinal synovial sarcoma presenting as superior vena cava syndrome (SVCS) complicated by respiratory failure

CASE PRESENTATION: : A 44 year old black male with no significant past medical history presented with one week of progressive shortness of breath, and facial and upper extremity edema. Chest x-ray and CT scan confirmed an 11 cm mediastinal mass causing tracheal deviation and compression, superior vena cava compression, and a right subclavian thrombosis. Anticoagulation was initiated. He later developed wheezing, stridor, dysphagia, dyspnea, and required tracheal intubation. Tumor staging revealed no metastatic disease. Despite aggressive radiation, there was no reduction in tumor size. Bronchoscopy demonstrated continued tracheal compression and narrowing by mass effect. An open biopsy was performed. Immunohistologic testing revealed high grade malignant monophasic spindle cell tumor positive for BCL-2 and CD99. Molecular testing demonstrated rearrangement of the SYT gene region at 18q11.2. Tracheal stent placement was attempted prior to chemotherapy initiation but stent placement beyond the carina was not possible. Unfortunately the patient had increasing oxygenation requirements, developed cardiopulmonary arrest refractory to resuscitative measures, and expired.

DISCUSSION: Mediastinal tumor causing SVCS is an unusual presentation of SS. The challenge in this case is the proper diagnosis of a mediastinal tumor with the monophasic spindle cell variant. The differential diagnosis for a primary mediastinal mass includes sarcomatoid carcinoma, spindle cell thymoma, spindle cell thymic carcinoma and other rarer tumors. The presence of the fusion of SYT gene on chromosome 18 and SSX gene on chromosome X, has led to highly reliable diagnosis of synovial sarcoma in over 95% of cases.

CONCLUSIONS: The overall prognosis of SS is poor with 5-year survival rate of 36-64%. Recurrences are common after treatment. Our patient’s age, tumor variant, size, location, and mass effect all contribute to a very poor prognosis for this unusual soft tissue tumor.

Reference #1: Suster S, Moran CA. Primary synovial sarcomas of the mediastinum: a clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases. Am J Surg Pathol. 2005 May;29(5):569-78.

Reference #2: Trupiano JK, Rice TW, Herzog K, Barr FG, Shipley J, Fisher C, Goldblum JR. Mediastinal synovial sarcoma: report of two cases with molecular genetic analysis. Ann Thorac Surg. 2002 Feb;73(2):628-30.

DISCLOSURE: The following authors have nothing to disclose: Jonathan Lawson, Jeremy Anthony, Adil Degani, Thomas Dillard

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