Open AccessPrimary synovial sarcoma of the mediastinum: a poor prognosis in a 14‐year‐old girl
Author(s) -
Saad Asma,
Bouacida Imen,
Ben Radhia Bechir,
Zribi Hazem,
Dridi Amira,
Marghli Adel
Publication year - 2021
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.811
Subject(s) - medicine , synovial sarcoma , mediastinum , superior vena cava syndrome , sarcoma , radiology , spindle cell sarcoma , pathology , superior vena cava
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.