Phatak Prajakta, Khanagavi Jagadish, Aronow Wilbert S, Puri Sonam, Yusuf Yasmin, Puccio Carmelo
Department of Medicine, Westchester Medical Center / New York Medical College, Valhalla, NY 10595, USA.
Division of Cardiology, Westchester Medical Center/ New York Medical College, Valhalla, NY 10595, USA.
F1000Res. 2014 Jan 17;3:15. doi: 10.12688/f1000research.3-15.v2. eCollection 2014.
Pericardial synovial sarcoma is an extremely rare tumor with poor prognosis. Timely diagnosis and aggressive multimodal management improves patient outcome. We present our experience of diagnosis and management of a young patient with monophasic synovial sarcoma arising from pericardium.
A 27-year-old man presented with dyspnea and cough of three weeks duration. Examination revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE) noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed tomography (CT) of the chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Histology and immunohistochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent in-situ hybridization (FISH) was positive for SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Work-up for metastases was negative. Neo-adjuvant chemotherapy with high dose ifosfamide led to substantial reduction in the size of the tumor. The patient underwent surgical resection and external beam radiation therapy (EBRT) post surgery. He had symptom-free survival for 8 months prior to local recurrence. This was managed with left lung upper lobectomy and follow-up chemotherapy with docetaxel. The patient is currently stable with an acceptable functional status.
In patients with pericardial effusions of unknown etiology, multiple modalities of cardiac imaging must be employed if there is suspicion of a pericardial mass. CT and magnetic resonance imaging (MRI) are useful to evaluate for pericardial thickening or masses in addition to TTE. Treatment of synovial sarcoma is not well established. Surgery is the cornerstone of treatment. In non-resectable tumors, aggressive neo-adjuvant chemotherapy with ifosfamide followed by surgical resection and EBRT may lead to improved outcome.
心包滑膜肉瘤是一种极其罕见的肿瘤，预后较差。及时诊断和积极的多模式治疗可改善患者预后。我们介绍了一名患有源于心包的单相滑膜肉瘤的年轻患者的诊断和治疗经验。
一名27岁男性，出现持续三周的呼吸困难和咳嗽。检查发现窦性心动过速、心音遥远和颈静脉压升高。胸部X线显示纵隔增宽。经胸超声心动图（TTE）发现大量心包积液伴心脏压塞征象。治疗性心包穿刺抽出了血性液体。胸部计算机断层扫描（CT）显示心包积液持续存在，左前纵隔有一肿块。行左前开胸术、心包开窗术和左前纵隔切开术，发现一个起源于心包的包膜完整的胶冻状肿瘤。组织学和免疫组化特征显示该肿瘤为单相滑膜肉瘤。荧光原位杂交（FISH）检测显示18号染色体q11区域的SS18（SYT）基因重排呈阳性，证实了诊断。转移灶检查为阴性。高剂量异环磷酰胺新辅助化疗使肿瘤大小显著缩小。患者接受了手术切除，并在术后接受了外照射放疗（EBRT）。在局部复发前，他无症状生存了8个月。通过左肺上叶切除术和多西他赛后续化疗对复发进行了处理。患者目前病情稳定，功能状态尚可。
对于病因不明的心包积液患者，如果怀疑有心包肿块，必须采用多种心脏成像方式。除TTE外，CT和磁共振成像（MRI）有助于评估心包增厚或肿块情况。滑膜肉瘤的治疗方法尚未完全确立。手术是治疗的基石。对于不可切除的肿瘤，采用异环磷酰胺积极进行新辅助化疗，随后进行手术切除和EBRT可能会改善预后。
