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Case Reports in Oncological Medicine
Volume 2015, Article ID 651813, 4 pages
http://dx.doi.org/10.1155/2015/651813
Case Report

Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

1GCRI, Civil Hospital Complex, Asarwa, Ahmedabad, Gujarat 830016, India
2Department of Radiotherapy & Clinical Oncology, Swami Rama Cancer Hospital & Research Institute, Haldwani, Uttarakhand, India
3Department of Pulmonary Medicine, IGMC, Shimla, Himachal Pradesh, India

Received 4 December 2014; Revised 21 January 2015; Accepted 15 February 2015

Academic Editor: Jorg Kleeff

Copyright © 2015 Irappa Madabhavi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH) demonstrated the t(X:18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.