Primary synovial sarcoma of the mediastinum: a poor prognosis in a 14‐year‐old girl

• Published in: Respirology case reports Vol. 9; no. 8; pp. e00811 - n/a
• Main Authors: Saad, Asma, Bouacida, Imen, Ben Radhia, Bechir, Zribi, Hazem, Dridi, Amira, Marghli, Adel
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01-08-2021; John Wiley & Sons, Inc; Wiley
• Subjects: Biopsy; Case Report; Case Reports; Chemotherapy; Dyspnea; Immunochemistry; Localization; mediastinum; prognosis; Radiation therapy; Sarcoma; synovial sarcoma; Thoracic surgery; Tumors; Vertebrae; synovial sarcoma; Immunochemistry; mediastinum; prognosis
• ISSN: 2051-3380; 2051-3380
• DOI: 10.1002/rcr2.811

Synovial sarcoma is a soft tissue sarcoma. Its occurrence as a primary mediastinal neoplasm is very rare. We report a case of mediastinal synovial sarcoma in a 14‐year‐old girl with exertive dyspnoea at presentation. On physical examination, there was a superior vena cava syndrome and café‐au‐lait spots. Chest X‐ray revealed a white left hemithorax with mediastinal deviation to the opposite side. Thoraco‐abdomino‐pelvic scanner showed a voluminous posterior cervico‐mediastinal tissue mass. Computed tomography (CT)‐guided biopsy of the mediastino‐pleural mass was performed. Histological examination reported fusocellular malignant mesenchymal proliferation. A complementary immunohistological study with a broad range of antibodies was performed with a high‐grade single‐phase spindle cell synovial sarcoma of the mediastinum. She presented a respiratory distress, did not respond to resuscitation, and died. Mediastinal synovial sarcoma is a rare tumour that is difficult to diagnose. Its slow progression and delay in diagnosis may lead to a fatal evolution.
Synovial sarcoma is a soft tissue sarcoma. Its occurrence as a primary mediastinal neoplasm is very rare. We report a fatal case of mediastinal synovial sarcoma in a 14‐year‐old girl with exertive dyspnoea at presentation. We highlight the role of imaging, the means of diagnosis, and the importance of the speed of therapeutic management. Mediastinal synovial sarcoma is a rare tumour that is difficult to diagnose. Its slow progression and delay in diagnosis may lead to a fatal evolution.