Gauthier F, Saliou C, Valayer J, Montupet P
Département de Pédiatrie, Hôpital de Bicêtre, Le-Kremlin-Bicêtre.
Chir Pediatr. 1988;29(6):307-12.
Twenty seven children aged 1 to 15 years were admitted from 1981 to 1987 for the investigation and/or the surgical treatment of a primary liver malignancy (25 hepatoblastomas and 2 hepatocellular carcinomas). All were submitted to preoperative multidrug chemotherapy. One child died from circulatory failure early after the diagnosis and two children considered to be non operable died also. A macroscopically satisfactory resection could be achieved in 24/27 cases. Sixteen out of these 24 children are alive and disease free, 11 of them with a follow-up over 2 years. Two of these 16 survivors underwent a second operation for resection of a local recurrence. Four children died after a massive unresectable hepatic relapse, two after a second relapse and a one after the onset of pulmonary metastases during her chemotherapeutic postoperative course. One further child is at yet in a critical situation because of a late small secondary hepatic relapse. Two factors contributed to improvement of survival an cure rates during the past ten years: 1. Routine preoperative chemotherapy, which may help in controlling the disease and greatly facilitates surgery by shrinking of massive tumors. 2. Better surgical management and especially the total hepatic vascular exclusion, used in 13 cases of this series. Three situations still have a poor outlook: 1. Hepatocellular carcinoma 2. Hepatoblastoma with a low response to chemotherapy 3. Multifocal tumors and those involving the main hepatic vessels. The feasibility of a liver transplantation in some selected cases is discussed.
1981年至1987年期间,27名年龄在1至15岁的儿童因原发性肝脏恶性肿瘤(25例肝母细胞瘤和2例肝细胞癌)的调查和/或手术治疗而入院。所有患儿均接受术前多药化疗。1名患儿在诊断后不久死于循环衰竭,另外2名被认为无法手术的患儿也死亡。27例中有24例实现了肉眼下满意的切除。这24名儿童中有16名存活且无疾病,其中11名随访时间超过2年。这16名幸存者中有2名因局部复发接受了二次手术切除。4名儿童在肝脏出现无法切除的巨大复发后死亡,2名在二次复发后死亡,1名在术后化疗过程中出现肺转移后死亡。另有1名儿童因晚期肝脏小的继发性复发而处于危急状态。在过去十年中,有两个因素有助于提高生存率和治愈率:1. 常规术前化疗,这可能有助于控制疾病,并通过缩小巨大肿瘤极大地促进手术。2. 更好的手术管理,特别是本系列13例中使用的全肝血管阻断。三种情况的预后仍然很差:1. 肝细胞癌 2. 对化疗反应低的肝母细胞瘤 3. 多灶性肿瘤以及累及肝主要血管的肿瘤。文中还讨论了在某些选定病例中进行肝移植的可行性。