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Pediatric Hepatocellular Carcinoma, a Single Center Study from the South of Iran: Case Series

机译:小儿肝细胞癌,来自伊朗南部的单中心研究:病例系列

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Background: Hepatocellular carcinoma is a rare tumor in children, thus, there are not many studies regarding clinicopathologic findings of this tumor and no report regarding this tumor in this age group from Iran. Objectives: In this study, we tried to find out the clinicopathologic findings including outcome of hepatocellular carcinoma in the patients under the age of 18. Methods: During the last 10 years (2005 - 2016), we collected all hepatocellular carcinoma cases in the affiliated hospitals of Shiraz University of Medical Sciences (as the largest referral center in the South of Iran and the only center of pediatric liver transplantation in the whole country) from the archives of Pathology department. Next all clinical data were subsequently extracted from the patients’ hospital charts. Results: We collected 30 cases of hepatocellular carcinoma with the age range of 9 months to 18 years with no significant sex preference. All of the patients, except 3, showed abnormal levels of AFP. The most common underlying cause of liver tumorigenesis was type I tyrosinemia. Conclusions: Hepatocellular carcinoma in children is a rare tumor and is mostly secondary to an underlying liver disease, which is tyrosinemia in our center. Hepatitis B related hepatocellular carcinoma is rare in our country, which seems to be related to successful early neonatal vaccination against this viral disease. This tumor has a poor outcome in children and without liver transplantation, has a very short survival rate.
机译:背景:肝细胞癌是儿童中罕见的肿瘤,因此,关于该肿瘤的临床病理学发现的研究并不多,来自伊朗的这一年龄组也没有关于该肿瘤的报道。目的:在这项研究中,我们试图找出18岁以下患者的临床病理结果,包括肝细胞癌的结局。方法:在过去10年(2005年至2016年),我们收集了所有在附属机构中的肝细胞癌病例设设于病理学系档案中的设拉子医学大学的医院(伊朗南部最大的转诊中心,也是全国唯一的小儿肝移植中心)。接下来,所有临床数据随后均从患者的医院病历中提取。结果:我们收集了30例年龄在9个月至18岁之间且无明显性别偏爱的肝细胞癌病例。除3例外,所有患者均显示AFP水平异常。肝肿瘤发生的最常见根本原因是I型酪氨酸血症。结论:儿童肝细胞癌是一种罕见的肿瘤,主要继发于潜在的肝脏疾病,即我们中心的酪氨酸血症。乙型肝炎相关的肝细胞癌在我国很少见,这似乎与针对这种病毒性疾病的新生儿早期成功疫苗接种有关。该肿瘤在儿童中预后较差,且未进行肝移植,存活率非常短。

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