Department of Surgical Oncology, University of Toronto, Toronto, ON, Canada.
Grant Medical Center, Columbus, OH, USA.
World J Surg Oncol. 2017 Aug 30;15(1):168. doi: 10.1186/s12957-017-1223-3.
Tumor embolisms (TE) are an underappreciated source of pulmonary embolisms in sarcoma. Most evidence in the literature is limited to case reports and none have described the presence of TE secondary to myxofibrosarcoma. We report the first case of myxofibrosarcoma TE and perform a review of the literature for TE secondary to bone and soft tissue sarcomas (STS).
A 36-year-old female presented with debilitating pain of the right upper extremity secondary to a recurrent soft tissue sarcoma. She had distant metastasis to the lung. An MRI revealed a 25-cm shoulder mass involving the proximal arm muscles with encasement of the axillary artery, vein, and brachial plexus. A palliative forequarter amputation was performed and tumor thrombus was evident within the axillary artery and vein. Postoperatively, she developed an acute onset of dyspnea and hypoxia. A computed tomography scan revealed a pulmonary saddle embolism. A bilateral lower extremity venous duplex was negative. She became hemodynamically unstable despite resuscitation and was placed on vasopressor support. A transthoracic echocardiogram revealed elevated pulmonary artery pressure, tricuspid regurgitation, right heart dilation, and reduced right heart systolic function consistent with acute cor pulmonale. The patient did not want to pursue a median sternotomy with pulmonary artery embolectomy and expired from cardiopulmonary arrest within 24 h of the operation. The final pathology revealed a 25 × 16 × 13 cm high-grade myxofibrosarcoma with invasion into the bone, skin, and neurovascular bundle as well as evidence of tumor thrombus.
TE is a rare but deadly cause of pulmonary embolism in sarcoma. A high index of suspicion is necessary in individuals who present with respiratory-related symptoms, especially dyspnea. Diagnostic confirmation with a computed tomography scan of the chest and echocardiogram should be rapid. Unlike venous thromboembolism, pulmonary embolectomy remains the preferred therapeutic approach.
肿瘤栓子(TE)是肉瘤肺栓塞被低估的来源。文献中的大多数证据仅限于病例报告,没有一个描述过黏液纤维肉瘤继发 TE 的存在。我们报告首例黏液纤维肉瘤 TE,并对骨和软组织肉瘤(STS)继发 TE 的文献进行了回顾。
一名 36 岁女性因复发性软组织肉瘤出现右上肢无力性疼痛就诊。她已有肺部远处转移。MRI 显示肩部有 25cm 大小的肿块,累及近端手臂肌肉,腋窝动脉、静脉和臂丛神经被包裹。进行了姑息性前四分之一截肢术,腋窝动脉和静脉内可见肿瘤血栓。术后,她突发呼吸困难和缺氧。计算机断层扫描显示肺鞍部栓塞。双侧下肢静脉双功超声检查为阴性。尽管进行了复苏,她仍出现血流动力学不稳定,并接受升压药物支持。经胸超声心动图显示肺动脉压升高、三尖瓣反流、右心扩张和右心收缩功能降低,符合急性肺心病。患者不想接受正中开胸肺动脉取栓术,并在手术后 24 小时内死于心肺骤停。最终病理显示 25×16×13cm 大小高级别黏液纤维肉瘤,侵犯骨骼、皮肤和神经血管束,并有肿瘤血栓证据。
TE 是肉瘤肺栓塞罕见但致命的原因。对于出现呼吸相关症状,尤其是呼吸困难的患者,需要高度怀疑。通过胸部 CT 扫描和超声心动图快速确诊。与静脉血栓栓塞症不同,肺动脉取栓术仍然是首选的治疗方法。