Primary Mediastinal Synovial Sarcoma; a rare case

Document Type : Case Report

Authors

1 Department of Radiology, Shiraz University of Medical Sciences, Shiraz, Iran

2 Department of Radiology, Rafsanjan University of Medical Sciences, Rafsanjan, Iran

Abstract

Background: Synovial sarcoma, a rare subtype of soft-tissue sarcomas, typically manifests in the extremities of young individuals. Mediastinal occurrences are exceedingly uncommon, with sparse cases documented.
Case: This article details the atypical presentation of a mediastinal synovial sarcoma in a 68-year-old female patient, diverging from the common demographic and anatomical predilections. Initial symptoms of a dry cough and fatigue led to the discovery of a mediastinal mass via chest CT scan. Subsequent pathological examination confirmed the diagnosis of mediastinal synovial sarcoma. Despite the identification of this rare tumor, the patient's condition progressed rapidly, resulting in her demise within two months post-diagnosis.
Conclusion: This case underlines the critical need for increased awareness and research into the mediastinal variant to improve diagnostic accuracy and treatment efficacy. Despite synovial sarcoma being predominantly identified in the extremities, occurrences in the mediastinum present unique diagnostic dilemmas and therapeutic hurdles due to limited literature and atypical clinical presentations. The utilization of advanced imaging modalities, immunostaining techniques, and multimodal treatment approaches is essential in the management of mediastinal synovial sarcoma.

Keywords


  1. Murphey MD, Gibson MS, Jennings BT, Crespo-Rodríguez AM, Fanburg-Smith J, Gajewski DA. Imaging of synovial sarcoma with radiologic-pathologic correlation. Radiographics. 2006;26(5):1543-65.
  2. Fletcher CD, Unni K, Mertens F. World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone: IARC press; 2002.
  3. Foo WC, Cruise MW, Wick MR, Hornick JL. Immunohistochemical staining for TLE1 distinguishes synovial sarcoma from histologic mimics. Am J Clin Pathol. 2011;135(6):839-44. Epub 2011/05/17. doi: 10.1309/ajcp45ssnaopxyxu. PubMed PMID: 21571956.
  4. Salter DM. Pulmonary and thoracic sarcomas. Current Diagnostic Pathology. 2006;12(6):409-17.
  5. Eilber FC, Dry SM. Diagnosis and management of synovial sarcoma. Journal of surgical oncology. 2008;97(4):314-20.
  6. Rossi S, Nascimento AG, Canal F, Dei Tos AP. Small round-cell neoplasms of soft tissues: an integrated diagnostic approach. Current Diagnostic Pathology. 2007;13(2):150-63.
  7. Miettinen M, Limon J, Niezabitowski A, Lasota J. Patterns of keratin polypeptides in 110 biphasic, monophasic, and poorly differentiated synovial sarcomas. Virchows Archiv. 2000;437:275-83.
  8. Hirakawa N, Naka T, Yamamoto I, Fukuda T, Tsuneyoshi M. Overexpression of bcl-2 protein in synovial sarcoma: a comparative study of other soft tissue spindle cell sarcomas and an additional analysis by fluorescence in situ hybridization. Human pathology. 1996;27(10):1060-5.
  9. Jagdis A, Rubin BP, Tubbs RR, Pacheco M, Nielsen TO. Prospective evaluation of TLE1 as a diagnostic immunohistochemical marker in synovial sarcoma. The American journal of surgical pathology. 2009;33(12):1743-51.
  10. Jadvar H, Gamie S, Ramanna L, Conti PS, editors. Musculoskeletal system. Seminars in nuclear medicine; 2004: Elsevier.
  11. Syred K, Weissferdt A. Primary mediastinal synovial sarcomas. Mediastinum. 2020;4.