Computer-assisted three-dimensional individualized extreme liver resection for hepatoblastoma in proximity to the major liver vasculature

BACKGROUND The management of hepatoblastoma(HB)becomes challenging when the tumor remains in close proximity to the major liver vasculature(PMV)even after a full course of neoadjuvant chemotherapy(NAC).In such cases,extreme liver resection can be considered a potential option.AIM To explore whether computer-assisted three-dimensional individualized extreme liver resection is safe and feasible for children with HB who still have PMV after a full course of NAC.METHODS We retrospectively collected data from children with HB who underwent surgical resection at our center from June 2013 to June 2023.We then analyzed the detailed clinical and three-dimensional characteristics of children with HB who still had PMV after a full course of NAC.RESULTS Sixty-seven children diagnosed with HB underwent surgical resection.The age at diagnosis was 21.4±18.8 months,and 40 boys and 27 girls were included.Fifty-nine(88.1%)patients had a single tumor,39(58.2%)of which was located in the right lobe of the liver.A total of 47 patients(70.1%)had PRE-TEXT III or IV.Thirty-nine patients(58.2%)underwent delayed resection.After a full course of NAC,16 patients still had close PMV(within 1 cm in two patients,touching in 11 patients,compressing in four patients,and showing tumor thrombus in three patients).There were 6 patients of tumors in the middle lobe of the liver,and four of those patients exhibited liver anatomy variations.These 16 children underwent extreme liver resection after comprehensive preoperative evaluation.Intraoperative procedures were performed according to the preoperative plan,and the operations were successfully performed.Currently,the 3-year event-free survival of 67 children with HB is 88%.Among the 16 children who underwent extreme liver resection,three experienced recurrence,and one died due to multiple metastases.CONCLUSION Extreme liver resection for HB that is still in close PMV after a full course of NAC is both safe and feasible.This approach not only reduces the necessity for liver transplantation but also results in a favorable prognosis.Individualized three-dimensional surgical planning is beneficial for accurate and complete resection of HB,particularly for assessing vascular involvement,remnant liver volume and anatomical variations.

Pediatric Hepatoblastoma: A Case Report and Review of the Literature

Introduction and Objective: Hepatoblastoma is the most frequent malignant embryonal liver tumor, and its incidence is increasing. Children under 3 years of age are particularly affected. The etiology is largely unknown, but a genetic predisposition exists. This case highlights the clinical and imaging characteristics and management strategies. Case Report: We report the case of an 18 months old male admitted for exploration of an abdominal mass and was diagnosed with hepatoblastoma. Conclusion: Hepatoblastoma is an aggressive tumor that begins locally and eventually metastasizes to the brain, lungs, lymph nodes, and other organs. Tumor stage at presentation and resectability are the most significant prognostic factors.

Application of endoscopic retrograde cholangiopancreatography for treatment of obstructive jaundice after hepatoblastoma surgery:A case report

BACKGROUND We aimed to investigate the effectiveness of endoscopic retrograde cholangiopan-creatography(ERCP)for treating obstructive jaundice(OJ)post hepatoblastoma(HB)surgery(post-HB OJ)by analyzing the data of a case and performing a literature review.CASE SUMMARY Clinical data of one patient with post-HB OJ treated by ERCP were retrospectively analyzed.Furthermore,clinical characteristics and insights into the diagnosis and treatment of post-HB OJ in children were summarized via searching various databases and platforms,such as China National Knowledge Infrastructure,Wanfang database,CQVIP database,PubMed,Ringer Link,and Google Scholar.The patient reported herein underwent five chemotherapy sessions after the diagnosis of HB and right hemihepatectomy after tumor size reduction;these were followed by two postoperative chemotherapy sessions.Three months postoperatively,the patient developed icteric sclera,strong tea-colored urine,and clay-like stools,and showed signs of skin itchiness;blood analysis showed significantly an increased conjugated bilirubin(CB)level(200.3μmol/L).Following the poor efficacy of anti-jaundice and hepatoprotective treatments,the patient underwent ERCP.Intraoperative imaging showed a dilated bile duct in the porta hepatis with significant distal stenosis.A 5 Fr nasopancreatic tube was placed in the dilated area through the stricture for external drainage,and the patient was extubated on postoperative day 6.Postoperatively,the patient’s stool turned yellow,and the CB level decreased to 78.2μmol/L.Fifteen days later,ERCP was repeated due to unrelieved jaundice symptoms,wherein a 7 Fr naso-biliary drainage tube was successfully placed.Three months post-ERCP,the jaundice symptoms resolved,and the CB level was reduced to 33.2μmol/L.A follow-up examination one year postoperatively revealed no jaundice symptoms and normal CB level.CONCLUSION Post-HB OJ is rare.Compared to biliary tract reconstruction,ERCP is less invasive and has a better therapeutic effect.

Role of liver transplantation in the management of hepatoblastoma in the pediatric population

Hepatoblastoma(HB) is the most common primary liver tumor in children and accounts for two-thirds of all malignant liver neoplasms in the pediatric population. For patients with advanced HB(unresectable or unresponsive to chemotherapy), combined treatment with chemotherapy and liver transplantation is an excellent option. The etiology of HB is mostly obscure because of its extreme rarity although some inherited syndromes and very low birth weight have been associated with it. The prognosis for children with HB has significantly improved in the past three decades thanks to advancements in chemotherapy, surgical resection and postoperative care. In 2002 a surgical staging system called pretreatment extent of disease(PRETEXT) was designed to allow a universal, multidisciplinary approach to patients with HB. Between one-third to two-thirds of patients initially present with unresectable tumors or distant metastases, but up to 85% of these tumors become operable after neoadjuvant chemotherapy. Patients with PRETEXT categories 1, 2, and some 3 are referred for neoadjuvant chemotherapy followed by surgical resection with the goal of complete tumor removal. Classic treatments regimens include a combination of cisplatin, fluorouracil, and vincristine or cisplatin and doxorubicin. Liver transplantation is the only treatment option for unresectable HB. In 2010 the pediatric end-stage liver disease, a pediatric-specific scoring system that determines a patient's ranking on the liver transplant list, began to award additional "exception" points for patients with HB. We analyzed the Standard Transplant Analysis and Research dataset to assess the impact of changes in exception point criteria for HB on outcomes after liver transplantation at Texas Children's Hospital in Houston, Texas. We found that patients who were listed for transplantation with current HB exception criteria experienced a shorter waitlist time but survival was similar between the two eras.

Robot-assisted gallbladder-preserving hepatectomy for treating S5 hepatoblastoma in a child: A case report and review of the literature

BACKGROUND Hepatoblastoma(HB) is the most common hepatic malignant tumour in children,accounting for approximately 50%-60% of primary hepatic malignant tumours in children, mostly in children under 3 years old. In Western countries, the incidence of hepatoblastoma is approximately 1-2/100000. Da Vinci surgical system is fast becoming a key instrument in microinvasive surgery. The past decade has seen the rapid development of robot-assisted laparoscopy, which expends many fields including the liver surgery. This paper discusses the significance and feasibility of robot-assisted gallbladder-preserving hepatectomy for treating S5 hepatoblastoma in children. The aim of this essay is to compare the safety and effectiveness of robotic surgery with conventional laparoscopic surgery, and explore the meaning of preservation of the gallbladder by sharing this case.CASE SUMMARY A 3-year-old child with a liver mass in the 5 th segment was treated using the Da Vinci surgical system, and the gallbladder was retained. The child was admitted to the hospital for 20 d for the discovery of the right hepatic lobe mass.Ultrasonography revealed a low echo mass, 46 mm × 26 mm × 58 mm in size,indicating hepatoblastoma in the right lobe, and enhanced computed tomography showed continuous enhancement of iso-low-density lesions with different sizes and nodules and unclear boundaries, without the dilation of the intrahepatic bile duct, no enlargement of the gallbladder, and uniform thickness of the wall. The diagnosis was "liver mass, hepatoblastoma". It was decided to perform S5 liver tumour resection. During surgery, the tumour and gallbladder were isolated first, and the gallbladder could be completely separated from the tumour surface without obvious infiltration; therefore, the gallbladder was preserved. The cutting line was marked with an electric hook. The hepatic duodenal ligament was blocked with a urethral catheter using the Pringle method, and the tumour and part of the normal liver tissue were completely resected with an ultrasound knife along the incision. The hepatic portal interdiction time was approximately 25 min. An abdominal drainage tube was inserted. The auxiliary hole was connected to the lens, and the specimen was removed. The patient's status was uneventful, and the operation time was 166 min. The robotic time was 115 min, and the bleeding amount was approximately 200 mL. In total, 300 mL of red blood cell suspension and 200 mL of plasma were injected. No serious complications occurred. Pathological findings confirmed fetal hepatoblastoma and R0 resection. A gallbladder contraction test was performed two weeks after surgery.CONCLUSION Robot-assisted S5 hepatectomy with gallbladder preservation is safe and feasible for specific patients.

Focal nodular hyperplasia coexistent with hepatoblastoma in a 36-d-old infant

Focal nodular hyperplasia(FNH) is a benign hepatic tumor characterized by hepatocyte hyperplasia and a central stellate scar.The association of FNH with other hepatic lesions,such as adenomas,hemangiomas and hepatocellular carcinoma,has been previously reported,but FNH associated with another hepatic tumor is rare in infants.Here we report a case of FNH coexistentwith hepatoblastoma in a 36-d-old girl.Computed tomography(CT) imaging showed an ill-delineated,inhomogeneous enhanced mass with a central star-like scar in the right lobe of the liver.The tumor showed early mild enhancement at the arterial phase(from 40 HU without contrast to 52 HU at the arterial phase),intense enhancement at the portal phase(87.7HU) and 98.1HU in the 3-min delay scan.A central scar in the tumor presented as low density on non-contrast CT and slightly enhanced at delayed contrast-enhanced scanning.This infant underwent surgical resection of the tumor.Histopathology demonstrated typical FNH coexistent with a focal hepatoblastoma,which showed epithelioid tumor cells separated by proliferated fibrous tissue.

Periostin mediates epithelial-mesenchymal transition through the MAPK/ERK pathway in hepatoblastoma

Objective: The aim of the present study was to analyze the prognostic factors in patients with hepatoblastoma(HB) in our single center and to evaluate periostin(POSTN) expression in HB and its association with clinicopathological variables. In addition, the underlying mechanism of how POSTN promotes HB progression was discussed.Methods: POSTN expression was investigated in HB tumors by immunohistochemistry(IHC), immunofluorescence(IF) and Western blot(WB). The association among POSTN expression, clinicopathological features and overall survival(OS) was also evaluated. The migration and adhesion ability of HB cells were measured using chemotaxis and cell-matrix adhesion assays,respectively. Epithelial-mesenchymal transition(EMT)-associated markers and activation of the ERK pathway were detected by WB.Results: HB patients had poor prognosis which displayed lymph node metastasis, vascular invasion, POSTN and vimentin expression. POSTN expression was also associated with lymph node metastasis. Furthermore, overexpressed POSTN promoted migration and the adhesive ability of HB cells in vitro. In addition, we demonstrated that POSTN activated the MAPK/ERK pathway, upregulated the expression of Snail and decreased the expression of OVOL2. Finally, POSTN promoted the expression of EMT-associated markers.Conclusions: POSTN might modulate EMT via the ERK signaling pathway, thereby promoting cellular migration and invasion.Our study also suggests that POSTN may serve as a therapeutic biomarker in HB patients.

A Feasible Surgical Approach for Treating Extensive Hepatoblastoma Using a Backup for Living Donor Liver Transplantation: Case Report

Hepatoblastoma is the most common type of malignant liver tumor in children and occurs most frequently in patients two years of age and younger. The outcomes for children with hepatoblastoma have been improving in recent years due to the introduction of cisplatin-based chemotherapy regimens. Recently, performing liver transplantation after neoadjuvant chemotherapy has become increasingly advocated as a primary surgical treatment for children with unresectable hepatoblastoma involving three or four sectors of the liver. Surgical exploration is frequently required to determine which tumors are resectable, which require liver transplantation and timing of resection. When the quality of the remnant liver is in question, the decision for transplantation versus resection should be made by a liver team in experienced center with capability of liver transplantation. This report presents the findings of the patient with hepatoblastoma who successfully underwent extreme resection with a backup for living donor liver transplantation. The patient was discharged home in good condition and the follow-up continued for three years with normal AFP levels observed.

Hepatoblastoma in Developing Countries;Eight Years of Single Centre Experience

Background and objectives: Although hepatoblastoma (HB) is a rare childhood tumor and constitutes only 0.9% of all pediatric cancers, there was an obvious improvement in risk stratification and prognosis over the last two decades. This study aimed to assess the outcome of HB patients treated in our center and to investigate the impact of different prognostic factors on the survival of these patients. Patients and methods: This was a retrospective study included newly diagnosed patients with HB presented to the Children Cancer Hospital Egypt (CCHE 57357), from July 2007 to June 2015. Patient’s data were analyzed for the clinical characteristics and survival outcome of the included patients. Results: One hundred twenty-four children were included during the study period with a median age of 14 months. The tumor was found occupying the entire liver in 25 patients (20%);while it was confined to one lobe in 80% of them, portal vein thrombosis was diagnosed in 10 patients, and there were 30 patients (24%) had metastatic disease at presentation. Only five patients (4%) underwent primary surgical excision, and all of them were grossly excised (stage I);77/119 (64.7%) experienced delayed surgery after two to six courses (median, four courses) of C5VD and the overall resection rate was 66%. There were 42 patients (35.3%) failed to do surgical excision either because they still had evident metastatic disease with poor chemotherapy response, or because their tumor remained unresectable after six courses of chemotherapy. The 3-year event-free and overall survivals for the whole studied patients were 45.7% (95% CI, 36.9% - 56.7%), and 66.7% (95% CI, 57.1% - 77.8%) respectively. The 3-year EFS and OS were significantly better in those who underwent surgical excision (68.63% and 80.74% respectively, P-value 0.001). Also, the survival rates were significantly affected by the presence of metastatic disease at presentation, tumor stage and initial risk grouping of the studied patients. Conclusion: Surgical excision, tumor stage and COG risk grouping are the main prognostic variables affecting patients’ outcome. Efforts to achieve resectability of the tumor either by advanced surgical techniques or by developing effective preoperative treatment, especially for advanced and metastatic disease, are mandatory.

Ruptured hepatoblastoma with massive internal bleeding in an adult

Hepatoblastoma is the most common primary hepatic tumor of children. However, only a very few cases have been reported in adults. Most studies support treatment with chemotherapy followed by surgical resection. We present the first reported case of adult hepatoblastoma in Taiwan. A 52-year-old female suffered from sudden onset of abdominal pain and general weakness for days.Internal bleeding with hemorrhagic shock was suspected and two massive lesions in both lobes of the liver with hemoperitoneum were noted from imaging studies.Surgical resection of the larger left lobe tumor and radio-frequency ablation of the right smaller one were performed. The histopathology diagnosis was of a hepatoblastoma.

Signaling pathway of insulin-like growth factor-Ⅱ as a target of molecular therapy for hepatoblastoma

AIM: To address the possibility that insulin-like growth factor (IGF)-Ⅱ is a growth factor and its signaling pathway so as to develop a molecular therapy for hepatoblastoma. METHODS: Huh-6 and HepG2, human hepatoblastoma cell lines, were used. IGF-Ⅱ was added to the medium deprived of serum. Western blot analysis was performed to clarify the expression of IGF-I receptor (IGF-IR). Inhibitors of IGF-IR (piclopodophyllin, PPP), phosphatidyl-inositol (PI) 3-kinase (LY294002 and Wortmannin), or mitogen-activated protein (MAP) kinase (PD98059) were added to unveil the signaling pathway of IGF-Ⅱ. Cells were analyzed morphologically with hematoxylin-eosin staining to reveal the mechanism of suppression of cell proliferation.RESULTS: IGF-Ⅱ stimulated cells proliferated to 2.7 (269% ± 76%) (mean ± SD) (Huh-6) and 2.1 (211% ± 85%) times (HepG2). IGF-IR was expressed in Huh-6 and HepG2. PPP suppressed the cell number to 44% ± 11% (Huh-6) and 39% ± 5% (HepG2). LY294002 and Wortmannin suppressed the cell number to 30% ± 5% (Huh-6), 44% ± 0.4% (HepG2), 49% ± 1.0% (Huh-6) and 46% ± 1.1% (HepG2), respectively. PD98059 suppressed the cell number to 33% ± 11% for HepG2 but not for Huh-6. When cell proliferation was prohibited, many Huh-6 and HepG2 cells were dead with pyknotic or fragmented nuclei, suggesting apoptosis.CONCLUSION: IGF-Ⅱ was shown to be a growth factor of hepatoblastoma via IGF-I receptor and PI3 kinase which were good candidates for target of molecular therapy.

Success in Treating Hepatoblastoma by Transcatheter Arterial Infusion Combined with Radical Resection:a Case Report

Hepatoblastoma is the most common malignant hepatic tumor in children. About 50% of the cases occur before the age of 18 months and almost all before the age of 2 years. We present an extremely rare case of hepatoblastoma in an 11-year-old girl, which was pathologically verified and treated twice with transcatheter arterial infusion combined with radical resection. To date she has been followed-up for 15 years and has shown normal development after the operation. She has received no chemotherapy or radiation and has displayed no evidence of metastatic or recurrent disease.

miR-181b-5p/SOCS2/JAK2/STAT5 axis facilitates the metastasis of hepatoblastoma

Introduction:Hepatoblastoma(HB)is a malignant liver tumor predominantly found in children and tumor metastasis is one of the main causes of poor prognosis in affected patients.The precise molecular mechanisms responsible for HB metastasis remain incom-pletely understood.However,there is evidence suggesting a connection between the dysregulation of microRNAs(miRNAs)and the progression of tumor metastasis in HB.Methods:The study utilized weighted gene co-expression network analysis(WGCNA)to analyze a miRNA microarray dataset of HB.The expression of miR-181b-5p in HB tissues and cells was detected using quantitative real-time PCR.The impact of miR-181b-5p on the metastatic capacity of HB was evaluated through scratch and Transwell assays.The effects of exogenously expressing miR-181b on the metastatic phenotypes of HB cells were evaluated in vivo.Furthermore,a luciferase reporter assay was performed to validate a potential target of miR-181b-5p in HB.Results:We found that miR-181b-5p was highly expressed in HB tissues and HB cell lines.Overexpression of miR-181b enhanced scratch healing,cell migration,and invasion abilities in vitro,as well as enhancing HB lung metastasis potential in vivo.Dual-luciferase reporter assays showed that Suppressor Of Cytokine Signaling 2(SOCS2)was a direct target of miR-181b.The overexpression of miR-181b resulted in the suppression of SOCS2 expression,subsequently activating the epithelial-mesenchymal transition and JAK2/STAT5 signaling pathways.The rescue experiment showed that SOCS2 overexpression attenuated the effects of miR-181b on HB cells.Conclusion:Our study showed that miR-181b promotes HB metastasis by targeting SOCS2 and may be a potential therapeutic target for HB.

Hepatoblastoma with neonatal necrotizing enterocolitis:Two case reports

We report two children with hepatoblastoma(HB)with a history of neonatal necrotizing enterocolitis(NEC).Case 1 was diagnosed with HB at 5 months of age.Liver enlargement was found during the NEC operation at 3 months of age and then was clinically diagnosed by imaging.After six chemotherapy courses,a partial hepatectomy was performed.Three months after ceasing the chemotherapy,a chest computed tomography scan suggested that distant metastasis of the tumor should be considered,and the lesion was removed.However,9 months after the operation,alpha-fetoprotein concentrations were increased,and abdominal imaging showed a recurrence of the tumor in situ,resulting in a hepatectomy.Case 2 was diagnosed with NEC shortly after birth and underwent an intestinal resection and anastomosis 1 month later.He was diagnosed with HB at 3 years of age.Hepatectomy was performed after five courses of chemotherapy.Chemotherapy was stopped after 10 courses,and alpha-fetoprotein concentrations were normal.At present,both children have survived and are in a healthy condition.Physicians should be aware of the possibility of HB and a history of NEC in children.Premature birth and low birth weight are common factors leading to the pathogenesis of HB and NEC.The association between these two diseases requires further study。

Blastomas of the digestive system in adults:A review

Blastomas,characterized by a mixture of mesenchymal,epithelial,and undifferentiated blastematous components,are rare malignant neoplasms originating from precursor blast cells.This review focuses on digestive system blastomas in adult patients,including gastroblastoma,hepatoblastoma,and pancreatoblastoma.Gastroblastoma is a biphasic,epitheliomesenchymal tumor,with only sixteen cases reported to date.In addition to the characteristic histology,metastasisassociated lung adenocarcinoma transcript 1-glioma-associated oncogene homolog 1 gene fusion is typical,although recently novel ewing sarcoma breakpoint region 1-c-terminal binding protein 1 and patched 1-glioma-associated oncogene homolog 2 fusions have been described.Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty.Pancreatoblastoma,primarily a pediatric tumor,displays acinar differentiation and squamoid nests with other lines of differentiation also present,especially neuroendocrine.Diagnostic approaches for these blastomas include a combination of imaging modalities,histopathological examination,and molecular profiling.The treatment generally involves surgical resection,which may be supplemented by chemotherapy or radiotherapy in some cases.Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes,particularly in the setting of metastases.This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.

Incidence trends and survival prediction of hepatoblastoma in children:a population-based study

Background:Hepatoblastoma is a rare disease that nevertheless accounts for the majority of liver malignancies in children.Due to limited epidemiological data,therapy for hepatoblastoma tends to be individualized.This study aimed to evaluate incidence trends of hepatoblastoma and to develop a nomogram to predict the survival of children with newly diagnosed hepatoblastoma on a population-based level.Methods:Individuals up to 18 years of age with hepatoblastoma recorded in 18 registries of the Surveillance,Epi-demiology,and End Results(SEER)database between 2004 and 2015 were examined.Joinpoint regression analyses were applied to assess incidence trends in annual percentage change(APC).Multivariable Cox regression was used to identify factors associated with overall survival(OS).A nomogram was constructed to predict OS in individual cases based on independent predictors.Concordance index(C-index)and calibration curves were used to evaluate predic-tive performance.Results:Between 2004 and 2015,hepatoblastoma incidence increased significantly(APC,2.2%;95%confidence interval[CI]0.5%to 3.8%,P<0.05).In particular,this increase was observed among 2-to 4-year-old patients,males,and African-Americans.The 5-and 10-year OS rates were 81.5%and 81.0%,respectively.Age of 2 to 4 years,Afri-can-American ethnicity,and no surgery were independent predictors for short OS.Distant disease at presentation was found not to be an independent factor of survival.The nomogram had a C-index of 0.79(95%CI 0.74-0.84)with appropriate calibration curve fitting.Conclusions:We constructed a nomogram that integrates common factors associated with survival for hepatoblas-toma patients.It provides accurate prognostic prediction for children with hepatoblastoma.

Sodium valproate induces mitochondria-dependent apoptosis in human hepatoblastoma cells

Background Sodium valproate inhibits proliferation in neuroblastoma and glioma cells, and inhibits proliferation and induces apoptosis in hepatoblastoma cells. Information describing the molecular pathways of the antitumor effects of sodium valproate is limited; therefore, we explored the mechanisms of action of sodium valproate in the human hepatoblastoma cell line, HepG2.Methods The effects of sodium valproate on the proliferation of HepG2 cells were evaluated by the Walsh-sohema transform and colony formation assays. Sodium valproate-induced apoptosis in HepG2 cells was investigated with fluorescence microscopy to detect morphological changes; by flow cytometry to calculate DNA ploidy and apoptotic cell percentages; with Western blotting analyses to determine c-Jun N-terminal kinases （JNK）, p-JNK, Bcl-2, Bax, and caspase-3 and-9 protein expression levels; and using JC-1 fluorescence microscopy to detect the membrane potential of mitochondria. Statistical analyses were performed using one-way analysis of variance by SPSS 13.0 software.Results Our results indicated that sodium valproate treatment inhibited the proliferation of HepG2 cells in a dose-dependent manner. Sodium valproate induced apoptosis in HepG2 cells as it： caused morphologic changes associated with apoptosis, including condensed and fragmented chromatin; increased the percentage of hypodiploid cells in a dose-dependent manner; increased the percentage of annexin Ⅴ-positive/propidium iodide-negative cells from 9.52％ to 74.87％; decreased JNK and increased phosphate-JNK protein expression levels; reduced the membrane potential of mitochondria; decreased the ratio of Bcl-2/Bax; and activated caspases-3 and-9.Conclusion Sodium valproate inhibited the proliferation of HepG2 cells, triggered mitochondria-dependent HepG2 cell apoptosis and activated JNK.

CTNNB1、TP53蛋白在儿童肝母细胞瘤组织中的表达及与病理特征、预后的关系

目的 探讨钙黏蛋白相关蛋白(cadherin associated protein beta 1,CTNNB1)、肿瘤抑制因子P53(tumor suppressor factor P53,TP53)蛋白在儿童肝母细胞瘤(hepatoblastoma, HB)组织中的表达及与病理特征、预后的关系。方法 选取2017-06/2020-06月作者医院收治的72例HB患儿为研究对象,手术留取癌组织标本及对应的癌旁组织。连续随访3年,记录患儿的预后生存情况,并计算总生存率(overall survival, OS)。采用免疫组织化学法检测CTNNB1、TP53蛋白在HB患儿癌组织及癌旁组织中的表达情况;采用χ~2检验分析CTNNB1、TP53蛋白表达与HB患儿临床病理特征的关系;采用多因素Cox回归分析探讨HB患儿预后的影响因素。结果 HB患儿癌组织中CTNNB1阳性表达率(76.39%)高于对照组(43.06%),TP53阴性表达率(70.83%)高于对照组(38.89%)(P均<0.05)。初诊甲胎蛋白浓度≥100μg/L、POST-TEXT分期Ⅲ~Ⅳ期、肿瘤直径≥3 cm、有肿瘤侵袭或转移HB患儿的CTNNB1阳性表达率、TP53阴性表达率高于初诊甲胎蛋白<100μg/L、POST-TEXT分期Ⅰ~Ⅱ期、肿瘤直径<3 cm、无肿瘤侵袭或转移HB患儿(P均<0.05)。初诊甲胎蛋白≥100μg/L、POST-TEXT分期Ⅲ~Ⅳ期、有肿瘤侵袭或转移、CTNNB1阳性表达、TP53阴性表达的HB患儿3年OS低于初诊甲胎蛋白<100μg/L、POST-TEXT分期Ⅰ~Ⅱ期、无肿瘤侵袭或转移、CTNNB1阴性表达、TP53阳性表达的HB患儿(P均<0.05)。多因素Cox回归分析结果显示,POST-TEXT分期Ⅲ~Ⅳ期(HR=2.077,95%CI:1.423~3.032)、有肿瘤侵袭或转移(HR=2.291,95%CI:1.536~3.417)、CTNNB1阳性表达(HR=2.757,95%CI:1.781~4.268)、TP53阴性表达(HR=2.477,95%CI:1.635~3.753)是HB患儿预后的独立危险因素(P<0.05)。结论 HB患儿癌组织中CTNNB1主要呈阳性表达,而TP53主要呈阴性表达,二者与初诊甲胎蛋白、POST-TEXT分期、肿瘤直径、有肿瘤侵袭或转移密切相关,有望作为评估HB患儿预后的生物学指标。

不同病理类型儿童肝母细胞瘤的超声表现分析

目的分析不同病理类型儿童肝母细胞瘤的超声表现。方法回顾性分析我院收治的36例肝母细胞瘤患儿的超声及病理资料,总结不同病理类型(完全上皮细胞型、不伴畸胎样混合型、伴畸胎样混合型)儿童肝母细胞瘤的超声表现。结果36例肝母细胞瘤中,完全上皮细胞型13例,其中肿瘤位于肝右叶7例,左右叶3例,左叶2例,尾状叶1例,平均肿瘤大小(286.34±23.75)mm^(3),形态规则10例,边界清晰9例,瘤内坏死囊变11例;不伴畸胎样混合型9例,其中肿瘤位于肝右叶4例,左右叶2例,左叶2例,尾状叶1例,平均肿瘤大小(282.59±21.98)mm^(3),形态规则6例,边界清晰5例,瘤内坏死囊变7例;伴畸胎样混合型14例,其中肿瘤位于肝右叶8例,位于左右叶3例,左叶2例,尾状叶1例,平均肿瘤大小(284.61±22.07)mm^(3),形态规则11例,边界清晰10例,瘤内坏死囊变12例。不同病理类型肝母细胞瘤肿瘤位置、大小、形态、边界及瘤内坏死囊变占比比较差异均无统计学意义。完全上皮细胞型、不伴畸胎样混合型、伴畸胎样混合型肿瘤内部回声和钙化情况比较差异均有统计学意义(均P<0.05);其中完全上皮细胞型肿瘤内部强回声占比高于不伴畸胎样混合型和伴畸胎样混合型,不伴畸胎样混合型肿瘤内部强回声占比高于伴畸胎样混合型,完全上皮细胞型肿瘤钙化占比低于不伴畸胎样混合型和伴畸胎样混合型,差异均有统计学意义(均P<0.05)。结论不同病理类型儿童肝母细胞瘤内部强回声和钙化均存在差异,其可为临床初步判断肝母细胞瘤患儿病理分型提供参考依据。

Surgical management of intrahepatic vessels in children with stage III/IV hepatoblastoma

Background Hepatoblastoma （HB） is a rare childhood tumor. We investigated the effect of intraoperative management of the intrahepatic major vessels in children with HB. Methods Between April 2005 and August 2012, surgical resection was performed on 50 children with hepatoblastoma. These children were divided into a vessel-ligation group （n=20） and a vessel-repair group （n=30）. In the vessel-ligation group, the intrahepatic major vessels were ligated and removed together with the tumor and the affected liver lobe/liver parenchyma. In the vessel-repair group, the affected intrahepatic major vessels were dissected and preserved as much as possible and the normal liver lobe/liver parenchyma and blood supply from these vessels were also preserved. The outcomes were analyzed by postoperative follow-up. Results In the vessel-ligation group, two patients gave up surgery, six patients underwent palliative resection, and 12 patients underwent en bloc resection; four patients died of liver failure and eight patients fully recovered and were discharged. In the vessel-repair group, all 30 patients underwent en bloc resection and were discharged after satisfactory healing. After a follow-up time of 5-36 months （median： 20 months）, two patient in the vessel-ligation group survived and 22 patients in the vessel-repair group survived. Conclusions Patients with HB can be successfully treated by tumor resection with vascular repair. This method prevents postoperative liver failure, ensures patient safety during the perioperative period, and allows for early chemotherapy.