El-Gendi Ahmed, Fadel Shady, El-Shafei Mohamed, Shawky Ahmed
Department of Surgery, Faculty of Medicine, Alexandria University, Alexandria, Egypt.
Department of Medical and Radiation Oncology, Faculty of Medicine, Alexandria University, Alexandria, Egypt.
Pediatr Int. 2018 Sep;60(9):862-868. doi: 10.1111/ped.13634. Epub 2018 Aug 9.
Primary liver transplantation is recommended for central post-treatment extent of disease (POST-TEXT) III and IV hepatoblastoma. The aim of this study was to prospectively assess the safety and oncological efficacy of aggressive non-transplant extended hepatic resection in these patients.
A prospective study involved 18 children with central pretreatment extent of disease (PRETEXT) III and IV: three had primary liver transplantation whereas 15 underwent hepatic resection after neoadjuvant chemotherapy.
Median tumor volume was 317 mL (range, 135-546 mL). After four cycles of chemotherapy, POST-TEXT stage was III in 12 patients and IV in three patients. There was no perioperative mortality. Postoperative complications consisted of two bile leaks, one temporary decompensation and one sub-phrenic collection requiring drainage. One and 3 year disease-free survival was 93.3% and 73.3% respectively. The 3 year overall survival was 86.6%. Four patients developed recurrence, of whom two died. Early recurrence in 1 year occurred in one patient. All recurrences were distant metastases.
Extended major hepatic resection for selected cases of POST-TEXT III and IV hepatoblastoma is a technically challenging but feasible approach with acceptable morbidity and mortality rates. Oncological outcomes are similar to liver transplantation without the long-term commitment of immunosuppression or donor risk and morbidity, but a potential donor should always be organized on standby.
对于治疗后疾病范围(POST-TEXT)为III期和IV期的肝母细胞瘤,推荐进行原位肝移植。本研究的目的是前瞻性评估在这些患者中进行积极的非移植扩大肝切除术的安全性和肿瘤学疗效。
一项前瞻性研究纳入了18例治疗前疾病范围(PRETEXT)为III期和IV期的儿童:3例行原位肝移植,15例在新辅助化疗后接受肝切除术。
肿瘤体积中位数为317 mL(范围135 - 546 mL)。化疗四个周期后,12例患者的POST-TEXT分期为III期,3例为IV期。无围手术期死亡。术后并发症包括2例胆漏、1例暂时失代偿和1例需要引流的膈下积液。1年和3年无病生存率分别为93.3%和73.3%。3年总生存率为86.6%。4例患者出现复发,其中2例死亡。1例患者在1年内出现早期复发。所有复发均为远处转移。
对于POST-TEXT III期和IV期肝母细胞瘤的选定病例,扩大肝切除术是一种技术上具有挑战性但可行的方法,发病率和死亡率可接受。肿瘤学结局与肝移植相似,无需长期免疫抑制或承担供体风险及发病率,但应始终安排潜在供体待命。