Salah Samer, Al-Ibraheem Akram, Daboor Amal, Al-Hussaini Maysa
Department of Medical Oncology, King Hussein Cancer Center, Amman, Jordan.
BMC Res Notes. 2013 Jun 25;6:240. doi: 10.1186/1756-0500-6-240.
Synovial sarcoma presenting in the mediastinum is exceedingly rare. Furthermore, data addressing optimal therapy is limited. Herein we present a case where an attempt to downsize the tumor to a resectable state with chemotherapy was employed.
A 32 year female presented with massive pericardial effusion and unresectable huge mediastinal mass. Computed axial tomography scan - guided biopsy with adjunctive immunostains and molecular studies confirmed a diagnosis of synovial sarcoma. Following three cycles of combination Ifosfamide and doxorubicin chemotherapy, no response was demonstrated. The patient refused further therapy and had progression of her disease 4 months following the last cycle.
Synovial sarcoma presenting with unresectable mediastinal mass carry a poor prognosis. Up to the best of our knowledge there are only four previous reports where primary chemotherapy was employed, unfortunately; none of these cases had subsequent complete surgical resection. Identification of the best treatment strategy for patients with unresectable disease is warranted. Our case can be of benefit to medical oncologists and thoracic surgeons who might be faced with this unique and exceedingly rare clinical scenario.
纵隔滑膜肉瘤极为罕见。此外，关于最佳治疗方法的数据有限。在此，我们报告一例通过化疗将肿瘤缩小至可切除状态的病例。
一名32岁女性出现大量心包积液和无法切除的巨大纵隔肿块。计算机断层扫描引导下的活检及辅助免疫染色和分子研究确诊为滑膜肉瘤。接受三个周期的异环磷酰胺和阿霉素联合化疗后，未显示出反应。患者拒绝进一步治疗，在最后一个周期后4个月病情进展。
出现无法切除的纵隔肿块的滑膜肉瘤预后较差。据我们所知，此前仅有四份关于采用一线化疗的报告，遗憾的是，这些病例均未随后进行完整的手术切除。确定针对无法切除疾病患者的最佳治疗策略很有必要。我们的病例可能会对可能面临这种独特且极为罕见临床情况的肿瘤内科医生和胸外科医生有所帮助。
