Zhong Shanshan, Zhao Yang, Fan Chuifeng
Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences of China Medical University Department of Hepatobiliary and Spleenary Surgery, The Affiliated Shengjing Hospital, China Medical University, Shenyang, China.
Medicine (Baltimore). 2018 Jan;97(2):e9647. doi: 10.1097/MD.0000000000009647.
Hepatoblastoma is a rare malignant embryonal tumor that only accounts for approximately 1% of all pediatric cancers and mostly develops in children younger than 5 years old. Moreover, the occurrence of hepatoblastoma in adults is extremely rare.
Herein, we present a rare case of hepatoblastoma with pure epithelial differentiation in a 10-year-old boy.Pathological examination was performed. The tumor was 15 cm × 15 cm in size with clear margins. The cut surface was multiple nodular and grey-yellow. Histologically, the small cuboidal tumor cells were arranged in trabeculae with 2-3 cell layers. The tumor cells had eosinophilic or clear cytoplasm, formed dark and light areas, and were positive for alpha-fetoprotein, CK, CK8/18, CD10, hepatocyte, and GPC3. CD34 staining revealed that the sinusoids were lined by endothelial cells in the tumor tissues. The Ki67 index was approximately 20%.
Based on these findings, the case was diagnosed as hepatoblastoma with pure fetal epithelial differentiation.
The tumor was completely removed.
No recurrence was found 3 months after the operation.
Hepatoblastoma with pure epithelial differentiation can also occur in older children. Children rarely notice and report any physical abnormality, and this may be among the primary reasons for the late diagnosis of the tumor. Annual heath checks may be beneficial in the detection of these rare tumors and improvement of patient outcomes.
肝母细胞瘤是一种罕见的恶性胚胎性肿瘤,仅占所有儿童癌症的约1%,主要发生在5岁以下儿童。此外,成人发生肝母细胞瘤极为罕见。
在此,我们报告一例10岁男孩罕见的具有纯上皮分化的肝母细胞瘤病例。进行了病理检查。肿瘤大小为15厘米×15厘米,边界清晰。切面呈多个结节状,灰黄色。组织学上,小立方形肿瘤细胞呈小梁状排列,有2 - 3层细胞。肿瘤细胞有嗜酸性或透明的细胞质,形成明暗区域,甲胎蛋白、细胞角蛋白、细胞角蛋白8/18、CD10、肝细胞和磷脂酰肌醇蛋白聚糖3呈阳性。CD34染色显示肿瘤组织中的血窦由内皮细胞衬里。Ki67指数约为20%。
基于这些发现,该病例被诊断为具有纯胎儿上皮分化的肝母细胞瘤。
肿瘤被完全切除。
术后3个月未发现复发。
具有纯上皮分化的肝母细胞瘤也可发生于年龄较大的儿童。儿童很少注意并报告身体的任何异常,这可能是肿瘤诊断延迟的主要原因之一。年度健康检查可能有助于发现这些罕见肿瘤并改善患者预后。