肝母细胞瘤化疗前后细胞凋亡检测及其临床意义

目的：检测化疗前后肝母细胞瘤（ＨＢ）细胞凋亡,评价其在判断ＨＢ预后及其化疗疗效中的价值并探讨化疗机制。方法：应用ＴＵＮＥＬ技术检测１３例ＨＢ化疗前后标本细胞凋亡。结果：化疗前后标本的可见ＨＢ细胞凋亡;化疗后凋亡指数（ＰＣＡＩ）较化疗前自发性凋亡指数（ＳＡＩ）显著增高（Ｐ〈０．０５）,ＰＣＡＩ与ＳＡＩ呈正相关（ｒ＝０．９０７）,化疗后肿瘤体积（直径）减少值与ＳＡＩ呈正相关（ｒ＝０．６９４）,ＳＡＩ高     

A case of hepatoblastoma occurring in an adult

We present herein a rare case of hepatoblastoma occurring in an adult male. The patient was 22 years old and his laboratory investigations on admission showed a marked elevation of α-fetoprotein in the serum. CT scan and other examinations revealed a primary tumor, 6.5 cm in size, in the left hepatic lobe with metastasis in the head of the pancreas. Thus, left hepatic lobectomy and pancreaticoduodenectomy were performed, but metastasis to the right hepatic lobe, left lung and abdominal skin were found 2 months later. Despite repeated courses of chemotherapy with adriamycin and cisplatin, the patient died 9 months after his operation. Pathological findings revealed poorly differentiated type hepatoblastoma. A review of the literature revealed only twelve other such cases.     

应用生物信息学挖掘肝母细胞瘤发病差异基因

目的筛选肝母细胞瘤(hepatoblastoma)组织与小儿正常肝脏组织中的差异表达基因,探究肝母细胞瘤的发病机制,为其诊断和治疗提供新方向。方法从GEO数据库中检索获取肝母细胞瘤组织和小儿正常肝脏组织的芯片数据,通过R语言软件RSTUDIO筛选芯片中的差异表达基因,使用DAVID数据库对筛选所得的差异表达基因进行功能注释,通过STRING数据库构建蛋白质相互作用网络,并进行中心性分析。结果经筛选共获得肝母细胞瘤组织中290个差异表达基因,其中上调基因99个,下调基因191个(P0.05)。GO(Gene Ontology)功能注释分析显示,上调差异基因主要涉及细胞分裂、细胞外外泌体、金属离子结合等94个功能簇,下调差异基因主要涉及脂蛋白代谢、细胞外外泌体、血红素结合等100个功能簇。蛋白质相互作用网络分析示IMPDH2、AGXT、ALDH1A1、ALDH2、PFAS、SERPINC1、AGXT2、KNG1、APOA1、MAT1A、APOC3和HSD17B6 12个基因为与其他节点关系最密切的核心调控基因。结论通过多种生物信息学方法联合分析三组高通量基因芯片,获得了肝母细胞瘤组织与正常小儿肝脏组织间的差异表达基因,并进一步从不同角度分析肝母细胞瘤异常增殖、转移等恶性生物学过程的发生机制,为肝母细胞瘤的诊断和治疗提供新方向。     

Focal liver diseases in neonatal and pediatric liver transplant candidates: a pictorial essay

Caruso S, Mamone G, Marrone G, Milazzo M, Carollo V, Miraglia R, Maruzzelli L, Pasta A, Minervini MI, Spada M, Riva S, Luca A, Gridelli B. Focal liver diseases in neonatal and pediatric liver transplant candidates: a pictorial essay.
Clin Transplant 2009: DOI: 10.1111/j.1399‐0012.2009.01139.x
© 2009 John Wiley & Sons A/S. Abstract: The aim of this review is to present the wide spectrum of common and uncommon focal liver diseases affecting neonatal and pediatric liver transplant candidates, analyzed using ultrasonography (US), 16‐ or 64‐multidetector row helical CT (MDCT) and 1.5‐T magnetic resonance (MR) fast imaging. Correlation of imaging findings and explanted liver or histology is illustrated in representative cases. Associated uncommon congenital anomalies are shown.     

Bcl-2 gene silencing in pediatric epithelial liver tumors

BACKGROUND: Proteins of the Bcl-2 family prevent cells of various tumor types from undergoing apoptosis and thus contribute to their chemotherapy resistance. The phenotype of multidrug resistance is a major factor for poor treatment results of advanced epithelial liver tumors in children. The role of Bcl-2 proteins in these tumors is yet unclear. The purpose of this study was to analyze the influence of Bcl-2 on the chemotherapy resistance of hepatoblastoma (HB) and pediatric hepatocellular carcinoma (HCC). MATERIALS AND METHODS: Bcl-2 expression was analyzed in the HB cell lines HUH6 and HepT1 as well as in the HCC cell line HepG2 before and after treatment with cisplatin, doxorubicin, taxol, and etoposid. Silencing of the Bcl-2 gene was performed via RNA interference using specific siRNA. Treatment efficiencies of cytotoxic agents were assessed against original and Bcl-2 siRNA transfected tumor cells. RESULTS: The mixed HB cell line HUH6 showed a relevant amount of Bcl-2 expression, which increased after chemotherapy. In these cells Bcl-2 appeared within the nuclei and the cytosol. Treatment with all cytotoxic agents was significantly improved through Bcl-2 siRNA (P < 0.001-0.0054) in this cell line. There was no effect of Bcl-2 siRNA in HepT1 and HepG2 cells. CONCLUSIONS: Bcl-2 seems to play a role in antiapoptotic mechanisms of some HB subtypes. Thus, this gene might serve as target for a gene-directed adjuvant therapy. Further studies seem necessary to clear the susceptibility of pediatric epithelial liver tumors toward the described approach.     

Neuroendocrine differentiation in embryonal type hepatoblastoma

Hepatoblastoma, a malignant tumor which arises occasionally in older children but very rarely in adults, exhibits divergent differentiation with embryonal cells, fetal hepatocytes and immature elements. This report describes an embryonal type hepatoblastoma with neuroendocrine differentiation in a 16‐year‐old patient, which was diagnosed postoperatively. Clinical and radiologic work‐up failed to reveal a primary gastrointestinal malignancy and no primary lesions were detected in any other organ. This feature of hepatoblastoma is considered to be a multidirectional differentiation of the small epithelial or stem cells of the liver. At 2‐year follow up, the patient remains symptom‐free, with normal laboratory and diagnostic imaging studies, and no recurrent or metastatic disease identified.     

Immunoreactive prothrombin-like material in hepatoblastoma and other tumor monolayers

Using an indirect immunofluorescence assay, we looked for the presence of immunoreactive prothrombin-like material in human tumor cell and fibroblast cultures. Prothrombin-like material was found in one hepatoblastoma, one choroidal melanoma, and 3/7 neuroectodermal cell lines. However, it was not found in peripheral melanoma or fibroblast cells.     

Sorafenib and bevacizumab for recurrent metastatic hepatoblastoma: Stable radiographic disease with decreased AFP

We report the use of sorafenib and bevacizumab in combination for a patient with recurrent metastatic hepatoblastoma (HB). This combination demonstrated activity against our patient's refractory HB that had been extensively treated with multiple prior chemotherapeutic regimens. The patient had stabilization of radiographic disease coupled with an 83% decrease in his alpha‐fetoprotein level. Given the response in this setting and the paucity of other available options, consideration could be given to using this combination as therapy in patients with recurrent HB who have failed more traditional agents. Pediatr Blood Cancer 2012; 59: 939–940. © 2012 Wiley Periodicals, Inc.