儿童非肝源性肿瘤致肝外胆道梗阻5例报告。

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

台湾大学附属医院小儿科1986 ~ 1988年间连续199例癌症患儿中,有5例发生非肝源性肿瘤所致的肝外胆管外压迫所致的梗阻性黄疸。5例患者中,2例为非霍奇金淋巴瘤,3例为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。肝外胆道梗阻是3例恶性肿瘤最初表现的一部分,另外2例在病程中出现。在每个病例中,腹部超声和计算机断层扫描显示由于肿块压迫作用而导致肝内胆道树扩张。3例患者均行剖腹探查术,发现肝门附近有一个巨大的多分叶肿瘤和多个肿大的淋巴结。肝楔形活检未见癌细胞侵袭。1例在化疗开始前死亡。其余4例患者接受化疗,其中3例患者接受放疗。虽然黄疸和肿瘤在这种治疗模式下显著消退,但3例患者随后迅速复发无黄疸的肿瘤。除1例外,均在发生黄疸后18个月内死亡。这表明这些患者处于疾病晚期,应及早诊断并大力治疗。因此,如果要提高梗阻性黄疸患儿的生存率,尽管非肝源性癌症很少见,但在梗阻性黄疸的鉴别诊断中应予以考虑。
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Extrahepatic biliary obstruction caused by cancer of non-liver origin in children: report of 5 cases.

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.

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