Open AccessHuge primary mediastinal synovial sarcoma fully occupying the right hemithorax
Author(s) -
Ahmed AbuZaid,
Asma Alnajjar,
Sarah Alotaibi,
Rasha Alshawaf,
Noor Alqeshtaini,
Rwan Alhaidar,
Shamayel Mohammed,
Khaled Alkattan
Publication year - 2018
Publication title -
journal of cancer research and therapeutics/journal of cancer research and therapeutics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.475
H-Index - 39
eISSN - 0973-1482
pISSN - 1998-4138
DOI - 10.4103/0973-1482.172137
Subject(s) - medicine , synovial sarcoma , mediastinum , differential diagnosis , sarcoma , radiology , radiation therapy , surgery , pathology
Primary mediastinal synovial sarcomas are exceedingly uncommon tumors. Herein, we present the case of primary mediastinal synovial sarcoma (monophasic spindle cell-shaped variant) fully occupying the right hemithorax in a 37-year-old woman who presented to clinic with a 2-month history of right-sided chest pain and shortness of breath. Although extremely rare, however, synovial sarcoma should be considered in the differential diagnosis of all monophasic and biphasic spindle cell neoplasms of the mediastinum. Despite molecular testing for (t[x; 18] [p11.2; q11.2]) is characteristically positive in 90% of synovial sarcoma cases, it is not routinely done. Histopathological and immunohistochemical analyses can greatly confirm the diagnosis. Optimal surgical resection is the standard of care. Adjuvant therapy (radiotherapy and/or chemotherapy) is indicated in inoperable advanced disease or unachieved surgical tumor-free surgical margins. Prognosis is poor with a 5-year overall survival (OS) rate of 35.7%. Early diagnosis and prompt appropriate management yield better disease-free and OS rates.