Marcheix B, Dambrin C, Muscari F, Joseph-Hein K, Guimbaud R, Otal P
Service de Chirurgie et Cardio-Vasculaire A, CHU Rangueil -Toulouse.
J Chir (Paris). 2003 Jun;140(3):140-8.
Leiomyosarcoma of the inferior vena cava is a rare tumor of mesenchymal origin most commonly found in women. Clinical signs are non-specific. Imagery with ultrasound, CT, or MRI may strongly suggest the diagnosis, but it can only be confirmed by histologic examination of tissue obtained pre or intra-operatively. The tumor is slow growing but nonetheless carries a bad prognosis; it may grow to a large size before directly invading adjacent structures. Systemic spread is a late occurrence. Radical surgical resection is the only treatment which offers any hope for prolonged survival. Standard vascular surgical techniques are usually sufficient. Progress in the techniques of hepatectomy and liver transplantation have allowed the experienced surgeon to undertake the removal of retrohepatic lesions once considered unresectable. High-lying lesions adjacent to the hepatic veins or with thrombus extending into the proximal vena cava may require extracorporeal circulation with or without profound hypothermic circulatory arrest. The efficacy of chemotherapy, whether pre-operative for inaccessible tumors or post-operative for incompletely resected or recurrent tumor, is poorly defined and very limited.
下腔静脉平滑肌肉瘤是一种罕见的间叶源性肿瘤，多见于女性。临床症状不具特异性。超声、CT或MRI成像可能强烈提示诊断，但只有通过术前或术中获取的组织进行组织学检查才能确诊。该肿瘤生长缓慢，但预后不良；它可能在直接侵犯相邻结构之前长得很大。全身转移较晚出现。根治性手术切除是唯一有望延长生存期的治疗方法。标准的血管外科技术通常就足够了。肝切除术和肝移植技术的进步使经验丰富的外科医生能够切除曾经被认为无法切除的肝后病变。靠近肝静脉或血栓延伸至近端腔静脉的高位病变可能需要体外循环，可伴有或不伴有深度低温循环停搏。化疗的疗效，无论是对无法切除的肿瘤进行术前化疗，还是对不完全切除或复发肿瘤进行术后化疗，都不明确且非常有限。
