Majeski J, Crawford E S, Majeski E I, Duttenhaver J R
Department of Surgery, Medical University of South Carolina, Charleston, USA.
J Vasc Surg. 1998 Mar;27(3):555-8. doi: 10.1016/s0741-5214(98)70333-7.
Primary tumors of the aorta are rare and are difficult to diagnose preoperatively. These tumors are malignant and ultimately fatal. A patient had initial evidence of aortoiliac obstructive disease. Aortic endarterectomy produced an excellent postoperative clinical result until the pathology report from the endarterectomy specimen revealed aortic sarcoma. The patient refused a second operation for en bloc aortic resection. One year later with recurrent symptoms, the patient underwent en bloc resection of the aorta and common iliac vessels until clear margins were obtained. The tumor was found to be intimal aortic sarcoma of endothelial cell origin. The only metastasis was to bone, and it was controlled with radiation and chemotherapy. The patient lived a total of 8 years from the time of initial diagnosis. Abdominal aortic intimal sarcoma of the endothelial cell type is a rare variety of aortic sarcoma that can be managed successfully if diagnosed early. This specific type of tumor has a longer survival period and better prognosis than other types of aortic sarcoma when managed with aggressive surgical resection, radiation, and chemotherapy.
主动脉原发性肿瘤罕见，术前难以诊断。这些肿瘤为恶性，最终会导致死亡。一名患者最初有腹主动脉髂动脉阻塞性疾病的迹象。主动脉内膜切除术术后临床效果良好，直到内膜切除标本的病理报告显示为主动脉肉瘤。患者拒绝接受第二次主动脉整块切除术。一年后，患者因症状复发，接受了主动脉和髂总血管整块切除术，直至切缘清晰。肿瘤为内皮细胞起源的主动脉内膜肉瘤。唯一的转移部位是骨骼，通过放疗和化疗得到了控制。从最初诊断起，患者共存活了8年。内皮细胞型腹主动脉内膜肉瘤是主动脉肉瘤中一种罕见的类型，如果早期诊断，可成功治疗。与其他类型的主动脉肉瘤相比，这种特定类型的肿瘤在接受积极的手术切除、放疗和化疗后，生存期更长，预后更好。
