Malignant hepatic tumours in children: incidence, clinical features and aetiology.
Malignant hepatic tumours in children: incidence, clinical features and aetiology.
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DOI：
10.1111/j.1365-3016.1990.tb00651.x
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发表时间：
1990-07
影响因子：
2.8
通讯作者：
Jillian R. Mann;N. Kasthuri;F. Raafat;J. Pincott;S. Parkes;K. Muir;L. Ingram;A. Cameron
中科院分区：
文献类型：
--
作者：
Jillian R. Mann;N. Kasthuri;F. Raafat;J. Pincott;S. Parkes;K. Muir;L. Ingram;A. Cameron
先天性缺陷和其他疾病与恶性肝肿瘤有关。为了评估其重要性，对1957-1986年30年间被诊断患有恶性肝肿瘤的15岁以下儿童进行了一项基于人口的调查。这些病例是从西米德兰兹地区儿童肿瘤登记处收集的信息中确定的。从他们的临床记录中提取相关数据，由三名儿科病理学家组成的小组审查原始活检和任何尸检材料。在登记的50例合格病例中，8例被排除，因为组织学检查显示他们患有非恶性疾病（3）或肝外起源的恶性肿瘤（4）或因为没有病理材料可用（1）。其余42例诊断为肝母细胞瘤（27例）、肝细胞癌（3例）、横纹肌肉瘤（6例）、横纹肌样瘤（3例）和卵黄囊瘤（3例）。原发性恶性肝脏肿瘤的发病率为1.20/10（6）人年，肝母细胞瘤亚组的发病率为0.77/10（6）人年（西米德兰兹郡的平均儿童人口为1，166，500人）。目前的临床，放射学和生化特征与其他系列报道的相似，种族和社会阶层分布与当地人群相比并不明显。9例（21%）患者存在先天性缺陷或其他可能相关的特征。我们的研究结果，与其他报告，表明肝母细胞瘤是一种恶性肿瘤有关的发育不良，可能与11 p或5 q突变，而肝细胞癌更通常是一种并发症的代谢和其他疾病，导致肝硬化。
Congenital defects and other disorders have been reported in association with malignant liver tumours. In order to assess their significance, a population-based survey was undertaken on children aged less than 15 years diagnosed with malignant liver tumours during the 30 years 1957-1986. The cases were identified from information collected by the West Midlands Regional Children's Tumour Registry. Pertinent data were extracted from their clinical records, and the original biopsy and any necropsy material were reviewed by a panel of three paediatric pathologists. Of the 50 eligible cases registered, eight were excluded because histology review showed that they had non-malignant conditions (3) or malignancies of extrahepatic origin (4) or because no pathological material was available (1). The diagnoses in the remaining 42 cases were hepatoblastoma (27), hepatocellular carcinoma (3), rhabdomyosarcoma (6), rhabdoid tumour (3) and yolk sac tumour (3). The incidence of primary malignant liver tumours was 1.20 per 10(6) person years and that of the hepatoblastoma sub-group was 0.77 (average childhood population of the West Midlands for the time period being 1,166,500). The presenting clinical, radiological and biochemical features were similar to those reported in other series and the ethnic and social class distributions were unremarkable compared with the local population. Congenital defects or other possibly related features were present in nine (21%) patients. Our results, taken with other reports, suggest that hepatoblastoma is a malignant tumour related to maldevelopment, possibly associated with 11p or 5q mutations, whereas hepatocellular carcinoma is more usually a complication of metabolic and other disorders which lead to cirrhosis.