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儿童非肝脏起源癌症所致肝外胆管梗阻:5例报告

Extrahepatic biliary obstruction caused by cancer of non-liver origin in children: report of 5 cases.

作者信息

Chen B W, Chang M H, Lin D T, Lin K H, Chuu W M, Lin K S

出版信息

Taiwan Yi Xue Hui Za Zhi. 1989 Aug;88(8):819-23.

PMID:2592945
Abstract

Obstructive jaundice secondary to external compression of the extrahepatic bile duct caused by tumor of non-liver origin was found in 5 of 199 consecutive children with cancer between 1986 and 1988 at the Department of Pediatrics, National Taiwan University Hospital. Of the 5 patients, 2 had non-Hodgkin's lymphoma and the other 3 had acute promyelocytic leukemia, histiocytosis X and neuroblastoma, respectively. Extrahepatic biliary obstruction occurred as part of the initial presentation of malignancy in 3 cases, and later in the course of disease in the other 2 cases. In each instance, abdominal ultrasonography and computed tomography revealed dilatation of intrahepatic biliary trees due to mass compressing effects. A huge multilobulated tumor and multiple enlarged lymph nodes near the porta hepatis were found in all 3 patients who underwent an exploratory laparotomy. Wedge biopsy of the liver showed no cancer cell invasion. One case died before chemotherapy had commenced. The other 4 patients received chemotherapy and 3 of them received additional radiotherapy. Although jaundice and tumor regressed dramatically with this mode of treatments, subsequent recurrence of tumor without jaundice rapidly ensued in 3 patients. They all died, except 1 case, within 18 months from the occurrence of jaundice. This suggests that these patients were in an advanced stage of disease and should be diagnosed early and treated vigorously. Accordingly, cancer of non-liver origin, although rare, should be considered in the differential diagnosis of obstructive jaundice if survival is to be improved in these cancer children.

摘要

1986年至1988年间,在台湾大学医院儿科的199例连续癌症患儿中,发现5例因非肝脏来源肿瘤导致肝外胆管受压而继发梗阻性黄疸。这5例患者中,2例患有非霍奇金淋巴瘤,另外3例分别患有急性早幼粒细胞白血病、组织细胞增多症X和神经母细胞瘤。肝外胆管梗阻在3例中作为恶性肿瘤的初始表现出现,在另外2例中在疾病过程中出现。在每例中,腹部超声和计算机断层扫描显示由于肿块压迫效应肝内胆管树扩张。在所有3例行剖腹探查术的患者中均发现巨大的多叶肿瘤和肝门附近多个肿大的淋巴结。肝脏楔形活检未显示癌细胞浸润。1例在化疗开始前死亡。其他4例患者接受了化疗,其中3例还接受了放疗。尽管采用这种治疗方式黄疸和肿瘤显著消退,但3例患者随后迅速出现无黄疸的肿瘤复发。除1例患者外,他们均在黄疸出现后18个月内死亡。这表明这些患者处于疾病晚期,应早期诊断并积极治疗。因此,如果要提高这些癌症患儿的生存率,在梗阻性黄疸的鉴别诊断中应考虑非肝脏来源的癌症,尽管这种情况罕见。

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