Celtik Ulgen, Dokumcu Zafer, Divarci Emre, Guler Ezgi, Ataseven Eda, Nart Deniz, Yilmaz Funda, Celik Ahmet, Ergün Orkan
Faculty of Medicine, Department of Pediatric Surgery, Ege University, 35100, Izmir, Turkey.
Faculty of Medicine, Department of Radiology, Ege University, 35100, Izmir, Turkey.
Pediatr Surg Int. 2020 Jul;36(7):817-825. doi: 10.1007/s00383-020-04672-6. Epub 2020 May 20.
Wnt/Beta-catenin pathway plays an essential role in liver development and regeneration. Abnormal activation in this pathway leads to development of hepatoblastoma (HB). Although its importance has invoked attention, its prognostic role is debatable. We aimed to evaluate the significance of intracellular localization of beta-catenin (BC) expression in the outcome of hepatoblastoma patients.
Medical records of HB patients between 2004 and 2018 were reviewed. Patients were grouped according to intracellular localization of BC expression by immunohistochemistry as being cytoplasmic or nuclear. Demographics, radiological images, PRETEXT classifications, vascular involvement, risk groups, chemotherapy responses, and survival rates were analyzed and compared between groups.
There were 41 patients. Thirteen patients were excluded for unavailability of records in four, negative/unclear BC expressions in seven. Cytoplasmic expression of BC was observed in 17 patients whereas 13 patients displayed nuclear expression. Demographics were similar in both groups. Cytoplasmic BC expression was associated with poor chemotherapy response (p = 0.001) and increased vascular involvement (p = 0.0162) requiring more extensive surgeries (p = 0.039).
Although the numbers are limited in our series, the intracellular localization of BC expression has been found to be a promising determining factor for hepatoblastoma prognosis. With larger patient series, more reliable results can be achieved.
Wnt/β-连环蛋白信号通路在肝脏发育和再生中起重要作用。该信号通路的异常激活会导致肝母细胞瘤（HB）的发生。尽管其重要性已引起关注，但其预后作用仍存在争议。我们旨在评估β-连环蛋白（BC）表达的细胞内定位在肝母细胞瘤患者预后中的意义。
回顾了2004年至2018年间HB患者的病历。通过免疫组织化学根据BC表达的细胞内定位将患者分为细胞质或细胞核两组。分析并比较两组患者的人口统计学资料、放射影像、PRETEXT分类、血管受累情况、风险组、化疗反应和生存率。
共有41例患者。4例因记录不全、7例因BC表达阴性/不清楚而被排除。17例患者观察到BC的细胞质表达，13例患者表现为细胞核表达。两组患者的人口统计学资料相似。细胞质BC表达与化疗反应差（p = 0.001）、血管受累增加（p = 0.0162）相关，这需要更广泛的手术（p = 0.039）。
尽管我们系列中的病例数有限，但已发现BC表达的细胞内定位是肝母细胞瘤预后的一个有前景的决定因素。通过更大的患者系列，可以获得更可靠的结果。
