Abu-Zaid Ahmed, AlNajjar Asma, Alotaibi Sarah, Alshawaf Rasha, Alqeshtaini Noor, Alhaidar Rwan, Mohammed Shamayel, AlKattan Khaled
Department of Surgery, College of Medicine, Alfaisal University; Department of Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Department of Surgery, College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
J Cancer Res Ther. 2018 Apr-Jun;14(3):682-686. doi: 10.4103/0973-1482.172137.
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.
原发性纵隔滑膜肉瘤是极为罕见的肿瘤。在此，我们报告一例37岁女性原发性纵隔滑膜肉瘤（单相梭形细胞型变异），肿瘤完全占据右半胸，该患者因右侧胸痛和气短2个月就诊。尽管滑膜肉瘤极为罕见，但在纵隔所有单相和双相梭形细胞肿瘤的鉴别诊断中都应考虑到。尽管90%的滑膜肉瘤病例中特征性的（t[x; 18] [p11.2; q11.2]）分子检测呈阳性，但并非常规进行。组织病理学和免疫组化分析可极大地确诊。最佳手术切除是治疗标准。不可切除的晚期疾病或手术切缘未达到无瘤状态时，需行辅助治疗（放疗和/或化疗）。预后较差，5年总生存率（OS）为35.7%。早期诊断并及时进行恰当治疗可获得更好的无病生存率和总生存率。
