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Multimodality Therapy of Primary Mediastinal Synovial Sarcoma: Case Report FREE TO VIEW

Lucas Hoyos, MD; Daniel Valdivia, MD; Lidia Macias, MD; Andres Varela, PhD; David Gomez, PhD; Jose Luis Campo, MD
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Puerta de Hierro, Madrid, Spain

Chest. 2014;146(4_MeetingAbstracts):636A. doi:10.1378/chest.1994406
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SESSION TITLE: Cancer Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Synovial sarcoma are aggressive neoplasm accounting for up 14% of all soft tissue sarcomas (1), 80% arise in the extremities (2) and less 17% in the thorax (3). The complete resection is the only therapy associate with long term survival, uncertainly exists regarding the best therapeutic strategy with patients with initial unresectable disease. We present a patient with initial response to chemotherapy followed by a complete surgical resection.

CASE PRESENTATION: A 54-year-old woman presented with sub-acutea chest pain , along with dyspnea . She has past medical history of hypertension and diabetes on treatment whit espironolactone, valsartan and metformin. No abnormal finding on the physical exam. X Ray revealed a superior mediastinal widening, Computed tomography (CT) images showed a lobulated soft tissue mass in the right superior mediastinum. The mass was bulky, 8×5 cm in size, and in close relation whit the aorta, superior vena cava, right upper lobe of the lung and superior pulmonary vein (Fig. 1). A CT-guided biopsy was done for pathologic diagnosis. The biopsy showed neoplastic spindle-shaped cells whit low mitosis grade and the inmunohistochemical pattern showed E1A/E3+, EMA+, CD56+, BCL2+. CD34, ALC, Chromogranin and P53 was negative (Fig 2). The Fisher test reveled positive t(X:18)SSX1/SSX2.. A diagnosis of Mediastinal Synovial Sarcoma was made. The patient started with a neoadjuvance chemotherapy protocol (ifosfamide/adryamicin) and after 3 cycles the CT images showed partial response. En bloc complete resection, including thymus gland, pericardial patch and right upper lobe was performed(Fig 2). The patient made an uneventful recovery and was discharged home 5 day later. Our final pathologic diagnosis was primary mediastinal Synovial Sarcoma (monophasic fibrous type). No metastasic disease has been report at the time.

DISCUSSION: Synovial sarcoma is a very rare tumor, 80% of the cases arise in the extremities and that’s most of the experience and evidence of his treatment it is base on. Tumor size (< 5 cm) and complete resection are the most consistent prognosis factors. Other less consistently identified are bone, neurovascular invasion, hystolic grade and subtype. Because of is location, the tumor arise in the mediastinum at the time of the diagnosis the tumor are commonly larger in size and frequently involved pleura, pericardium and lung making most of the patients inoperable and poor prognosis. Recent systemic review of the literature showed a total of twenty-two publications, which include 40 patients. Complete resection was the most applied strategy 23 patient (57%) and only 8 eight of those patients received additional chemo or radiotherapy, the other 17 patient receive partial resection or chemo radiotherapy, After follow up 67% had progression of the disease and after univariate analysis, completeness of the resection was identified as the only associate with improvement in survive.

CONCLUSIONS: Mediastinal synovil sarcoma it a rare disease, have a poor prognosis as the tend to present with large tumor and whit advance stage. Completeness of the resection is the only identified factor that influence survival.

Reference #1: S. Salah, A. Al-Ibraheem, A. Daboor, and M. Al-Hussaini, “Synovial sarcoma presenting with huge mediastinal mass: a case report and review of literature,” BMC Research Notes, vol. 6, no. 1, p. 240, 2013.

Reference #2: S. Salah and A. Salem. Primary Synovial Sarcomas of the Mediastinum: A Systematic Review and Pooled Analysis of the Published Literature. ISRN Oncology Volume 2014, Article ID 412527, 7 pages

Reference #3: B. Henninger, M. Freund, B. Zelger et al., “Primary mediastina synovial sarcoma: a case report and review of the literature,” Cases Journal, vol. 2, no. 8, article 6948, 2009

DISCLOSURE: The following authors have nothing to disclose: Lucas Hoyos, Daniel Valdivia, Lidia Macias, Andres Varela, David Gomez, Jose Luis Campo

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