Angelico Roberta, Passariello Annalisa, Pilato Michele, Cozzolino Tommaso, Piazza Marcello, Miraglia Roberto, D'Angelo Paolo, Capasso Mariella, Saffioti Maria Cristina, Alberti Daniele, Spada Marco
Department of Abdominal Transplantation and Hepatobiliary and Pancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Department of Translational Medical Science, University of Naples "Federico II", Naples, Italy; Department of Pediatric Oncology, Ospedale Santobono- Pausilipon, Naples, Italy.
Int J Surg Case Rep. 2017;37:90-96. doi: 10.1016/j.ijscr.2017.06.008. Epub 2017 Jun 13.
Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear.
A 11-months-old boy was referred for a right hepatic lobe mass(90×78mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61×64mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft.
We report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.
肝母细胞瘤伴肿瘤血栓延伸至下腔静脉和右心房通常无法切除，预后极差。手术入路在技术上具有挑战性，可能需要进行大范围肝切除并进行血管重建和体外循环。然而，最佳的手术技术尚不清楚。
一名11个月大的男孩因右肝叶肿块（90×78mm）就诊，怀疑为肝母细胞瘤，肿瘤血栓延伸至下腔静脉和右心房，双侧肺部有病变，血清甲胎蛋白水平为50.795IU/mL。经过8个月的化疗（SIOPEL 2004高危方案），肺部病变不再清晰可见，肝母细胞瘤大小降至61×64mm。因此，计划进行原位肝切除：肝实质离断后，开始低温心肺转流，整块切除扩大的右肝叶、肝后/肝上腔静脉和肿瘤血栓，同时对残余肝脏进行冷灌注。下腔静脉用来自血型匹配尸体供体的主动脉移植物替换。术后过程顺利，经过8个月的随访，患儿肝功能和甲胎蛋白水平正常，无疾病复发，血管移植物通畅。
我们首次报告了一例在肝母细胞瘤患儿中进行原位肝切除和下腔静脉置换并伴有低温心肺转流的病例。在此，我们广泛回顾了有关伴有肿瘤血栓的肝母细胞瘤的文献，并描述了原位入路的技术要点，这是在其他情况下无法切除的肝母细胞瘤的一种可行选择。
