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Pericardial Synovial Sarcoma: Challenges in Diagnosis and Management FREE TO VIEW

Jagadish Khanagavi, MD; Wilbert Aronow, MD; Carmelo Puccio, MD; Delong Liu, MD
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New York Medical College, Valhalla, NY

Chest. 2013;144(4_MeetingAbstracts):620A. doi:10.1378/chest.1703943
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

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

INTRODUCTION: Primary pericardial synovial sarcoma is an extremely rare tumor with very poor prognosis. Recent evidence suggests that timely diagnosis and aggressive multimodal management may substantially improve patient outcome. We present our institutional experience of management of only the second reported case with a favorable outcome amongst the fifteen reported so far.

CASE PRESENTATION: 27-year-old male presented with dyspnea and cough of three weeks. Exam revealed tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE) noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed Tomography (CT) of chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Morphological and immuno-histochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent In-Situ Hybridization (FISH) was positive for SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Workup for metastases was negative. High dose Ifosfamide was used in preoperative and postoperative adjuvant chemotherapy along-with local radiation leading to substantial reduction in the size of tumor. Patient underwent curative surgical resection and is doing well.

DISCUSSION: Diagnosis and management of pericardial synovial sarcoma can pose quite a challenge. Multiple modalities of cardiac imaging must be employed if there is suspicion for a pericardial mass. Cardiac sarcomas may elude diagnosis on TTE, and CT and Magnetic Resonance Imaging (MRI) are useful. Open tumor biopsy is often needed to establish diagnosis and extent of tumor. Treatment of synovial sarcoma is not well established. Neo-adjuvant chemotherapy with high dose Ifosfamide as used in the treatment of our patient has shown impressive results. Local radiation therapy is useful in reducing the size of the tumor prior to attempt for curative surgical resection.

CONCLUSIONS: Multi-modal approach to diagnosis and aggressive treatment as outlined in our case report can lead to favorable outcome in management of this rare tumor with dismal prognosis.

Reference #1: Moorjani N, Peebles C, Gallagher P, Tsang G. Pericardial Synovial Sarcoma. Journal of Cardiac Surgery 2009; 24: 349-351

Reference #2: Van der Mieren G, Willems S, Sciot R et al. Pericardial synovial sarcoma: 14-year survival with multimodality therapy. Ann Thorac Surg 2004; 78: e41-e42

Reference #3: Cheng Y, Sheng W, Zhou X, Wang J. Pericardial synovial sarcoma, a potential for misdiagnosis: clinicopathologic and molecular cytogenetic analysis of three cases with literature review. Am J Clin Pathol. 2012 Jan; 137: 142-149

DISCLOSURE: The following authors have nothing to disclose: Jagadish Khanagavi, Wilbert Aronow, Carmelo Puccio, Delong Liu

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