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[儿童原发性肝肿瘤:62例连续病例分析]

[Primary liver tumors in children: analysis of 62 consecutive cases].

作者信息

Martínez L, López-Santamaría M, Gámez M, Murcia J, Tovar J A

机构信息

Departamento de Cirugía Pediátrica, Hospital Infantil La Paz, Paseo de la Castellana 261, 28046 Madrid.

出版信息

Cir Pediatr. 2000 Jul;13(3):110-5.

Abstract

Aiming at assessing the impact of the improvements introduced in the treatment of primitive liver tumors of children, we reviewed our experience with these tumors since 1980. Between January 1980 and June 1999 we treated 62 children with primitive liver tumors: 35 hepatoblastomas (HB), 5 hepatocarcinomas (HC), 4 sarcomas, 2 malignant mesenchymomas, 11 hemangioendotheliomas (HE), 3 mesenchymal hamartomas, 1 adenoma and 1 focal nodular hyperplasia. Thirty five were boys and 27 girls. The age at diagnosis was 34.5 +/- 43.1 months (mean +/- standard deviation). Eleven patients had HE and 4 of them (36.3%) died due to haemodynamic (n = 3) or other tumor-related causes. All the remaining patients with benign tumors survive. Among the HB patients, 3 had stage I, II stage II, 8 stage III and 13 stage IV tumors (with lung metastases at diagnosis in 4). For survival analysis we divided patients into two groups according to their treatment before (Group 1, n = 14) or after 1991 (Group 2, n = 21). Five-year actuarial survival was 49% for Group 1 and 78% for Group 2 (p < 0.05). We performed liver transplantation in 5 patients with 3 long-term survivals. Three children with widespread HC died whereas 2 with localized tumors had liver transplantation and survive. In conclusion, the treatment of primitive liver tumors in children requires collaborative protocols, concentration of patients in institutions capable of offering high-standard liver surgery and transplantation when the tumor is localized to the liver and irresectable.

摘要

为评估儿童原发性肝肿瘤治疗方法改进所带来的影响,我们回顾了自1980年以来对这些肿瘤的治疗经验。1980年1月至1999年6月期间,我们共治疗了62例儿童原发性肝肿瘤患者:35例肝母细胞瘤(HB)、5例肝细胞癌(HC)、4例肉瘤、2例恶性间叶瘤、11例血管内皮细胞瘤(HE)、3例间叶性错构瘤、1例腺瘤和1例局灶性结节性增生。其中35例为男孩,27例为女孩。诊断时的年龄为34.5±43.1个月(平均±标准差)。11例患者患有HE,其中4例(36.3%)因血流动力学原因(n = 3)或其他与肿瘤相关的原因死亡。其余所有良性肿瘤患者均存活。在HB患者中,3例为I期,2例为II期,8例为III期,13例为IV期肿瘤(4例诊断时伴有肺转移)。为进行生存分析,我们根据患者在1991年之前(第1组,n = 14)或之后(第2组,n = 21)的治疗情况将患者分为两组。第1组的5年精算生存率为49%,第2组为78%(p < 0.05)。我们对5例患者进行了肝移植,其中3例长期存活。3例广泛转移的HC患儿死亡,而2例局限性肿瘤患儿接受了肝移植并存活。总之,儿童原发性肝肿瘤的治疗需要协作方案,将患者集中在有能力在肿瘤局限于肝脏且无法切除时提供高标准肝脏手术和移植的机构。

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