Association between lncRNA-H19 polymorphisms and hepatoblastoma risk in an ethic Chinese population.

H19 polymorphisms are associated with increased susceptibility to several cancers; however, their role in hepatoblastoma remains unclear. In this study, we investigated the association between three H19 polymorphisms (rs2839698 G>A, rs3024270 C>G, rs217727 G>A) and hepatoblastoma susceptibility in 213 hepatoblastoma patients. The rs2839698 and rs3024270 polymorphisms were associated with significantly increased hepatoblastoma risk, with the GG genotype associated with a higher risk of hepatoblastoma than the CC genotype at the rs3024270 locus. The rs217727 polymorphism was associated with significantly decreased hepatoblastoma risk, with the AG genotype associated with a lower risk of hepatoblastoma than the GG genotype. These findings were confirmed by combined analysis, and stratification analysis revealed that age, gender and clinical stage were associated with increased hepatoblastoma susceptibility. The GGG and AGG haplotypes were significantly associated with increased hepatoblastoma risk compared with the GCA reference (rs2839698, rs3024270, rs217727). The rs2839698 and rs3024270 polymorphisms correlated with decreased MRPL23-AS1 expression, whereas the rs217727 polymorphism was associated with increased MRPL23-AS1 expression. Overall, the H19 rs2839698, rs3024270 and rs217727 polymorphisms were associated with hepatoblastoma susceptibility in a Chinese Han population.

Analysis of Clinical Characteristics, Pathological Changes and Changes of Interleukin-6 (IL-6) and C-Reactive Protein (CRP) in Children with Castleman's Disease.

BACKGROUND The aim of this study was to analyze the pathological changes, clinical characteristics and changes in immunity, interleukin-6 (IL-6) and C-reactive protein (CRP) in children with Castleman's disease (CD). MATERIAL AND METHODS A total of 15 CD child patients were enrolled as observation group, while 20 normal children receiving healthy examination were enrolled as healthy control group. The pathological changes, clinical characteristics and changes in immunity and serum IL-6 and CRP expressions were retrospectively analyzed in observation group. RESULTS The clinical manifestation of unicentric CD (UCD) was mainly enlargement of cervical lymph nodes without liver-spleen enlargement and fever, and the major pathological type was the hyaline-vascular type. Multicentric CD (MCD) child patients all had anemia, fever and other systemic symptoms, and the major pathological type was the plasma-cell type. There were expressions of the immune indexes, including cluster of differentiation 3 (CD3), CD4, CD8, CD20, and CD79, in a certain degree, while CD138 and VS38C expressions displayed the polyclonal proliferation of plasma cells, rather than neoplastic proliferation. The Epstein-Barr virus and human herpes virus-8 detection results were negative, and CD21 in follicular dendritic cells in abnormal germinal center was positive. The expression levels of serum IL-6 and CRP in observation group were higher than those in control group (P.

Outcomes of children with hepatoblastoma who underwent liver resection at a tertiary hospital in China: a retrospective analysis.

BACKGROUND: To report the outcomes of hepatoblastoma resected in our institution. METHODS: We diagnosed 135 children with hepatoblastoma at our institution between January 2010 and December 2017. Patients who underwent liver resection were included for analysis. However, patients who abandoned treatment after diagnosis were excluded from analysis, but their clinical characteristics were provided in the supplementary material. RESULTS: Forty-two patients abandoned treatment, whereas 93 patients underwent liver resection and were included for statistical analysis. Thirty-six, 23, 3, and 31 patients had PRETEXT stages II, III, IV, and unspecified tumours, respectively. Seven patients had ruptured tumour; 9 had lung metastasis (one patient had portal vein thrombosis concurrently). Sixteen patients underwent primary liver resection; 22, 25, and 30 patients received cisplatin-based neoadjuvant chemotherapy and delayed surgery, preoperative transarterial chemoembolization (TACE) and delayed surgery, and a combination of cisplatin-based neoadjuvant chemotherapy, TACE, and delayed surgery, respectively. Forty patients had both PRETEXT and POST-TEXT information available for analysis. Twelve patients were down-staged after preoperative treatment, including 2, 8, and 2 patients from stages IV to III, III to II, and II to I, respectively. Ten patients with unspecified PRETEXT stage were confirmed to have POST-TEXT stages II (n = 8) and I (n = 2) tumours. Seven tumours were associated with positive surgical margins, and 12 patients had microvascular involvement. During a median follow-up period of 30.5 months, 84 patients survived without relapse, 9 experienced tumour recurrence, and 4 died. The 2-year event-free survival (EFS) and overall survival (OS) rates were 89.4 ± 3.4%, and 95.2 ± 2.4%, respectively; they were significantly better among patients without metastasis (no metastasis vs metastasis: EFS, 93.5 ± 3.7% vs 46.7 ± 19.0%, adjusted p = 0.002. OS, 97.6 ± 2.4% vs 61.0 ± 18.1%, adjusted p = 0.005), and similar among patients treated with different preoperative strategies (chemotherapy only vs TACE only vs Both: EFS, 94.7 ± 5.1% vs 91.7 ± 5.6% vs 85.6 ± 6.7%, p = 0.542. OS, 94.1 ± 5.7% vs 95.7 ± 4.3% vs 96.7 ± 3.3%, p = 0.845). CONCLUSION: The OS for patients with hepatoblastoma who underwent liver resection was satisfactory. Neoadjuvant chemotherapy and TACE seemed to have a similar effect on OS. However, the abandonment of treatment by patients with hepatoblastoma was common, and may have biased our results.

Liver biopsy for hepatoblastoma: a single institution's experience.

BACKGROUND/PURPOSE: Hepatoblastoma diagnoses require liver biopsies. We aimed to investigate factors affecting the success of liver biopsy for hepatoblastoma diagnoses. METHODS: Data from patients with hepatoblastoma, including their demographic and clinical data, biopsy procedure information, pathologic diagnoses and subclassification, and surgical complications, were retrospectively reviewed. RESULTS: Of 153 patients who underwent liver biopsy, 28, 93, and 31 underwent computed tomography-guided, digital subtraction angiography-guided, and ultrasound-guided percutaneous biopsies, respectively, and one underwent a laparoscopic liver biopsy. One patient developed postoperative bleeding requiring a blood transfusion. The median number of specimens collected was 3. One-hundred and forty-four (94.1%) patients' HB diagnoses were confirmed through biopsies, and 96 (62.7%) patients' HB diagnoses were subclassified. Seven surgeons and eight interventional radiologists performed the biopsies. The diagnostic success rate did not correlate with the biopsy technique or the specialist who performed the biopsy. Significantly more specimens were biopsied from the patients whose diagnoses were subclassified (3.34 ± 1.08) than from those whose diagnoses were not subclassified (2.81 ± 0.79). Surgeons tended to collect more specimens than the interventional radiologists. CONCLUSION: Percutaneous liver biopsy is safe and effective for diagnosing hepatoblastoma, and its complication rate is very low. Collecting >3 pieces of tissue is preferred. LEVEL OF EVIDENCE: III.

Impact of 3D printing technology on the comprehension of surgical liver anatomy.

BACKGROUND AND AIMS: Surgical planning in liver resection depends on the precise understanding of the three-dimensional (3D) relation of tumors to the intrahepatic vascular trees. This study aimed to investigate the impact of 3D printing (3DP) technology on the understanding of surgical liver anatomy. METHODS: We selected four hepatic tumors that were previously resected. For each tumor, a virtual 3D reconstruction (VIR) model was created from multi-detector computed tomography (MDCT) and was prototyped using a 3D printer. Forty-five surgical residents were evenly assigned to each group (3DP, VIR, and MDCT groups). After evaluation of the MDCT scans, VIR model, or 3DP model of each tumor, surgical residents were asked to assign hepatic tumor locations and state surgical resection proposals. The time used to specify the tumor location was recorded. The correct responses and time spent were compared between the three groups. RESULTS: The assignment of tumor location improved steadily from MDCT, to VIR, and to 3DP, with a mean score of 34.50, 55.25, and 80.92, respectively. These scores were out of 100 points. The 3DP group had significantly higher scores compared with other groups (p < 0.001). Furthermore, 3DP significantly improved the accuracy of surgical resection proposal (p < 0.001). The mean accuracy of the surgical resection proposal for 3DP, VIR, and MDCT was 57, 25, and 25%, respectively. The 3DP group took significantly less time, compared with other groups (p < 0.005). The mean time spent on assessing the tumor location for 3DP, VIR, and MDCT groups was 93, 223, and 286 s, respectively. CONCLUSIONS: 3D printing improves the understanding of surgical liver anatomy for surgical residents. The improved comprehension of liver anatomy may facilitate laparoscopy or open liver resection.

The rs2147578 C > G polymorphism in the Inc-LAMC2-1:1 gene is associated with increased neuroblastoma risk in the Henan children.

BACKGROUND: The rs2147578 C > G polymorphism in the long non-coding RNA gene Lnc-LAMC2-1:1 is associated with increased susceptibility to a few types of cancers. However, its role in neuroblastoma has not been evaluated yet. METHODS: We investigated the association between the lnc-LAMC2-1:1 rs2147578 C > G polymorphism and neuroblastoma susceptibility in Chinese Han populations. A total of 393 neuroblastoma cases and 812 healthy individuals from the Henan and Guangdong provinces were enrolled and subjected to genotyping. Odds ratio (OR) and 95% confidence interval (CI) were used to determine the strength of the association of interest. RESULTS: Combined analysis revealed that the lnc-LAMC2-1:1 rs2147578 C > G polymorphism was associated with increased neuroblastoma susceptibility (CG vs. CC: adjusted OR = 1.33, 95% CI = 1.01-1.75, P = 0.045; CG/GG vs. CC: adjusted OR = 1.34, 95% CI = 1.03-1.74, P = 0.028). In stratification analysis, children under 18 months with rs2147578 CG/GG genotypes had an increased neuroblastoma risk (adjusted OR = 1.70, 95% CI = 1.08-2.67, P = 0.022). Females with rs2147578 CG/GG genotypes also had increased neuroblastoma susceptibility (adjusted OR = 2.08, 95% CI = 1.37-3.18, P = 0.0007). In addition, children with lnc-LAMC2-1:1 rs2147578 CG/GG genotypes were prone to develop earlier stages of neuroblastoma (adjusted OR = 1.46, 95% CI = 1.01-2.12, P = 0.046). CONCLUSIONS: The Lnc-LAMC2-1:1 rs2147578 C > G polymorphism may contribute to increased neuroblastoma susceptibility in children of Henan province.

Impact of 3D Printing Technology on Comprehension of Surgical Anatomy of Retroperitoneal Tumor.

OBJECTIVES: To investigate the impact of 3D printed model on understanding of surgical anatomy of retroperitoneal tumor. MATERIALS AND METHODS: Three-dimensional model was printed, based on multi-detectors computed tomography (MDCT) of a retroperitoneal tumor. Participants (10 students, 10 residents and 10 surgeons) were asked to identify vasculatures which were important in resection of the tumor, after viewing MDCT images, 3D visualization model and 3D printed model, respectively. Regarding this tumor, left renal vein (LRV), right renal pedicles (RRP) and inferior vena cava (IVC) were chosen as indicators to assess participants' performances. Identification of vasculatures was evaluated and a score was given (1 point = success; 0 point = failure). The total number and percentage of correct identification were used to measure how these three types of anatomic presentation were able to transfer in terms of anatomical recognition. Recorded data were analyzed both pooling together data from three groups of participants and separately for each group. RESULTS: In analysis of overall comparison among 3D printing, 3D visualization and MDCT, recognition of all three vasculatures simultaneously was 83.33, 73.33 and 46.67%, respectively (P = 0.007); recognition of LRV was 90, 80 and 63.33% (P = 0.043), respectively; recognition of RRP was 96.67, 83.33 and 73.33% (P = 0.035), respectively; recognition of IVC was 93.33, 90 and 80% (P = 0.366), respectively. In subgroup analysis of performances of three groups of participants, no significant differences regarding anatomic recognition were observed among MDCT, 3D visualization and 3D printed model for each group of participants. CONCLUSION: Three-dimensional printed model improved the understanding of surgical anatomy of retroperitoneal tumor.

Neonatal Gastric Perforation: Case Series and Literature Review.

PURPOSE: We reported clinical findings of neonatal gastric perforation in a tertiary children's hospital. PATIENTS AND METHODS: Retrospective chart reviews were conducted for neonatal gastric perforation between 1980 and 2016. Factors including sex, gestational age, birth weight, age, main symptoms and signs, white blood cell count (WBC), surgical intervention time (time between development of main symptom and surgical intervention), surgical findings, pathologic results, clinical outcomes, and causes of death were collected. RESULTS: Sixty-eight patients were identified. In total, 76.5% were male infants, the median age was 4 days, median birth weight was 2500 g, and 42.6% were premature. Abdominal distention and vomiting were the most common symptoms, and pneumoperitoneum was the most common radiographic finding. The median surgical intervention time was 51 h (range 8-312). In total, 73.5% of perforations occurred in the great curvature, 17.6% in the lesser curvature, and 8.9% unspecified. The median perforation size was 4 cm (range 0.2-16). Associated gastrointestinal anomalies were found in 20.6% of patients, and the most common anomaly was intestinal malrotation. Of the 51 patients with pathologic results, 11 showed the presence of musculature in the perforated gastric wall, while 40 showed the absence of musculature. Of the 66 patients with known clinical outcomes, 26 (39.4%) died, 23 of who died of infection. Among those aforementioned factors, WBC has a significant impact on survival. The mortality for four arbitrary divided year groups (1980-1989, 1990-1999, 2000-2009, and 2010-2016) was 100, 50, 31.6, and 16.7%, respectively. CONCLUSIONS: The mortality of neonatal gastric perforation is constantly decreasing. Associated gastrointestinal anomalies and the presence of musculature are found in a minority of this condition.

Surgical management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed.

PURPOSE: Clinical researches about the management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed are limited. We report the surgical outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed in a single institution. METHODS: Ganglioneuroma and ganglioneuroblastoma-intermixed diagnosed and resected between May 2009 and May 2015 in a tertiary children's hospital were retrospectively reviewed. Patients' demographic data, INSS stage, surgical complications, residual tumor size and outcomes were collected. RESULTS: Thirty-four patients were included in the current study. All had localized tumors and were surgically managed. The overall acute complications rates were 8.8% (3/34) and none were fatal. Thirty-three of 34 patients had at least macroscopic tumor resection. Six patients had radiographically detected residual tumor after surgery, 25 none and 3 undocumented. Thirty-three (97.1%) patients were alive during a median follow-up of 36 months (range 1-82). In subgroup analysis, no significant difference regarding surgical complications and survival was found between ganglioneuroma and ganglioneuroblastoma-intermixed. Increased complete resection rates were observed in thoracic tumor compared with abdominal ones (p = 0.03). However, no significant difference (p = 0.089) regarding overall survival was found between patients with residual tumors and those without. Of the six patients with residual tumors, three showed complete resolution, two were unchanged and one died 3 years after initial surgery (the only death in this study). CONCLUSION: Ganglioneuroma and ganglioneuroblastoma-intermixed can be safely and effectively resected, the residual tumor seems not to influence overall survival.

Clinical Application of Indocyanine Green Fluorescence Imaging in the Resection of Hepatoblastoma: A Single Institution's Experiences.

Purpose: Indocyanine green (ICG) fluorescence imaging is becoming increasingly popular in adult oncologic surgery, but remains relatively uncommon in pediatric oncologic surgery. Herein, we report our experience with the use of ICG fluorescence imaging in the resection of hepatoblastoma (HB). Patients and Methods: Hepatoblastoma patients who underwent liver resection with ICG fluorescence imaging between January 2020 and March 2021 were included in this study. Patients' demographic data, clinical information, and detailed information of the use of ICG fluorescence imaging were retrospectively reviewed. Results: Sixteen HB patients underwent ICG fluorescence imaging-guided liver resection. There were 11 males and 5 females, age ranged from 8 to 134 months. The initial alpha-fetoprotein ranged from 436 to 528,390 ng/ml. There were one pre-treatment extent of tumor stage I, nine stage II, four stage III, and two stage IV. Three patients underwent up-front hepatectomy, 13 patients received 2-8 cycles of platinum-based neoadjuvant chemotherapy and underwent delayed hepatectomy. ICG (0.5 mg/kg) was given intravenously 48-72 h prior to surgery. The operative time ranged from 180 to 400 min. All patients achieved negative surgical margins. In two patients, ICG identify additional lesions which were not detected in preoperative imaging. Conclusion: ICG fluorescence imaging is useful in the resection of HB and may detect small lesions not shown in preoperative imaging.

SNHG25 facilitates SNORA50C accumulation to stabilize HDAC1 in neuroblastoma cells.

Increasing studies have pointed out that small nucleolar RNAs (snoRNAs) and their host genes (SNHGs) have multi-functional roles in cancer progression. Bioinformatics analysis revealed the importance of snoRNA host gene 25 (SNHG25) in neuroblastoma (NB). Hence, we further explored the function and molecular mechanism of SNHG25 in NB. Our study revealed that SNHG25 expression was upregulated in NB cells. Through loss-of-function assays, we discovered that silencing of SNHG25 suppressed NB cell proliferation, invasion, and migration. Moreover, we found that SNHG25 positively regulated snoRNA small nucleolar RNA, H/ACA box 50 C (SNORA50C) in NB cells, and SNORA50C depletion had the same function as SNHG25 silencing in NB cells. Moreover, we proved that SNHG25 recruited dyskerin pseudouridine synthase 1 (DKC1) to facilitate SNORA50C accumulation and associated small nucleolar ribonucleoprotein (snoRNP) assembly. In addition, it was manifested that SNHG25 relied on SNORA50C to inhibit ubiquitination of histone deacetylase 1 (HDAC1), thereby elevating HDAC1 expression in NB cells. Further, HDAC1 was proven to be a tumor-facilitator in NB, and SNORA50C contributed to NB cell growth and migration through the HDAC1-mediated pathway. In vivo xenograft experiments further supported that SNHG25 promoted NB progression through SNORA50C/HDAC1 pathway. Our study might provide a novel sight for NB treatment.

TTF1 suppresses neuroblastoma growth and induces neuroblastoma differentiation by targeting TrkA and the miR-204/TrkB axis.

Neuroblastoma (NB) is the most common extracranial malignant solid tumor in children. We found that TTF1, TrkA, and miR-204 were lowly expressed, whereas TrkB was highly expressed in undifferentiated NB tissues. Meanwhile, TTF1 expression correlated positively with TrkA and miR-204 expression but negatively with TrkB expression. The TTF1 promoter was hypermethylated in undifferentiated NB tissues and SK-N-BE cells, leading to TTF1 downregulation. We also identified miR-204, which directly targets TrkB, as a transcriptional target of TTF1. Functionally, TTF1 suppressed proliferation, migration, and invasion of NB cells, whereas induced cell cycle arrest, apoptosis, and autophagy of NB cells by regulating TrkA and the miR-204-TrkB axis. Furthermore, TTF1 suppressed tumor growth and promoted neurogenic differentiation in a NB xenograft mouse model. Our study demonstrates that TTF1 reduces tumor growth and induces neurogenic differentiation in NB by directly targeting TrkA and the miR-204/TrkB axis.

lncRNA-uc003opf.1 rs11752942 A>G polymorphism decreases neuroblastoma risk in Chinese children.

Recent studies have revealed that long non-coding RNAs (lncRNAs) play critical roles in the tumorigenesis and proliferation of human cancer. Several polymorphisms of lncRNAs have been found to be involved in the risk of neuroblastoma (NB). However, studies on the relationship between polymorphisms in lncRNA exons and NB are infrequent. We evaluated the association between rs11752942 A > G polymorphism in lnc-RNA-uc003opf.1 exon and neuroblastoma susceptibility by performing a hospital-based study with 275 patients and 531 controls. Odds ratios (ORs) and 95% confidence intervals (CIs) assessed by using logistic regression models were used to determine the strength of the association. We found that the rs11752942 G allele is significantly associated with decreased neuroblastoma risk (AG vs. AA: adjusted OR = 0.72, 95% CI = 0.53-0.98, P = 0.038; and AG/GG vs. AA: adjusted OR = 0.74, 95% CI = 0.55-0.99, P = 0.045) after adjusting for age and gender. This association was more prominent in females, subjects with tumor in the mediastinum or early-stage. Furthermore, the expression quantitative trait locus analysis indicated that rs11752942 G was associated with decreased expression of its neighboring gene LRFN2 mRNA. These results indicate that lncRNA-uc003opf.1 may be a novel potentially functional lncRNA that may be used as a predictive marker, for it might contribute to decreased neuroblastoma risk.

Association of CMYC polymorphisms with hepatoblastoma risk.

BACKGROUND: Single-nucleotide polymorphisms (SNPs) in genes may affect gene expression and contribute to cancer susceptibility. This study aimed to explore the association between CMYC gene polymorphisms and hepatoblastoma risk. METHODS: Hepatoblastoma patients and cancer-free controls were recruited and matched by age and sex. Genotypes were determined by TaqMan, and the strength of the association of interest was determined by calculating odds ratios (ORs) and 95% confidence intervals (CIs). The distributions of various CMYC genotypes among subjects were recorded, followed by analyses of associations between CMYC polymorphisms and hepatoblastoma risk. RESULTS: A total of 213 hepatoblastoma patients and 958 cancer-free controls were enrolled. No significant associations between the CMYC rs4645943 and rs2070583 polymorphisms and hepatoblastoma risk were found (all P>0.05). In stratification analysis based on age, sex, and clinical stage, the CMYC rs4645943 and rs2070583 polymorphisms were not associated with hepatoblastoma susceptibility (all P>0.05). CONCLUSIONS: Thus, the CMYC rs4645943 and rs2070583 polymorphisms were not associated with hepatoblastoma risk in the study cohort.

Surgical risk factors of retroperitoneal teratoma resection in children.

BACKGROUND: Retroperitoneal teratoma is uncommon and carries considerable surgical risks. Some preoperative clinical and radiographic features could be predictive of surgical complication risk. We aimed to identify surgical risk factors that predicted perioperative complications. METHODS: Childhood retroperitoneal teratoma cases operated on at Guangzhou Women and Children's Medical Center were retrospectively reviewed. Demographic features; clinical, laboratory, radiographic, and intraoperative findings; perioperative complications; and pathology results were assessed. RESULTS: Between May 2000 and December 2017, 152 childhood retroperitoneal teratomas were resected from 102 female patients (median age 5.75 months). Sixty-three patients (41%) experienced perioperative complications (79 intraoperative and 5 postoperative), including kidney excision (4 patients) and adrenal gland excision (1 patient). Among 113 patients with preoperative computed tomography/magnetic resonance images, 112 (99%), 111 (98%), and 113 (100%) demonstrated artery, vein, and organ distortion, respectively, and 28 (25%) had vessels encased by tumors. Patients with perioperative complications had more veins and organs distorted by tumors. In multivariate analysis, the numbers of vessels encased and organs distorted by tumors were significantly associated with perioperative complications (odds ratio = 1.45 and 1.69, 95% confidence interval: 1.00-2.10 and 1.19-2.41, respectively). CONCLUSIONS: Retroperitoneal teratoma resection has a high perioperative complication rate. Teratomas encompassing the vasculature and distorting organs were associated with increased surgical risk. Additional studies aiming to better define the surgical approach to retroperitoneal teratoma are warranted. LEVELS OF EVIDENCE: III.

LINC00673 rs11655237 C>T Polymorphism Impacts Hepatoblastoma Susceptibility in Chinese Children.

BACKGROUND: Hepatoblastoma (HB) is the most common hepatic malignancy in children, accounting for approximately 80% of all childhood liver tumors. Previous genome-wide association studies (GWASs) have found that the LINC00673 rs11655237 C>T polymorphism is associated with the risk of several different adult cancers. However, the association between this polymorphism and HB susceptibility remains unclear. METHODS: We analyzed the association between the LINC00673 rs11655237 C>T polymorphism and HB susceptibility in a hospital-based study of Chinese children. We enrolled 213 HB patients and 958 healthy controls with genotypes determined by TaqMan, and the strength of the association of interest was determined by calculating odds ratios (ORs) and 95% confidence intervals (CIs). FINDINGS: We found a significant association between the LINC00673 rs11655237 C>T polymorphism and HB risk (CT/TT compared with CC: adjusted OR = 1.40, 95% CI = 1.04-1.88, p = 0.029). Furthermore, stratified analysis indicated that rs11655237 T allele carriers in the following subgroups were more likely to develop HB: children older than 17 months, males, and those with tumors of clinical stages III + IV. INTERPRETATION: In conclusion, we confirmed that the LINC00673 rs11655237 C>T polymorphism may be associated with HB susceptibility. Prospective studies with larger sample sizes and patients of different ethnicities are needed to validate our findings.

Association of the TP53 rs1042522 C>G polymorphism and hepatoblastoma risk in Chinese children.

The TP53 gene encodes an important class of cell cycle and tumor-suppressing factors that play critical roles in maintaining genomic stability. The TP53 Arg72Pro (rs1042522 C>G) polymorphism has been reported to be associated with the risk of several types of adult cancers; however, its risk for pediatric cancers remains unclear. Here, we analyzed the association of the TP53 gene rs1042522 C>G polymorphism with hepatoblastoma (HB) susceptibility in a hospital-based study among Chinese children. A total of 213 HB patients and 958 healthy controls were enrolled in the study. Genotypes were determined by a TaqMan assay, and the strength of the association was assessed by the odds ratios and 95% confidence intervals generated from logistic regression models, adjusted for age, gender, and clinical stage. No significant association between the TP53 rs1042522 C>G polymorphism and HB susceptibility was detected in the main analysis or in stratification analyses of age, gender, and clinical stages. Overall, the TP53 gene rs1042522 C>G polymorphism is not associated with HB susceptibility in the Chinese population, other polymorphisms alone or in combination should be investigated to further clarify HB susceptibility.

NRAS and KRAS polymorphisms are not associated with hepatoblastoma susceptibility in Chinese children.

BACKGROUND: Hepatoblastoma is the most common hepatic malignancy in children, accounting for approximately 80% of all childhood liver tumors. KRAS and NRAS, members of the RAS gene family, are closely linked to tumorigenesis, and are frequently mutated in a variety of malignancies. They may thus play critical roles in tumorigenesis. However, there are few studies on the association between the RAS gene polymorphisms and risk of hepatoblastoma. METHODS: We investigated whether the polymorphisms at these genes are associated with hepatoblastoma susceptibility in a hospital-based study of 213 affected Chinese children and 958 cancer-free controls. Genotypes were determined by TaqMan assay, and association with hepatoblastoma risk was assessed based on odds ratios and 95% confidence intervals. RESULTS: No significant differences were observed between patients and controls in terms of age and gender frequency. All NRAS and KRAS genotypes are in Hardy-Weinberg equilibrium in the entire study population. We did not observe any significant association between hepatoblastoma risk and polymorphisms at NRAS and KRAS. The association between selected polymorphisms and hepatoblastoma risk was assessed after stratification by age, gender, and clinical stage. However, no significant association was observed even after stratification by age, gender, and clinical stage. CONCLUSIONS: The data suggest that NRAS and KRAS polymorphisms are irrelevant to hepatoblastoma susceptibility among Chinese population.

The impact of using three-dimensional printed liver models for patient education.

Objective To investigate the impact of using a three-dimensional (3D) printed liver model for patient education. Methods Children with hepatic tumours who were scheduled for hepatectomy were enrolled, and patient-specific 3D liver models were printed with photosensitive resin, based on computed tomography (CT) images. Before surgery, their parents received information regarding liver anatomy, physiology, tumour characteristics, planned surgery, and surgical risks using these CT images. Then, parents completed questionnaires regarding this information. Thereafter, 3D printed models of each patient were presented along with an explanation of the general printing process, and the same questionnaire was completed. The median number of correct responses in each category before and after the 3D printed model presentation was compared. Results Seven children and their 14 parents were enrolled in the study. After the presentation of 3D printed models, parental understanding of basic liver anatomy and physiology, tumour characteristics, the planned surgical procedure, and surgical risks significantly improved. Parents demonstrated improvements in their understanding of basic liver anatomy by 26.4%, basic liver physiology by 23.6%, tumour characteristics by 21.4%, the planned surgical procedure by 31.4%, and surgical risks by 27.9%. Conclusions Using 3D printed liver models improved parental education regarding the understanding of liver anatomy and physiology, tumour characteristics, surgical procedure, and associated surgical risks.

Ruptured hepatoblastoma successfully treated with cisplatin monochemotherapy: A case report.

Traditionally, ruptured hepatoblastoma is considered a high-risk occurrence, necessitating a chemotherapy regimen usually consisting of cisplatin alternating with carboplatin plus doxorubicin, based on International Childhood Liver Tumours Strategy Group studies. However, ruptured hepatoblastomas with intact hepatic capsules may represent a unique subgroup that may be successfully treated with TAE, cisplatin monotherapy, and surgical excision. We herein present a case of ruptured hepatoblastoma (pretreatment tumor extension stage II) in a 1-year-old female patient that was successfully managed with transarterial embolization (TAE), eight courses of cisplatin chemotherapy and surgical removal. The patient currently remains disease-free for >12 months. Given the rarity of ruptured hepatoblastomas, further study of patients within this subgroup is required to confirm the findings of the present study.