Gastroenterology and Hepatology, Huntsman Cancer Center, University of Utah School of Medicine, 30 N 1900E 4R118, Salt Lake City, UT, 84312, USA.
Dig Dis Sci. 2014 Sep;59(9):2320-4. doi: 10.1007/s10620-014-3152-0. Epub 2014 Apr 19.
Cholangiocarcinoma (CCA) is very often an adulthood disease with primary sclerosing cholangitis (PSC) as one of the risk factors. It is rarely seen in the pediatric population, and when it is diagnosed before adulthood, it can be associated with PSC as well as HIV infection, biliary atresia, radiation therapy, and choledochal cyst. Although there have been some case reports of pediatric CCA, cases of childhood CCA associated with PSC are still relatively rare.
To describe the clinical and pathologic features of CCA in pediatric patients with previously diagnosed PSC.
Retrospective study
Four patients with PSC (age range 15-18, mean 17 years) were included in this study. All patients underwent ERCP for diagnosis. Tissue samples obtained included routine cytology and FISH. ERCP was used to target sites for tissue acquisition in all patients. 3/4 of patients have inflammatory bowel disease (two Crohn's disease and one ulcerative colitis). Alkaline phosphatase was elevated in 3/4 patients, aspartate aminotransferase/alanine aminotransferase were elevated in 2/4 patients, and total bilirubin/direct bilirubin were elevated in 2/4 patients. 4/4 patients had positive FISH studies, and 3/4 patients had brush cytology concerning for CCA. 2/4 patients received chemotherapy, one patient underwent orthotopic liver transplant, and one patient underwent Whipple procedure. Two patients died soon after being diagnosed.
Young patients with PSC can develop CCA. This finding has implications for both screening and surveillance for cancer in pediatric patients with PSC.
胆管癌（CCA）通常是成人疾病，原发性硬化性胆管炎（PSC）是其危险因素之一。在儿科人群中很少见，当它在成年前被诊断出来时，它可能与PSC以及 HIV 感染、胆道闭锁、放射治疗和胆总管囊肿有关。尽管有一些儿科 CCA 的病例报告，但与 PSC 相关的儿童 CCA 病例仍然相对较少。
描述先前诊断为 PSC 的儿科患者中 CCA 的临床和病理特征。
回顾性研究
本研究纳入了 4 例 PSC 患者（年龄 15-18 岁，平均 17 岁）。所有患者均接受 ERCP 以明确诊断。获得的组织样本包括常规细胞学和 FISH。所有患者均通过 ERCP 定位组织采集部位。4/4 例患者患有炎症性肠病（2 例克罗恩病和 1 例溃疡性结肠炎）。3/4 例患者碱性磷酸酶升高，2/4 例患者天门冬氨酸氨基转移酶/丙氨酸氨基转移酶升高，2/4 例患者总胆红素/直接胆红素升高。4/4 例患者的 FISH 检查阳性，3/4 例患者的刷检细胞学检查提示 CCA。2/4 例患者接受了化疗，1 例患者接受了原位肝移植，1 例患者接受了胰十二指肠切除术。2 例患者在确诊后不久死亡。
患有 PSC 的年轻患者可能会发展为 CCA。这一发现提示对患有 PSC 的儿科患者进行癌症筛查和监测。
