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肝切除及自体肝移植作为下腔静脉II-III区平滑肌肉瘤的手术选择:1例病例报告及文献复习

Liver Resection and Autotransplantation as Surgical Option for Zone II-III Leiomyosarcoma of IVC: A Case Report and Literature Review.

作者信息

Tuxun Tuerhongjiang, Li Tao, Apaer Shadike, He Yi-Biao, Bai Lei, Gu Shen-Sen, Wang Zhi-Peng, Huo Qiang, Wang Jiang, Zhao Jin-Ming

机构信息

Department of Liver Transplantation & Liver Surgery, Center of Organ Transplantation, 1st Affiliated Hospital of Xinjiang Medical University, Urumqi, China.

Department of Cardiac Surgery, 1st Affiliated Hospital of Xinjiang Medical University, Urumqi, China.

出版信息

Front Oncol. 2021 Jun 11;11:690617. doi: 10.3389/fonc.2021.690617. eCollection 2021.

Abstract

We report the first documented case of leiomyosarcoma at zone II-III of inferior with thrombi in three hepatic veins undergoing liver resection and autotransplantation (ELRA) and hepatic veins thrombectomy. A 33-year-old female patient presented with abdominal distention and lower extremities edema. Abdominal wall varicosis and shifting dullness were positive on physical examination. Her liver function was classified as Child-Pugh B and a solid tumor at retro-hepatic extending to right atrium with thrombi in three hepatic veins were confirmed. The diagnosis of leiomyosarcoma with Budd-Chiari syndrome was highly suspected with preoperative ultrasound, echocardiogram, CT scan, and three-dimensional reconstruction. A zone II-III leiomyosarcoma of IVC origin was confirmed at surgery and liver resection and autotransplantation, and hepatic vein thrombectomy with atrial reconstruction were performed under cardiopulmonary bypass (CPB). Operative time, anhepatic time, and CPB time were 12 h, 128 min, and 84 min, respectively. The patients experienced post-operative liver dysfunction and was cured with conservative therapy. Hepatic recurrence two years after surgery was managed with radiofrequency. The patient was alive with liver metastasis three years after surgery. Despite being regarded as an extremely aggressive procedure, ELRA could be considered in the treatment of advanced leiomyosarcoma with Budd-Chiari syndrome and hepatic vein thrombi.

摘要

我们报告首例记录在案的下腔静脉II - III区平滑肌肉瘤病例,该患者三条肝静脉有血栓形成,接受了肝切除自体肝移植术(ELRA)及肝静脉血栓切除术。一名33岁女性患者出现腹胀和下肢水肿。体格检查发现腹壁静脉曲张和移动性浊音阳性。其肝功能分级为Child-Pugh B级,经证实肝后有一实性肿瘤延伸至右心房,三条肝静脉有血栓形成。术前超声、超声心动图、CT扫描及三维重建高度怀疑为平滑肌肉瘤合并布加综合征。手术中证实为下腔静脉起源的II - III区平滑肌肉瘤,在体外循环(CPB)下进行了肝切除自体肝移植术及肝静脉血栓切除并心房重建术。手术时间、无肝期时间及CPB时间分别为12小时、128分钟和84分钟。患者术后出现肝功能障碍,经保守治疗治愈。术后两年肝复发采用射频治疗。患者术后三年带肝转移存活。尽管ELRA被认为是一种极具挑战性的手术,但对于晚期平滑肌肉瘤合并布加综合征及肝静脉血栓形成患者,可考虑采用该治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0949/8226245/550848460999/fonc-11-690617-g001.jpg

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