Selection of surgical modality for massive splenomegaly in children.

BACKGROUND: Laparoscopic splenectomy (LS), a treatment for both benign and malignant splenic diseases, can prove technically challenging in patients with massive splenomegaly. In particular, the optimal surgical modality for treating massive splenomegaly in children remains controversial. METHODS: The clinicopathologic data of 289 pediatric patients undergoing splenectomy for massive splenomegaly were studied in a retrospective analysis. Accordingly, the patients were classified into the LS surgery group and open splenectomy (OS) surgery group. In the laparoscopy cohort, they were separated into two subgroups according to the method of surgery: the multi-incision laparoscopic splenectomy (MILS) and the single-incision laparoscopic splenectomy (SILS) surgery groups, respectively. Patient demographics, clinical data, surgery, complications, and postoperative recovery underwent analysis. Concurrently, we compared the risk of adverse laparoscopic splenectomy outcomes utilizing univariable and multivariable logistic regression. RESULTS: The total operation time proved remarkably shorter in the OS group in contrast to the LS group (149.87 ± 61.44 versus 188.20 ± 52.51 min, P < 0.001). Relative to the OS group, the LS group exhibited lowered postoperative pain scores, bowel recovery time, and postoperative hospitalization time (P < 0.001). No remarkable difference existed in post-operation complications or mortality (P > 0.05). Nevertheless, the operation duration was remarkably longer in the SILS surgery group than in the MILS surgery group (200 ± 46.11 versus 171.39 ± 40.30 min, P = 0.02). Meanwhile, the operative duration of MILS and SILS displayed a remarkable positive association with splenic length. Moreover, the operative duration of SILS displayed a remarkable positive association with the age, weight, and height of the sick children. Splenic length proved an independent risk factor of adverse outcomes (P < 0.001, OR 1.378). CONCLUSIONS: For pediatric patients with massive splenomegaly who can tolerate prolonged anesthesia and operative procedures, LS surgery proves the optimal treatment regimen. SILS remains a novel surgery therapy which may be deemed a substitutional surgery approach for treating massive splenomegaly.

Surgical Management of Hirschsprung's Disease: A Comparative Study Between Conventional Laparoscopic Surgery, Transumbilical Single-Site Laparoscopic Surgery, and Robotic Surgery.

Background: Hirschsprung's disease (HD) is a commonly digestive malformation in children that usually requires surgery. This study aims to evaluate the short-term efficacy of conventional laparoscopic surgery (CLS), transumbilical single-hole laparoscopic surgery (TU-LESS), and robotic surgery (RS) in the treatment of Hirschsprung's disease. Methods: 90 patients with Hirschsprung's disease undergone laparoscopic surgery at our center between 2015 and 2019, divided into three groups (group CLS, TU-LESS and RS), were retrospectively analysed. Results: CLS and TU-LESS group showed no significant difference in operation duration (P > 0.05) but shorter operation duration than the RS group (P < 0.05). RS group had highest overall SCAR scores, while TU-LESS group had the lowest one (P < 0.05). Other parameters such as operative blood loss, hospital stays, recovery time of digestive function, postoperative complications had no significant difference among the three groups (P > 0.05). Conclusion: The three surgical methods for HD revealed similar efficacy, where TU-LESS and CLS spent less time than RS; TU-LESS led to the most aesthetic effect, followed by CLS and RS.

Surgical Management of Vesicoureteral Junction Obstruction in Children: A Comparative Study Between Transvesicoscopic Cohen Reimplantation and Transumbilical Laparoendoscopic Single-Site Lich-Gregoir Techniques.

Background: There are many reports on the application of minimally invasive technology in correction of children's vesicoureteral junction obstruction (VUJO), but there is no report on the treatment of children's VUJO with the transumbilical laparoendoscopic single-site surgery (TU-LESS) Lich-Gregoir method. We aimed to comparatively analyze the therapeutic outcomes of transvesicoscopic ureteral reimplantation Cohen (TUR-C) procedure and TU-LESS Lich-Gregoir (TU-LESS-LG) procedure in pediatric VUJO. Materials and Methods: The data of 49 children with VUJO, admitted from January 2016 to January 2020, were retrospectively analyzed. Based on different surgical methods, they were divided into the TUR-C group (23 cases) and the TU-LESS-LG group (26 cases). Demographic characteristics, perioperative characteristics, postoperative complications, recovery of renal function, and improvement of hydronephrosis were compared between the two groups. Results: There were no statistical differences in demographic characteristics and preoperative data between the two groups. The TU-LESS-LG group was superior to the TUR-C group in terms of average operation time and postoperative hospital stay. There was no statistical difference between the two groups in terms of postoperative complications, postoperative recovery of renal function, and improvement of hydronephrosis. Conclusions: The two surgical methods can achieve a similar curative effect in the treatment of VUJO. The TU-LESS-LG procedure has more advantages of operation time, postoperative hospital stay, wider age range for selection of cases, megaureter tapering, and cosmetic incision, but the operation is more difficult. Clinical Trial Registration number: 2021(KY-E-048).

Single-incision laparoscopic splenectomy in children with massive splenomegaly: A prospective, monocentric pilot study.

Background: Single-incision laparoscopic splenectomy (SILS) remains a challenging procedure because of the technical difficulty. In this prospective study, we aimed to evaluate the efficacy and safety of SILS in children with massive splenomegaly. Methods: Pediatric patients with massive splenomegaly were recruited for SILS in a university-affiliated hospital. The data on patient demographics, clinical features, operative variables, and perioperative outcomes were collected prospectively and analyzed. According to the different surgical instruments, the patients were randomly assigned into two groups: the SILS with straight surgical instrument (SILS-S) group and the SILS with curved surgical instrument (SILS-C) group. A two-group comparative analysis was conducted using perioperative data from the different surgical instrumentation systems. Results: A total of 120 patients were included, of which 103 patients (success group, 85.83%) had complete SILS, the other 17 (failure group, 14.17%) patients were converted to open (n = 4, 3.33%) or multi-incision laparoscopic surgery (n = 13, 10.83%). The major cause for surgical failure is uncontrollable bleeding (n = 14, 82.35%), and age, height, and weight were the risk factors for failure of SILS, but none of the parameters were independent risk factors. The blood loss in the success group was less than that in the failure group, but no significant differences in other operative and outcome indicators. For SILS, the mean (±SD) operative time was 188 (±48.70) minutes, the median intraoperative blood loss (min, max) was 20 (5, 290) ml, the mean (±SD) time of first anal exhaust was 23.9 (±7.73) hours, and the mean (±SD) postoperative hospital stay was 4.72 (±1.03) days. The median pain score was 3 on 1 day, and 1 on 3 days after the operation. Postoperative complications were identified in 8 (7.77%) cases. However, there were no peri-operative deaths in this series. The SILS-C group had a significantly shorter operation time than the SILS-S group (mean ± SD, 172 ± 44.21 vs. 205 ± 47.90 min). There were no significant differences between the two groups in other perioperative data (P < 0.05). Conclusion: SILS is a safe and feasible treatment in pediatric patients with massive splenomegaly, and curved surgical instrumentation has contributed to developing surgical manipulation.

The Expression and Potential Role of Tubulin Alpha 1b in Wilms' Tumor.

We explored the difference in expression of tubulin alpha 1b (TUBA1B) between Wilms' tumor (WT) and normal tissues (NT) from in-house patients and databases, to determine TUBA1B expression in WT and the predictive pathways of coexpressed genes. In-house RNA-sequencing data were performed with WT and NT from three patients from our institute. Other four RNA-sequencing and microarray data were also downloaded from multiple public databases. The TUBA1B expression between WT and NT was analyzed by Student's t-test and meta-analysis. The correlation between the expression of TUBA1B and other genes in each study was analyzed. Genes with p < 0.05 and r > 0.5 were considered as the coexpressing genes of TUBA1B. Overlapping the coexpressed genes of the five studies, including three in-house patients (3 WT vs. 3 NT), GTEx-TARGET (126 WT vs. 51 NT), GSE2172 (18 WT vs. 3 NT), GSE11024 (27 WT vs. 12 NT), and GSE73209 (32 WT vs. 6 NT), were performed with limma and VennDiagram packages in R software. The website of WEB-based GEne SeT AnaLysis toolkit were used to analyze the gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) functional annotations for the overlapped genes. The results showed that the relative expression of TUBA1B in WT tissues from in-house three patients was 280.0086, 141.7589, and 303.8292 and that in NT was 16.5836, 104.8141, and 12.79 (3 WT vs. 3 NT, p = 0.0285, ROC = 100%, SMD = 2.74). Student's t-test and meta-analysis in all studies revealed that the expression of TUBA1B was upregulated in WT tissues compared to that in NT (p < 0.05, SMD = 2.89, sROC = 0.98). Finally, the research identified the expression of TUBA1B in WT tissues was significantly upregulated than that in NT. The coexpressed genes of TUBA1B were enriched in the pathway of DNA replication, mismatch repair, cell cycle, pathogenic Escherichia coli infection, and spliceosome.

Nomogram for predicting overall survival in children with neuroblastoma based on SEER database.

PURPOSE: This study was performed to establish and validate a nomogram for predicting the overall survival in children with neuroblastoma. METHODS: The latest clinical data of neuroblastoma in Surveillance, Epidemiology, and End Results (SEER) database was extracted from 2000 to 2016. The cases included were randomly divided into training and validation cohorts. The survival curves were drawn with a Kaplan-Meier estimator to investigate the influences of certain single factors on overall survival. Also, least absolute shrinkage and selection operator regression was applied to further select the prognostic variables for neuroblastoma. Additionally, receiver operating characteristic (ROC) curves and calibration curves were used to evaluate the accuracy of the nomogram. RESULTS: In total, 1,262 patients were collected and 8 independent prognostic factors were achieved, including patients' age, sex, race, tumor grade, radiotherapy, chemotherapy, tumor site, and tumor size. Then we constructed a nomogram by using the data of the training cohort with 886 cases. Subsequently, the nomogram was validated internally and externally with 886 and 376 cases, respectively. The internal validation revealed that the area under the curves (AUC) of ROC curves of 1-, 3-, and 5-year overall survival were 0.69, 0.78, and 0.81, respectively. Accordingly, the external validation also showed that the AUC of 1-, 3-, and 5-year overall survival were all ≥0.69. Both methods of validation demonstrated that the predictive calibration curves were consistent with standard curves. CONCLUSION: The nomogram possess the potential to be a new tool in predicting the survival rate of neuroblastoma patients.

RNA-Sequencing, Connectivity Mapping, and Molecular Docking to Investigate Ligand-Protein Binding for Potential Drug Candidates for the Treatment of Wilms Tumor.

BACKGROUND Wilms tumor, or nephroblastoma, is a malignant pediatric embryonal renal tumor that has a poor prognosis. This study aimed to use bioinformatics data, RNA-sequencing, connectivity mapping, molecular docking, and ligand-protein binding to identify potential targets for drug therapy in Wilms tumor. MATERIAL AND METHODS Wilms tumor and non-tumor samples were obtained from high throughput gene expression databases, and differentially expressed genes (DEGs) were analyzed using the voom method in the limma package. The overlapping DEGs were obtained from the intersecting drug target genes using the Connectivity Map (CMap) database, and systemsDock was used for molecular docking. Gene databases were searched for gene expression profiles for complementary analysis, analysis of clinical significance, and prognosis analysis to refine the study. RESULTS From 177 cases of Wilms tumor, there were 648 upregulated genes and 342 down-regulated genes. Gene Ontology (GO) enrichment analysis showed that the identified DEGs that affected the cell cycle. After obtaining 21 candidate drugs, there were seven overlapping genes with 75 drug target genes and DEGs. Molecular docking results showed that relatively high scores were obtained when retinoic acid and the cyclin-dependent kinase inhibitor, alsterpaullone, were docked to the overlapping genes. There were significant standardized mean differences for three overlapping genes, CDK2, MAP4K4, and CRABP2. However, four upregulated overlapping genes, CDK2, MAP4K4, CRABP2, and SIRT1 had no prognostic significance. CONCLUSIONS RNA-sequencing, connectivity mapping, and molecular docking to investigate ligand-protein binding identified retinoic acid and alsterpaullone as potential drug candidates for the treatment of Wilms tumor.

A novel risk signature that combines 10 long noncoding RNAs to predict neuroblastoma prognosis.

Neuroblastoma (NBL) is the most frequently encountered extracranial solid neoplasm and impacts significantly on the survival of patients, especially in cases of advanced tumor stage or relapse. A long noncoding RNA (lncRNA) signature to predict the survival of patients with NBL is proposed in this paper. Differentially expressed lncRNA (DElncRNA) was selected using the Limma plus Voom package in R based on the RNA-sequencing data downloaded from the Therapeutically Applicable Research To Generate Effective Treatments database and Genotype-Tissue Expression database. Univariate cox regression analysis, least absolute shrinkage and selection operator regression analysis, and multivariate cox regression analysis were conducted to identify candidate DElncRNAs for the risk signature. Consequently, 10 DElncRNAs were designated as candidate DElncRNAs for the risk signature. Time-dependent receiver operating characteristic curves and Kapan-Meier survival curves confirmed the efficacy of the risk signature in predicting the survival of patients with NBL (area under the curve = 0.941; p ≤ .001). One of the DElncRNA constituent subparts (LINC01010) was significantly associated with the survival outcome of patients with NBL in GSE62564 (p = .004). Thus, a risk signature comprising 10 DElncRNAs was identified as effective for individual risk stratification and the survival prediction outcomes of patients with NBL.

Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature.

BACKGROUND: Primitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely rare anatomic locations, the abdominal wall and vulva. CASE SUMMARY: Case 1 was a 66-month-old girl with lesions on the abdominal wall; tumor size was about 3.4 cm × 6.1 cm × 2 cm. The patient underwent radical resection of the tumor. After the operation, an alternating vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide (IE) regimen was given for eight cycles, and the patient survived for 66 mo without progression. Case 2 was a 40-month-old girl, with a vulvar lesion; tumor size was about 3.3 cm × 5 cm × 2.5 cm. The tumor was partially resected by surgery. The family left treatment after two cycles of vincristine, pirarubicin, and cyclophosphamide/IE chemotherapy, and the patient died at home six months after surgery. CONCLUSION: PNET is a rare, fast-growing, highly malignant tumor that requires histologic and molecular analyses for exact diagnosis, and multimodal treatment is required to achieve a good prognosis.

Identification of a 3-mRNA signature as a novel potential prognostic biomarker in patients with ovarian serous cystadenocarcinoma in G2 and G3.

The use of mRNAs as biomarkers serves to diagnose, treat, as well as aid the prognosis of cancer. The present study involved an analysis of mRNAs in the cell cycle at the G2 and G3 tumor grades for the prognosis of ovarian serous cystadenocarcinoma (OSC) using 364 clinical samples (G2:G3=42:322). Statistics aided the identification of NPFFR2, XPNPEP2 and CELA3B; the 3-mRNA model that allows for classification of patients into high- and low-risk groups using a median value of 0.9580745. The rates of survival varied (P=0.00149) and the independent detection of stratification of the risk of this disease was validated with success using the 3-mRNA signature, which was demonstrated to be more successful than the weight model. This approach was revealed to provide the prognosis of grade G2 and G3 in patients with OSC compared with factors used traditionally. Compared with traditional factors, this 3-mRNA model was demonstrated to be the only and independent prognostic factor for patients with G2 and G3 stage OSC. A literature survey was also performed in the present study in order to assess the role of the 3 genes and indirectly prove their effectiveness. The establishment of this new genetic model will enhance prospective prognosis and treatment for patients with OSC.

Effects of modified penoplasty for concealed penis in children.

PURPOSE: To evaluate the effect of modified penoplasty in the management of concealed penis. METHODS: We retrospectively reviewed 96 consecutive patients with concealed penis, which had been surgically corrected between July 2013 and July 2015. All patients underwent modified Shiraki phalloplasty. All patients were scheduled for regular follow-up at 1, 3, and 6 months after the surgery. Data on the patients' age, operative time, postoperative complications, and parents' satisfaction grade were collected and analyzed. RESULTS: The mean follow-up period was 17.4 months (range 7-31 months). The mean operative time was 63.2 ± 8.7 min. The mean perpendicular penile length was 1.89 ± 0.77 cm preoperatively and 4.42 ± 0.87 cm postoperatively, with an improved mean length of 2.5 ± 0.68 cm in the flaccid state postoperatively (p < 0.05). The patients' satisfaction grades after the surgery were improved significantly (p < 0.05). Fifty-two patients had penile lymphedema postoperatively; however, it disappeared spontaneously within 3 months. Additionally, postoperative wound infection occurred in two patients. There were no complications such as flap necrosis, penile shaft contracture, voiding difficulty, and erection difficulties. CONCLUSION: The modified Shiraki phalloplasty for concealed penis can achieve maximum utilization of prepuce to assure coverage of the exposed penile shaft. It has fewer complications, achieving marked asthetics, and functional improvement. It is a relatively ideal means for treating concealed penis.

[Modified Mathieu urethroplasty for failed hypospadias repair].

OBJECTIVE: To review the indications, techniques and complications of reoperation for failed hypospadias repair using modified Mathieu urethroplasty. METHODS: Using modified Mathieu urethroplasty, we treated 24 hypospadias patients aged 3-12 (mean 4.5) years for whom the first (n = 20) or the second hypospadias repair (n = 4) had failed, including 13 cases of large coronary sulcus urethrocutaneous fistula, 5 cases of urethral meatus retraction and 6 cases of anterior urethra dehiscence. The modified procedure involved median longitudinal incision of the urethral posterior wall, dorsal tunica albuginea plication under the vascular and nerve bundle, and double dartos flap protection of the neourethra. RESULTS: Of the 24 patients, 19 (79.2%) were successfully treated by the first operation, which achieved desirable straightness, good cosmetic appearance and normal urethral meatus of the penis, without postoperative complications. Small urethrocutaneous fistula developed in 4 cases, of which 3 were cured by fistula repair and 1 self-healed. Urethral meatus stenosis occurred in 1 case, which was restored by meatal dilation. Urethrocele and urethrostenosis were not found in any of the cases. Two cases received urethroscopy postoperatively, which revealed no hypertrophic cicatrix at the site of median longitudinal incision in the urethral posterior wall. CONCLUSION: Modified Mathieu urethroplasty can be applied to hypospadias reoperation, particularly in such cases as large coronary sulcus urethrocutaneous fistula, urethral meatus retraction and anterior urethra dehiscence. The modified procedure includes median longitudinal incision of the urethral posterior wall, dorsal tunica albuginea plication under the vascular and nerve bundle, and double dartos flap protection of the neourethra.

[Treatment and outcome of 14 children with hepatoblastoma].

OBJECTIVE: To assess the outcome of childhood hepatoblastoma after a combination therapy of resection and chemotherapy. METHODS: The clinical data of 14 children with hepatoblastoma was retrospectively reviewed. Their long-term survival was followed-up. RESULTS: Twelve cases received surgery and planned chemotherapy. The follow-up duration averaged 18 months (range 1.5-74 months). Nine survived free of events, 1 died, 1 survived with multiple lung metastases, and 1 with increased alpha-fetoprotein (AFP) content but without residual tumor. CONCLUSIONS: Surgery assisted with chemotherapy can improve the outcome of hepatoblastoma.