Department of Pathology, Sri Ramachandra Medical College, Chennai, Tamil Nadu, India.
MBBS Student, Sri Ramachandra Medical College, Chennai, Tamil Nadu, India.
J Cancer Res Ther. 2022 Apr-Jun;18(3):677-680. doi: 10.4103/jcrt.jcrt_1575_21.
Primary hepatic malignancies account for 0.5-2% of all solid tumours in childhood. Hepatoblastoma, a rare embryonic tumour in the general population, represents the most frequent primary hepatic malignancy in the paediatric age group, with an incidence of one new case per million under 15 years of age, median age at diagnosis being 1 year. Aberrant activation of the Wnt/beta-catenin signalling pathway is likely to result in tumorigenesis of hepatoblastoma. The nuclear and intra-cytoplasmic accumulations of beta-catenin correlate with the likely prognosis of the disease. Nuclear expression of beta catenin is associated with a shorter survival, higher stage, and seen in embryonal/undifferentiated types.
To study the expression of beta-catenin in hepatoblastoma by immunohistochemistry and correlate it with the tumour histology and survival outcome.
MATERIALS & METHODS: This is a retrospective study of 11 children over a period of 5 years with the diagnosis of hepatoblastoma. These children underwent partial hepatectomy or liver transplantation at the Department of Paediatric Surgery. The clinical, histological and survival data were collected. Immunohistochemical analysis with beta-catenin was done and analysed.
Mean birth weight of the children was 2.75kg.63.6% had an epithelial type of histology.Beta catenin expression by IHC was studied in 11 cases and found to be positive in 4 cases. Nuclear positivity was noted in 2/4 cases of embryonal type and Cytoplasmic and membranous positivity was seen in the other 2/4 cases. Normal liver showed a membranous pattern of positivity in one case. Negative staining was seen in 6 out of 11 cases.
Beta catenin is considered to be an useful tool for assessing the prognosis of patients with hepatoblastoma and its expression is associated with a poor survival outcome. There are no validated biomarkers for prognosis so far. However, larger studies incorporating molecular profiling is warranted to establish prognostic factors for planning effective treatment strategies.
原发性肝恶性肿瘤约占儿童所有实体肿瘤的 0.5-2%。肝母细胞瘤是一种罕见的胚胎肿瘤，在儿童中最常见，发病率为每 100 万 15 岁以下儿童中就有 1 例，中位诊断年龄为 1 岁。Wnt/β-连环蛋白信号通路的异常激活可能导致肝母细胞瘤的发生。β-连环蛋白的核内和胞质内积聚与疾病的预后相关。β-catenin 的核表达与较短的生存时间、较高的分期有关，并且在胚胎/未分化型中可见。
通过免疫组织化学研究肝母细胞瘤中β-连环蛋白的表达，并将其与肿瘤组织学和生存结果相关联。
这是一项为期 5 年的 11 例肝母细胞瘤患儿的回顾性研究。这些患儿在小儿外科行部分肝切除术或肝移植。收集临床、组织学和生存数据。进行β-连环蛋白的免疫组织化学分析并进行分析。
患儿的平均出生体重为 2.75kg，63.6%的患儿具有上皮型组织学。11 例患儿进行了β-catenin 的免疫组化研究，其中 4 例为阳性。2/4 例胚胎型呈核阳性，另外 2/4 例为胞质和膜阳性。1 例正常肝显示膜阳性模式。11 例中有 6 例为阴性。
β-catenin 被认为是评估肝母细胞瘤患者预后的有用工具，其表达与不良生存结果相关。目前尚无经过验证的预后标志物。然而，需要进行更大规模的研究，纳入分子谱分析，以确定用于制定有效治疗策略的预后因素。
