Kiruthiga Kala Gnanasekaran, Ramakrishna Banumathi, Saha Soumitra, Sen Sudipta
Departments of Pathology, Christian Medical College, Vellore, India.
Paediatric Surgery, Christian Medical College, Vellore, India.
J Gastrointest Oncol. 2018 Apr;9(2):326-337. doi: 10.21037/jgo.2018.01.08.
Hepatoblastoma (HB) has different histological subtypes, with varying prognosis. Though the survival has drastically improved, subsets of patients are not responsive to therapy. Therefore, it becomes important to determine the factors which affect the behaviour of the tumour. This study was aimed to look at the histopathological subtypes and compare with immunohistochemical (IHC) expression of CK19, beta-catenin and EpCAM and survival.
This study included 55 cases of HB. IHC expression of CK19, beta-catenin and EpCAM were correlated with histological subtypes, tumour behaviour, response to chemotherapy and survival.
Most common epithelial subtype was fetal (43.2%) and mixed epithelial (54.8%) in pre- and post-chemotherapy groups respectively. Microvascular invasion (MVI) was present in 14/33 resected tumours. CK19 expression was seen in 54.2% and 72.2% of embryonal subtype, nuclear beta-catenin expression in 48.7% and 57.1% and EpCAM in 100% and 82.1% of tumours in pre- and post-chemotherapy groups, respectively. Fetal subtype had a lesser chance of MVI, recurrence, metastasis and death. Beta-catenin expression was associated with lower event free survival (EFS) and EpCAM with ≥50% viable tumour following chemotherapy (P=0.04). Age at diagnosis ≤2 years, male sex, alpha-fetoprotein <10,000 IU/mL following chemotherapy, solitary tumour (P=0.001), size ≤5 cm, pretreatment extent of disease (PRETEXT) I&II, mitosis ≤2/10 high power fields (hpf), viable tumour <50% (P=0.04) and absent nuclear expression of beta-catenin, predicted a higher EFS rate.
Beta-catenin expression is associated with lower EFS and EpCAM expression with tumour viability. Multifocality and viable tumour ≥50% were significant factors predicting lower EFS. These factors should be included in the prognostication of HBs.
肝母细胞瘤(HB)有不同的组织学亚型,预后各异。尽管生存率已大幅提高,但部分患者对治疗无反应。因此,确定影响肿瘤行为的因素变得很重要。本研究旨在观察组织病理学亚型,并与细胞角蛋白19(CK19)、β-连环蛋白和上皮细胞黏附分子(EpCAM)的免疫组化(IHC)表达及生存率进行比较。
本研究纳入55例HB病例。CK19、β-连环蛋白和EpCAM的IHC表达与组织学亚型、肿瘤行为、化疗反应及生存率相关。
化疗前和化疗后组中最常见的上皮亚型分别为胎儿型(43.2%)和混合上皮型(54.8%)。14/33例切除的肿瘤存在微血管侵犯(MVI)。化疗前和化疗后组中,胚胎型亚型的肿瘤分别有54.2%和72.2%表达CK19,48.7%和57.1%有β-连环蛋白核表达,100%和82.1%表达EpCAM。胎儿型亚型发生MVI、复发、转移及死亡的几率较低。β-连环蛋白表达与较低的无事件生存率(EFS)相关,而EpCAM表达与化疗后存活肿瘤≥50%相关(P=0.04)。诊断时年龄≤2岁、男性、化疗后甲胎蛋白<10,000 IU/mL、孤立肿瘤(P=0.001)、大小≤5 cm、治疗前疾病范围(PRETEXT)I&II、有丝分裂≤2/10高倍视野(hpf)、存活肿瘤<50%(P=0.04)以及无β-连环蛋白核表达,提示EFS率较高。
β-连环蛋白表达与较低的EFS相关,EpCAM表达与肿瘤存活相关。多灶性和存活肿瘤≥50%是预测较低EFS的重要因素。这些因素应纳入HB的预后评估。