Zhang Hongyi, Kong Yalin, Zhang Hui, He Xiaojun, Zhang Hong-yi, Liu Chengli, Xiao Mei, Xu Xinbao
Department of Hepatobiliary Surgery, Air Force General Hospital, Beijing, China.
Ann Vasc Surg. 2010 Apr;24(3):417.e5-9. doi: 10.1016/j.avsg.2009.07.032. Epub 2009 Dec 29.
Leiomyosarcoma of the inferior vena cava (IVC) is an extremely rare malignancy with poor prognosis due to late diagnosis. Surgical resection currently remains the best treatment; however, recurrence frequently occurs and the 5-year survival rate is only 31%. The aim of this study is to report a case of IVC leiomyosarcoma and treatment of recurrence with repeat surgery. A 36-year-old woman with a high-grade leiomyosarcoma originating from the infrahepatic IVC underwent an en bloc excision of the tumor. Eleven months after the initial operation, two metastases to the omentum were observed. Since the patient showed no response to adjuvant chemotherapy (i.e., a combination of 5-fluorouracil and gemcitabine), repeat operations were used as the main treatment modality for recurrence. The median time to recurrence was 15 months (range 8-27). The middle and upper IVC segments were involved in the local recurrence, and metastatic lesions occurred in multiple sites including the stomach, omentum, mesentery, left liver, and pelvic cavity. Repeat operations to remove the recurrent and metastatic tumors led to a long-term (at least 7 years) survival, and the patient is still alive. Postoperative recoveries were uneventful. Neither complication related to the venous blood flow in the IVC nor renal impairment was noted. Our results suggest that in the setting of chemotherapy-refractory IVC leiomyosarcoma repeat surgery may be an alternative treatment for recurrence and improve survival time.
下腔静脉平滑肌肉瘤是一种极为罕见的恶性肿瘤，因其诊断较晚，预后较差。目前手术切除仍是最佳治疗方法；然而，复发频繁发生，5年生存率仅为31%。本研究旨在报告1例下腔静脉平滑肌肉瘤病例及复发性肿瘤的再次手术治疗情况。一名36岁女性，患有起源于肝下下腔静脉的高级别平滑肌肉瘤，接受了肿瘤整块切除术。初次手术后11个月，发现网膜有两处转移灶。由于患者对辅助化疗（即5-氟尿嘧啶与吉西他滨联合使用）无反应，因此将再次手术作为复发的主要治疗方式。复发的中位时间为15个月（范围8 - 27个月）。局部复发累及下腔静脉的中上部节段，转移灶发生于多个部位，包括胃、网膜、肠系膜、左肝和盆腔。再次手术切除复发和转移肿瘤使患者获得长期（至少7年）生存，患者至今仍存活。术后恢复顺利。未发现与下腔静脉血流相关的并发症及肾功能损害。我们的结果表明，对于化疗难治性下腔静脉平滑肌肉瘤，再次手术可能是复发性肿瘤的一种替代治疗方法，并可延长生存时间。
