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The aim of this study was to elucidate the prevalence of urachal remnants in children in relation to patient age as well as to identify their anatomic variants, using a laparoscopic view.The medical records of 394 pediatric patients who underwent laparoscopic inguinal hernia repair were reviewed. Patients were divided into four groups based on their age at surgery. Using laparoscopic visualization, the presence and anatomic variants of urachal remnants were analyzed.A urachal remnant was confirmed in 140 children (35.5%). Although the prevalence was significantly higher in the group of children aged 1 year (63.2%) than in any other group, no significant difference in the prevalence was observed between the groups aged ≥ 1 year. In 42 cases (10.7%), the urachal remnant merged into the lateral umbilical ligament.Our results suggest a recommendation of nonoperative management of asymptomatic urachal remnants, especially in patients less than 1 year of age due to its probable spontaneous resolution. Knowledge of the anatomic variants could improve the accuracy of diagnosis of urachal remnants and the comprehension of its structure and localization for the achievement of accurate and complete excision.

Introduction: This study aimed to assess the preventive or therapeutic effects of blenderized food (BF) on selenium deficiency in pediatric patients with severe motor and intellectual disabilities (SMID). Methods: The medical records of all 40 consecutive pediatric patients with SMID who underwent nutritional assessment were retrospectively reviewed and compared between two groups: the enteral formula (EF) group and the BF group fed with BF providing more than 10% of total caloric intake. Next, for the selenium-deficient patients who were newly started on blenderized tube feeds after the first nutritional assessment, improvement of selenium deficiency and change of dietary contents were assessed. Results: The BF group patients had a significantly lower prevalence of selenium deficiency and higher serum selenium levels than the EF group patients. In all 7 selenium-deficient patients who started blenderized tube feeds after the first nutritional assessment, serum selenium levels were significantly increased at the second nutritional assessment, even though total selenium intake, selenium intake by EF, and total caloric intake did not differ significantly, and, in fact, caloric intake was significantly decreased by EF. Conclusion: Combined feeding of BFs can be useful for prevention and therapy of selenium deficiency in pediatric SMID patients.

MicroRNAs (miRNAs) play an important role in regulating fibrogenesis in the liver. The current study examined the ability of microRNA-214 (miR-214) level in liver and serum samples obtained from patients with BA to predict progressive liver fibrosis in patients with biliary atresia (BA). We examined miR-214 level in relation to conventional markers of liver fibrosis, with liver and serum samples from BA patients. Fifty-two patients with BA who underwent Kasai portoenterostomy and four control patients underwent liver biopsy. In 28 patients with BA, blood samples were collected to analyze circulating serum miR-214. MiR-214 levels in liver tissue were significantly upregulated in patients with BA who had severe liver fibrosis (F3–4) compared to those with none to mild fibrosis (F0–2), whereas suppressors-of-fused homolog (Sufu) mRNA levels were significantly suppressed in F3–4. Serum miR-214 levels were significantly higher in patients with F3–4 compared with F0–2. Area under the curve analysis showed that the serum miR-214 cut-off level for predicting F3–4 was 0.805 (p = 0.0046). Hepatic overexpression of miR-214 is associated with progression of liver fibrosis in patients with BA, and the circulating miR-214 level may serve as a non-invasive predictor of liver fibrosis.

In 2004, the Japanese government halted nationwide mass screening for neuroblastoma in 6-month-old infants as it led to overdiagnosis of localized tumors with favorable prognoses and failed to reduce neuroblastoma-related mortality. However, a new mass screening program for neuroblastoma in 18-month-old infants (18MS) was conducted in the Osaka prefecture. We assessed the efficacy of the 18MS in screening unfavorable cases. Public health centers in Osaka prefecture, excluding the Osaka city area, provided test kits to the guardians of infants who received a check-up at 18 months of age between 2004 and 2017. For patients whose standardized urinary levels of vanillylmandelic acid or homovanillic acid exceeded the threshold, they were further examined and treated in two specific hospitals Osaka University Hospital and Osaka Women’s and Children’s Hospital. Screening-positive patients with and without neuroblastoma were retrospectively reviewed. Among 142,423 children screened during the 18MS, 85 tested positive, and 14 were diagnosed with neuroblastoma. Twelve patients were classified as very low risk, while 2 were classified as high risk, based on the International Neuroblastoma Risk Group risk classification. The 18MS did not screen unfavorable cases with neuroblastoma efficiently, although few participants benefited from it.

Minimally invasive surgery (MIS) is appropriate for the treatment of some neuroblastomas (NBs); however, the indications and technical issues are unclear. This study aimed to clarify the current status of MIS for mediastinal NB in Japan. Preliminary questionnaires requesting the numbers of neuroblastoma cases in which MIS was performed from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. Secondary questionnaires were sent to institutions with MIS cases to collect detailed data. One hundred thirty-four (84.2%) institutions returned the preliminary questionnaire and 83 institutions (52.2%) reported a total of 1496 operative cases. MIS was performed for 175 (11.6%) cases. Among the 175 cases, completed forms of 140 patients were returned and 40 (male, n = 28; female, n = 12) cases had mediastinal NB. Fourteen patients received thoracoscopic biopsy, none were converted to thoracotomy. Twenty-eight patients received MIS for radical resection, none were converted to thoracotomy. Perioperative complications (Horner’s syndrome) were recognized after radical resection in one (2.5%) case. MIS was performed in a limited number of mediastinal NB cases. A thoracoscopic approach would be feasible for mediastinal NB.

Introduction: Laparoscopic resection has gradually been adopted for neuroblastoma patients; however, some authors reported that, due to its technically demanding procedures, this operation should be performed only by highly experienced surgeons. The aim of this study was to evaluate the safety and feasibility of laparoscopic resection of abdominal neuroblastoma by pediatric surgical trainees. Subjects and Methods: In this multicenter, retrospective study, including 18 children with abdominal neuroblastoma indicated for 19 laparoscopic resections (1 with bilateral neuroblastomas) from 1999 to 2018, the clinical data were retrospectively reviewed and compared between trainee and attending surgeons. Results: None of the cases had image-defined risk factors (IDRFs) at surgery. All patients successfully underwent complete laparoscopic resection without blood transfusion, open conversion, or intraoperative or postoperative complications. Of the 19 cases, 6 were performed by pediatric surgical trainees under the guidance of attending surgeons, and 13 were performed by attending surgeons. With comparable tumor, largest diameter, operative time, and bleeding amount were not significantly different between the two groups. In the trainee surgeon group, one local recurrence occurred at a secondary resection site in the bilateral neuroblastoma patient with Stage L2. Conclusions: Laparoscopic resection of neuroblastoma could be safe and feasible when limited to absent IDRFs at surgery by pediatric surgical trainees under the guidance of experienced attending surgeons, as well as by attending surgeons.

Some neuroblastoma (NB) cases are suitable for minimally invasive surgery (MIS), but indication and technical issue are unclear. We assessed the current status of MIS for abdominal NB after mass screening period in Japan. Preliminary questionnaires requesting the numbers of NB cases that underwent MIS from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. The secondary questionnaires were then sent to the institutions that reported MIS cases of NB in order to collect detailed data. One hundred and thirty-four (84.2%) institutions responded to the preliminary questionnaires, and 83 (52.2%) reported managing operative cases. The total number of operative cases was 1496. MIS was performed for 175 (11.6%) cases, of which the completed forms of 140 patients were returned, including 100 abdominal NB cases. The male/female ratio was 51/49. Forty-seven cases underwent a laparoscopic biopsy, and 2 (4.3%) cases were converted to laparotomy due to bleeding. Sixty-five cases underwent MIS for radical resection, and 7 (10.8%) were converted to laparotomy. The reasons for open conversion were bleeding and severe adhesion. Regarding open conversion, there were no significant relationships between conversion and neo-adjuvant chemotherapy, biopsies, stage, size, or MYCN amplification. We found no relationship between resectability and vascular encasement in this study. There was relationship between the resected tumor size and the patients’ height, which was expressed using the following formula: $$y = 0.0316x + 1.4812$$ (x, patients height, y, tumor size; p = 0.004219, SE: 1.55566). Postoperative complications after radical resection were recognized in 7 (10.8%) cases. MIS was performed in limited cases of abdominal NB. A laparoscopic biopsy with careful attention to bleeding is feasible. The resected tumor size was shown to correlate with the patients’ height. Tumor size within 6 cm of maximum diameter can be resected safely.

SMARCB1 is mutated in most rhabdoid tumors (RTs) developing in the kidney (RTK) and various other organs. Focal deletions found in patients with 22q11.2 deletion syndrome show breakpoints within clusters of segmental duplications (SDs), and those in some RTs show breakpoints in the 22q11-q12 region. SDs are known to cause focal deletion mediated by non-allelic homologous recombination. The present study identified SMARCB1 alterations in all 30 RTKs, using SNP array CGH, MLPA, and sequence analyses. Twenty-eight tumors had a total of 51 breakpoints forming focal 22q deletion and/or uniparental disomy (22qUPD), and the other two had compound mutation with no breakpoints in 22q. Twenty-four (47.1%) of the 51 breakpoints were within SDs, and occurred in 16 (53.3%) of the 30 tumors. The association of breakpoints with SDs was found not only in focal deletion, but also in 22qUPD, indicating that SDs mediate the first and second hits (focal deletion) and the second hit (22qUPD) of SMARCB1 alteration. Of the 51 breakpoints, 14 were recurrent, and 10 of the 14 were within SDs, suggesting the presence of hotspots in the 22q11.2 region. One recurrent breakpoint outside SDs resided in SMARCB1, suggesting inactivation of the gene by out-of-frame fusion. The association between SDs and focal deletion has been reported in two other types of cancer. RTKs may be the third example of SD-associated tumors. Thus, the present study indicated that RTKs exploit genomic instability in the 22q11.1-11.2 SDs region, and 22qUPD caused by mitotic recombination may also be mediated by SDs. This article is protected by copyright. All rights reserved.

Purpose The aim of this study was to elucidate the incidence of urachal remnants in children in relation to patient age as well as to identify their anatomic variants, using a laparoscopic view.Methods The medical records of 394 pediatric patients who underwent laparoscopic inguinal hernia repair were reviewed. Patients were divided into four groups based on their age at surgery. Using laparoscopic visualization, the presence and anatomic variants of urachal remnants were analyzed.Results A urachal remnant was confirmed in 140 children (35.5%). Although the incidence was significantly higher in the group of children aged < 1 year (63.2%) than in any other group, no significant difference in the incidence was observed between the groups aged ≥ 1 year. In 42 cases (10.7%), the urachal remnant merged into the lateral umbilical ligament.Conclusions Our results suggest a recommendation of nonoperative management of asymptomatic urachal remnants, especially in patients less than 1 year of age due to its probable spontaneous resolution. Knowledge of the anatomic variants could improve the accuracy of diagnosis of urachal remnants and the comprehension of its structure and localization for the achievement of accurate and complete excision.

A high-frequency radio wave (HFRW) scalpel has been reported to achieve a fine smooth incision with good hemostasis and minimizes the surrounding tissue destruction. Since an umbilicoplasty was performed for cosmetic purposes, we introduced the HFRW scalpel for umbilical hernia repair in July 2016. This retrospective study aimed to investigate the safety and efficacy of an HFRW scalpel for umbilical hernia repair in children, compared to a surgical scalpel with electrocautery. We reviewed the medical records of 139 patients who underwent umbilical hernia repair using a surgical scalpel with electrocautery or an HFRW scalpel between January 2013 and November 2018. Patients were divided into two groups: the surgical scalpel with electrocautery (SE) group (n = 62) and the HFRW scalpel (HFRW) group (n = 77), for comparison of surgical outcomes. Umbilical hernia repair using the HFRW scalpel was successful without intraoperative complications in all 77 patients. Although the HFRW scalpel was directly used for skin incision, no burn injury was observed. Procedure duration was significantly shorter in the HFRW group than the SE group (P

INTRODUCTION Laparoscopy-assisted percutaneous endoscopic gastrostomy (LAPEG) can reduce the risk of percutaneous endoscopic gastrostomy-related complications, such as intra-abdominal organ injury, and determine the optimal position for placement of the gastrostomy tube. We first employed LAPEG 10 years ago but limited its application to elderly patients. Indications for LAPEG have now expanded to small children. This retrospective study aimed to determine the feasibility of LAPEG in children weighing

MicroRNAs (miRNAs) play an important role in regulating fibrogenesis in the liver. The current study examined the ability of microRNA-214 (miR-214) level in liver and serum samples obtained from patients with BA to predict progressive liver fibrosis in patients with biliary atresia (BA).We examined miR-214 level in relation to conventional markers of liver fibrosis, with liver and serum samples from BA patients. Fifty-two patients with BA who underwent Kasai portoenterostomy and four control patients underwent liver biopsy. In 28 patients with BA, blood samples were collected to analyze circulating serum miR-214.MiR-214 levels in liver tissue were significantly upregulated in patients with BA who had severe liver fibrosis (F3-4) compared to those with none to mild fibrosis (F0-2), whereas suppressors-of-fused homolog (Sufu) mRNA levels were significantly suppressed in F3-4. Serum miR-214 levels were significantly higher in patients with F3-4 compared with F0-2. Area under the curve analysis showed that the serum miR-214 cut-off level for predicting F3-4 was 0.805 (p = 0.0046).Hepatic overexpression of miR-214 is associated with progression of liver fibrosis in patients with BA, and the circulating miR-214 level may serve as a non-invasive predictor of liver fibrosis.

Since previous consensus-based Wilms tumour (WT) surveillance guidelines were published, novel genes and syndromes associated with WT risk have been identified, and diagnostic molecular tests for previously known syndromes have improved. In view of this, the International Society of Pediatric Oncology (SIOP)-Europe Host Genome Working Group and SIOP Renal Tumour Study Group hereby present updated WT surveillance guidelines after an extensive literature review and international consensus meetings. These guidelines are for use by clinical geneticists, pediatricians, pediatric oncologists and radiologists involved in the care of children at risk of WT. Additionally, we emphasise the need to register all patients with a cancer predisposition syndrome in national or international databases, to enable the development of better tumour risk estimates and tumour surveillance programs in the future.

Treatment is more intensive for stage III Wilms tumor (WT) than for stages I and II non-metastatic WTs. Various factors including tumor spillage, unresectability, and lymph node metastasis are responsible for stage III disease. The present study aimed to not identify clinical factors associated with the features of stage III WT to establish new treatment strategies. Of 166 patients with non-metastatic WT enrolled in the Japan Wilms Tumor Study (JWiTS)-2, 51 patients had stage III disease. The treatment protocol for JWiTS-2 was essentially the same as that in the National Wilms Tumor study (NWTS)-5. Local hospitals were surveyed to collect details of clinical findings related to stage III disease, and data regarding 45 (88%) patients were obtained. Nine patients with massive tumors underwent preoperative chemotherapy. Biopsy was performed in 6. Reduction in the tumor size was achieved in 8 of the 9 cases. Nephrectomy was finally performed in all of them. Thirty-six patients underwent primary nephrectomy. The reason for the stage III disease was lymph node metastasis (n = 9, 25%), tumor spillage (n = 20, 56%), and tumor extension/incomplete resection (n = 17, 47%). Some patients had more than one of these factors. Most patients were treated with the DD-4A regimen, and 43 (95.6%) of the 45 patients received abdominal radiation therapy. Tumors recurred in three patients (local, 1; metastasis, 2), and two patients died. Overall and relapse-free survival rates were 95.2% and 90.8%, respectively. The prognosis of stage III WT was good. In the next stage, the doses of chemotherapy and radiotherapy should be reduced to avoid late effects. The high rate of tumor spillage after primary resection suggests that preoperative chemotherapy should be started instead of aggressive tumor resection in the large tumor cases with surgical risks

Ghrelin was recently reported to promote recovery from hepatic injury. We hypothesized that it could also be associated with clinical recovery of the transplanted liver from ischemia and reperfusion injury. Our aims were to investigate perioperative ghrelin changes following pediatric living donor liver transplantation (LDLT) and to analyze the association of these changes with postoperative hepatic function. We measured plasma acyl ghrelin (AG) concentrations before surgery, at the end of surgery and on postoperative days (PODs) 1, 3 and 7 in 12 children who underwent LDLTs, and, as controls, pre- and post-operatively and on POD1 in 7 children who underwent benign abdominal mass resection. The correlations between the participants’ ghrelin profiles and hepatic function-related data were evaluated. AG levels significantly declined to 15.6% of preoperative levels after LDLT and almost returned to baseline on POD3. Post-operative AG levels were significantly reduced to a greater extent following LDLT than benign abdominal mass resection. AG levels on POD1 inversely correlated with aspartate aminotransferase levels and cold/total ischemia time (P

To identify prognostic factors, array CGH (aCGH) patterns and mutations in WT1 and 9 other genes were analyzed in 128 unilateral Wilms tumors (WTs). Twenty patients had no aCGH aberrations, and 31 had WT1 alterations [silent and WT1 types: relapse-free survival (RFS), 95% and 83%, respectively]. Seventy-seven patients had aCGH changes without WT1 alterations (nonsilent/non-WT1 type) and were subtyped into those with or without +12, 11q−, 16q−, or HACE1 loss. RFS was better for those with than those without +12 (P = .010) and worse for those with than those without 11q−, 16q−, or HACE1 loss (P = .001, .025, or 1.2E-04, respectively). Silent and WT1 type and 8 subtype tumors were integrated and classified into 3 risk groups: low risk for the silent type and +12 subgroup; high risk for the no +12 plus 11q−, 16q−, or HACE1 loss subgroup; intermediate risk for the WT1 type and no +12 plus no 11q−, 16q−, or HACE1 loss subgroup. Among the 27 WTs examined, the expression of 146 genes on chromosome 12 was stronger in +12 tumors than in no +12 tumors, while that of 10 genes on 16q was weaker in 16q− tumors than in no 16q− tumors. Overexpression in 75 out of 146 upregulated genes and underexpression in 7 out of 10 downregulated genes correlated with better and worse overall survival, respectively, based on the public database. +12 was identified as a potential new marker predicting a favorable outcome, and chromosome abnormalities may be related to altered gene expression associated with these abnormalities.

BACKGROUND Wilms tumor (WT) demonstrates epidemiological differences by world region and ethnicity. To enhance understanding of these differences, we retrospectively analyzed clinical trial data sets from the UK and Japan over a 20-year period. PROCEDURE We used data from three consecutive clinical trials in the UK and a single study in Japan that enrolled patients diagnosed during 1996-2015, to compare clinical characteristics and outcomes between countries. RESULTS During 1996-2015, 1395 patients in the UK and 537 in Japan were included. Japanese patients have a significantly younger median age at diagnosis than those in the UK (28 months vs 39 months). The proportion of patients with stage IV, large tumors, and anaplastic histology appears to be higher in the UK than in Japan (18% vs 11%, 62% vs 49%, 8% vs 3%, respectively). During 2005-2015, 77 hospitals treated WT in Japan compared with only 20 hospitals in the UK. Five-year overall survival of patients with WT was over 90% in both countries, but five-year event-free survival of patients with stage IV was significantly lower in Japan than in the UK (50.0% vs 76.2%, P = 0.001). CONCLUSIONS Differences in age of onset, tumor size at diagnosis, and histology may reflect differences in the genetic background of patients with WT between countries, but population-based phenotype-genotype data are lacking. The difference in survival probability for stage IV patients may be due to different diagnostic criteria or different treatment strategies. Prospective, international clinical studies including genomic analyses are needed to confirm these findings and improve clinical practice.

Background/Purpose We examined the effects and mechanisms of rikkunshito (RKT) and hangeshashinto (HST) on cisplatin-induced mucosal injuries in the rat small bowel. Methods Juvenile rats were divided into 6 groups: sham control, cisplatin injection without kampo medicines, and cisplatin injection with oral administration of low and high doses of RKT (1000 mg/kg and 2000 mg/kg) and HST (500 mg/kg and 1000 mg/kg). Fecal condition, intestinal morphological changes, enterocyte proliferation, and enterocyte apoptosis were assessed. Results Diarrhea and atrophy of ileal villi observed in the cisplatin group were significantly improved in all kampo groups. Injury scores of the jejunum were significantly lower with RKT (2000 mg/kg) and HST (500 and 1000 mg/kg) than with cisplatin, and those of the ileum were significantly lower with HST (500 and 1000 mg/kg) than with cisplatin. Enterocyte proliferation of the jejunum was significantly increased with RKT (2000 mg/kg) and HST (500 mg/kg) compared with cisplatin, and those of the ileum were significantly increased in all kampo groups compared with the cisplatin group. Jejunal and ileal apoptosis following cisplatin administration was significantly inhibited by HST. Conclusions RKT and HST prevented cisplatin-induced intestinal mucosal injury with increasing proliferation of intestinal epithelial cells. HST also attenuated cisplatin-induced crypt cell apoptosis.

Summary Background & aims Nutritional metabolism is complex in pediatric patients with severe motor and intellectual disability (SMID), and therefore, appropriate estimation of the energy requirements is difficult. Focusing on ghrelin's role in energy metabolism regulation, we investigated plasma ghrelin levels in pediatric SMID patients and analyzed its nutritional significance as a regulatory marker of energy reserve. Methods Fasting plasma total, acyl, and des-acyl ghrelin levels in 40 patients with SMID, including cerebral palsy (CP) (n = 20) and muscular disease (MD) (n = 8), and healthy controls (n = 13) were investigated. The correlations of plasma ghrelin levels with anthropometry, blood nutritional markers, energy intake, and resting energy expenditure (REE) measured with indirect calorimetry were analyzed. A p value Results SMID patients had significantly higher acyl ghrelin, and lower body mass index (BMI), z-scores of body weight (BW), body height and BMI, and albumin than controls. CP patients had significantly higher total and acyl ghrelin, z-score of the mid-upper arm circumference (MUAC), retinol-binding protein, transthyretin, creatinine, and glucose than MD patients. Total and acyl ghrelin in CP patients and des-acyl ghrelin in MD patients had significant negative correlations with MUAC and upper arm fat area. In CP patients, total and acyl ghrelin had significant positive correlations with REE/BW (kcal/kg), and total ghrelin was predictive of REE/BW (r2 = 0.625, p Conclusions An increase in acyl ghrelin observed in SMID patients possibly indicates energy reserve deficiency. In CP patients, total and acyl ghrelin inversely reflected total body fat mass, resulting in strongly positive correlations with REE/BW. The measurement of plasma ghrelin may be useful to assess nutritional metabolism and energy reserve in pediatric SMID patients, such as CP and MD patients.

PURPOSEWe report here the outcomes and late effects of the Japanese Study Group for Pediatric Liver Tumors (JPLT)-2 protocol, on the basis of cisplatin-tetrahydropyranyl-adriamycin (CITA) with risk stratification according to the pretreatment extent of disease (PRETEXT) classification for hepatoblastoma (HB).PATIENTS AND METHODSFrom 1999 to 2012, 361 patients with untreated HB were enrolled. PRETEXT I/II patients were treated with up-front resection, followed by low-dose CITA (stratum 1) or received low-dose CITA, followed by surgery and postoperative chemotherapy (stratum 2). In the remaining patients, after 2 cycles of CITA, responders received the CITA regimen before resection (stratum 3), and nonresponders were switched to ifosfamide, pirarubicin, etoposide, and carboplatin (ITEC; stratum 4). Intensified chemotherapeutic regimens with autologous hematopoietic stem-cell transplantation (SCT) after resection were an optional treatment for patients with refractory/metastatic disease.RESULTSThe 5-year event-free and overall survival rates of HB patients were 74.2% and 89.9%, respectively, for stratum 1, 84.8% and 90.8%%, respectively, for stratum 2, 71.6% and 85.9%%, respectively, for stratum 3, and 59.1% and 67.3%%, respectively, for stratum 4. The outcomes for CITA responders were significantly better than those for nonresponders, whose outcomes remained poor despite salvage therapy with a second-line ITEC regimen or SCT. The late effects, ototoxicity, cardiotoxicity, and delayed growth, occurred in 61, 18, and 47 patients, respectively. Thirteen secondary malignant neoplasms (SMNs), including 10 leukemia, occurred, correlating with higher exposure to pirarubicin and younger age at diagnosis.CONCLUSIONThe JPLT-2 protocol achieved up-front resectability in PRETEXT I/II patients with no annotation factors, and satisfactory survival in patients who were CITA responders in the remaining patients. However, outcomes for CITA nonresponders were unsatisfactory, despite therapy intensification with ITEC regimens and SCT. JPLT-2 had a relatively low incidence of cardiotoxicity but high rates of SMNs.

INTRODUCTION This study aimed to compare the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) and conventional open repair (OR) for inguinal hernia in infants younger than 6 months. METHODS The medical records of 202 patients who had undergone LPEC or OR at an age younger than 6 months between 2010 and 2016 were reviewed. Patient characteristics and surgical outcomes were compared between LPEC and OR. RESULTS In total, there were 120 LPEC (76 males, 44 females) and 83 OR (76 males, 7 females) cases. There were no differences in median age, median bodyweight, and presence of incarceration between the two groups (LPEC group: 3 months (range, 0-5 months), 5.7 kg (range, 2.3-9.6 kg), and 13.3%; OR group: 3 months (range, 1-5 months), 5.5 kg (range, 2.1-9.0 kg), and 12.0%). LPEC was completed in all cases without any intraoperative complications or open conversions. In the LPEC group, 60.0% of unilateral hernia patients had a contralateral patent processus vaginalis and underwent prophylactic closure. The incidence of metachronous contralateral hernia was significantly lower in the LPEC group than in the OR group (0% vs 10.0%, P

The standard treatment for Wilms tumor (WT) is primary resection. However, in cases with unresectable tumor or tumor spillage, which are considered to have high surgical risks, more intensive chemotherapy and radiotherapy are required. In the present study, we retrospectively analyzed preoperative image parameters to identify factors associated with surgical risks. Twenty-nine patients with WT were enrolled in this study. Data on various preoperative image parameters, such as tumor size, tumor volume, displacement of great vessels, and contralateral extension of the tumor were collected, and their relationship with surgical factors, including operative time, hemorrhage, tumor spillage, and unresectability were analyzed. Patients with unresectable tumor or with tumor spillage (surgical high-risk group) more frequently demonstrated displacement of great vessels and contralateral tumor extension. Operative time and blood loss were also significantly related to tumor size, area, volume, displacement of great vessels and contralateral extension. Besides tumor size, displacement of great vessels and contralateral extension were significantly associated with surgical risks. These factors are easily determined using CT images and are, therefore, useful to decide whether preoperative chemotherapy should be started instead of primary tumor resection for large localized WTs.

Background As no standard chemotherapy exists for pancreatoblastoma (PB), surgical resection is the most effective way of achieving complete remission. Case report A 3-year-old girl with PB causing portal vein obstruction was referred to our hospital. Because of the portal vein involvement, she initially received 5 cycles of preoperative chemotherapy after biopsy, consisting of cyclophosphamide, vincristine, pirarubicin, and cisplatin. After chemotherapy, she underwent distal pancreatectomy, and the tumor was resected completely. She has been disease free for 4 years since the operation. Conclusions Even in cases of advanced PB, complete surgical resection with appropriate chemotherapy can lead to complete remission.

Congenital esophageal stenosis (CES) is rare and usually manifests in infants as dysphagia, failure to thrive, and food impaction. Dilatation is considered to be the first-line therapy for CES, but the incidence of complications (perforation and mediastinitis) is relatively high. We report two cases of CES treated by thoracoscopic resection without prior dilatation. Both infants recovered without recurrent stenosis and were able to eat solid food soon after surgery. One had postoperative gastroesophageal reflux and eventually required fundoplication. Thoracoscopic resection could be a valid option for CES.

A mature teratoma of the pancreas has rarely been reported, and postoperative superior mesenteric artery syndrome (SMAS) is extremely rare in pediatric pancreatic surgery. A 12-year-old girl underwent an enucleation of the large mature teratoma located at the pancreas uncus. Although her postoperative recovery was fair, the bile-stained gastric juice continued draining at a rate of>1.5 l per day for 2 weeks. An upper gastrointestinal series revealed an abrupt disruption with to-and-fro peristalsis at the third portion of the duodenum. Endoscopy revealed an extrinsic pulsatile compression of this third portion. The aortomesenteric angle measured 12° on ultrasonography, which met the criteria for SMAS. The patient underwent a modified transposition procedure, “switching jejunojejunostomy,” on postoperative day 18. The gastrointestinal passage gradually improved, and complete oral intake was established 1 month after the first surgery. It was considered that the clinical symptoms of SMAS were caused by an anatomical deformation after the pancreatic tumor resection. Switching jejunojejunostomy was found to be an acceptable therapeutic option for this condition.

To evaluate the clinical features and treatment results of anaplastic histology (AH) Wilms’ tumor (WT) patients registered in the Japan Wilms’ Tumor Study (JWiTS) group to elucidate the clinical characteristics of AH in the Japanese population. Of 344 WT patients who were enrolled in JWiTS between 1995 and 2013, 17 had AH. Treatment using the JWiTS protocols was similar to the fifth National Wilms’ Tumor Study 5 (NWTS-5) protocols. Clinical characteristics and mutation status of TP53 gene were evaluated and compared with those in NWST-5 study. AH incidences in JWiTS were 4.9 %, lower than that in NWTS-5. Seven tumors had focal AH and 10 had diffuse AH. Clinical stages of AH patients were stage I in seven, stage II in three, stage III in five, stage IV in one and unknown in one. Four-year event-free survival and overall survival rates were 90.9 and 86.7 %, respectively. Two patients with diffuse AH and none with focal AH had TP53 mutation. Japanese patients presented with higher incidence, earlier stages and may have better outcomes than American patients, indicating a possible biological heterogeneity of AH WT. Further analysis is necessary to elucidate the different characteristic of AH WT between Japanese and American populations.

CT-guided localization followed by video-assisted thoracoscopic surgery was performed in three children with pulmonary nodules less than 5 mm in diameter. The patients' respective primary diagnoses were Wilms tumor, hepatoblastoma, and osteosarcoma of the femur. The pulmonary nodules were marked preoperatively by a percutaneously placed hook-wire or dye under CT guidance. Although none of the nodules was grossly detected during the operation, they were correctly resected under the guidance of the hook-wire wound or dye. A histological assessment revealed viable metastatic lesions in the case of hepatoblastoma, completely necrotic lesions in the case of Wilms tumor, and inflammatory nodules in the case of osteosarcoma. CT-guided localization followed by video-assisted thoracoscopic surgery appears to be a beneficial procedure in children with tiny pulmonary nodules.

Coincidental cyanotic congenital heart disease and pheochromocytoma is uncommon, although some cases have been reported. We describe a girl aged 15 yr and 11 mo with pheochromocytoma and tricuspid atresia treated by performing the Fontan surgery. The patient did not have any specific symptoms of syndrome related to pheochromoytoma or a family history of pheochromocytoma. During cardiac catheterization, her blood pressure increased markedly, and an α-blocker was administered. Catecholamine hypersecretion was observed in the blood and urine, and abdominal computed tomography revealed a tumor in the right adrenal gland. Scintigraphy showed marked accumulation of 123I-metaiodobenzylguanidine in the tumor, which led to a diagnosis of pheochromocytoma. We did not detect any germline mutations in the RET, VHL, SDHB, SDHD, TMEM127, or MAX genes. This patient had experienced mild systemic hypoxia since birth, which may have contributed to the development of pheochromocytoma.

We herein report the findings of 3 patients with primary Ewing sarcoma in a pelvic lesion who underwent the placement of a tissue expander (TE) before radiation therapy to prevent the adverse effects of radiotherapy. The simulation study showed that the TE drastically reduced volume of the intestine that was irradiated at all dose levels. All patients could receive the scheduled dose of radiotherapy without any acute and late complications such as diarrhea, melena, the dislodging of the TE, infection, or the formation of fistulae. In the 4-year (minimum) observation period, we did not observe intestinal complications in any of our patients. TE placement is considered to be a safe and effective method for preventing the adverse effects of radiotherapy in pediatric malignant pelvic tumors.

Background Persistence of blastemal components after chemotherapy is a marker of poor outcome in Wilms tumor (WT). Recent reports from local Japanese areas have described pre-chemotherapy blastemal predominant type WT to also be a risk factor for relapse. The significance, however, of blastemal predominant WT remains to be evaluated in a larger study. This study retrospectively evaluated the prognostic significance of pre-chemotherapy blastemal predominant type WT in the Japan Wilms tumor Study (JWiTS) trials. Methods The JWiTS trial (1996-2013) was a prospective, single-arm study. The outcomes of blastemal predominant type WT were retrospectively evaluated compared with non-blastemal type WT excluding anaplasia between 1996 and 2013. Relapse-free survival (RFS) and overall survival (OS) were estimated. Results Of 319 primary renal tumors diagnosed by the central pathology review system, advanced stage of pre-chemotherapy blastemal predominant type WT (n = 53; 16.1%) occurred more frequently in older children than non-blastemal type WT (n = 225), and was especially frequent in female patients registered in the JWiTS trials. No significant difference in 10 years RFS and OS (78.8% vs 84.5; P = 0.201) or in 10 years RFS and OS (89.3% vs 93.5; P = 0.45) was seen between pre-chemotherapy blastemal predominant type and non-blastemal type WT. Conclusions Relapse-free survival and OS are not significantly different between pre-chemotherapy blastemal predominant type and non-blastemal type WT.

We herein report the rare case of a 4-year-5-month-old boy who presented with primary renal neuroblastoma. The patient developed repeated lung and liver metastatic recurrences, but, following a combination of chemotherapy, radiation therapy and aggressive surgical resection, the patient is now in remission. To investigate the pathogenesis of lung metastasis, immunohistochemistry was performed for matrix metalloproteinase-9 and -14 (MMP-9 and MMP-14), molecular markers of invasion, metastasis and angiogenesis in neuroblastoma. In the present case, MMP-9 expression was not observed, but MMP-14 expression was detected in the primary lesion and was more highly expressed in the metastatic lesion compared with the primary one. Given the MMP-14 staining in other cases, expression of MMP-14 may be associated with the aggressiveness of the tumor. This suggests that selected clones with high MMP-14 expression in the primary tumor might metastasize and form MMP-14-rich lesions.

Necrotizing enterocolitis (NEC) is a devastating inflammatory disease of preterm infants that may depend on overexpression of toll-like receptor-4 (TLR4) in the immature intestine. Surfactant protein (SP)-D is a member of the collectin family and plays an important role in innate immunity, particularly in the airways. Although SP-D also exists in the intestines, little is known about its function. This study investigated whether SP-D can attenuate the inflammatory response of TLR4-overexpressing embryonal intestinal cells. All experimental procedures were performed using the human intestinal cell line INT407 originally derived from human embryonal intestines. Platelet-activating factor (PAF), reported to be elevated in NEC patients, was used to induce TLR4 overexpression in the human embryonal intestinal cell line INT407. TLR4 expression was measured using quantitative real-time PCR. Inflammatory responses to PAF (5 µM), the TLR4 agonist lipopolysaccharide (LPS, 100 ng/ml), PAF + LPS, and PAF + LPS following SP-D pretreatment (20 µg/ml) were assessed by enzyme-linked immunosorbent assay (ELISA) of interleukin-8 (IL-8) release (in pg/ml). Expression of TLR4 mRNA (mean ± SD) was upregulated by PAF (369 % ± 28 %, p

The Japan Wilms Tumor Study group (JWiTS) was founded in 1996 to improve outcomes for children with renal tumor in Japan, and a nationwide multicenter cooperative study was initiated thereafter. JWiTS-1 (1996-2005) was analyzed, and JWiTS-2 (2005-2014) is now under analysis; the following problems have been identified and used to decide future study protocol: (i) there has been a decline in survival rate for patients with rhabdoid tumor of the kidney (RTK) and new treatment strategies are required; (ii) the survival rate for bilateral Wilms tumors (BWT) has improved, but results for renal preservation are unsatisfactory; (iii) the prognosis of stage IV favorable nephroblastoma is very good, suggesting that the current protocols provide overtreatment, particularly for patients with lung metastasis; and (iv) no effective biological risk factors exist for predicting the outcome of Wilms tumor, and a study of the genetic changes of these tumors is necessary to determine biological markers for use in risk classification. To solve these issues, the development of a new risk classification of pediatric renal tumors is required. In addition, different study protocols should be developed according to the risk-based classification of the patients. Further, a new study protocol for BWT began in 2015, and new study protocols are being prepared for RTK, and for Wilms tumor with lung metastasis. In addition, an analysis of biological markers with regard to risk classification is to be performed. Furthermore, to create new protocols for patients with rare renal tumors, international collaboration with Children's Oncology Group and International Society of Pediatric Oncology is necessary.

Transmesenteric hernia is abdominal visceral herniation through a congenital or acquired mesenteric defect. Treves' field is the area of terminal ileal mesentery circumscribed by the ileocolic artery and its last ileal branch. It is very susceptible to congenital defects. To clarify the clinical course of congenital Treves' field transmesenteric hernia (cTFTH), we retrospectively reviewed pediatric cases and conducted a literature review. Five consecutive pediatric cTFTH cases (one male, four females) underwent emergency laparotomy at our institutions from April 2009 and December 2014. We analyzed their demographics, preoperative findings, surgical procedures, and outcomes, along with a literature review. Abdominal plain X-ray showed displaced intestinal gas with gas paucity in the center of the abdomen in two cases and diffusely increased intestinal gas in two others. Abdominal computed tomography (CT) in four cases showed intestinal loop clusters, mesenteric vessel changes, small-bowel obstruction, and ascites. All cases underwent emergency laparotomy for ileal resection with ileocecal valve preservation. Simultaneous anastomoses were performed in four cases; the remaining case underwent ileostomy because of prematurity. Although one case needed reoperation for postoperative bowel obstruction, all patients survived. Literature review of pediatric cTFTH showed a high frequency of necrosis (70.5%) and mortality (26.5%). Among pediatric cTFTH cases, 67.6% were aged

Background/purpose We evaluated the long-term umbilical appearance and patient/parent satisfaction at follow-up after umbilical hernia repair (UHR). Methods In a retrospective review of 128 children who underwent UHR and were followed up for over 5 years, satisfaction was assessed using e-mailed questionnaires and attached photographs of the umbilicus. Results The survey response rate was 80.3% by parents, 79.5% by patients, and 72.1% using photographs. The median follow-up period was 7.6 (range, 5.3–10.1) years. The satisfaction rate was 78.9% among parents and 91.5% among patients; however, eight patients (8.5%) reported dissatisfaction with the results. The main reason for dissatisfaction was the shallow depth of the umbilicus (parents, 10/20; patients, 5/8). During the follow-up period, satisfaction increased in 14 cases (14.7%) and decreased in 10 cases (10.5%). Superior hooding was considered an ideal shape for the umbilicus. Concomitant laparoscopic surgery and higher age at surgery were significantly associated with dissatisfaction in parents ( P = 0.045) and patients ( P = 0.046), respectively. Large defect size was significantly associated with decreased satisfaction during the follow-up period ( P = 0.030). Conclusion E-mail surveys are useful for long-term follow-up after UHR. Patients with these above risk factors should receive long-term follow-up with careful attention to patient satisfaction. Level of evidence Type of study: prognosis study, level II.

An increasing number of children with advanced malignancies have recently received high-dose chemotherapy (HDC) with hematopoietic stem cell transplantation (HSCT), followed by surgery. In this study, we reviewed our experience with surgery after HDC and autologous (auto) or allogeneic (allo) HSCT to elucidate the problems associated with this treatment and establish the optimum surgical management strategy.We retrospectively reviewed the cases of 24 children with advanced malignancy treated with HDC and HSCT before tumor resection at our institution. The tumors included 18 neuroblastomas, 5 soft tissue sarcomas, 2 hepatoblastomas, and 1 Wilms tumor. The source of hematopoietic stem cells was auto-HSCT in 19 patients and allo-HSCT in 5 patients. To be able to undergo surgery, it was necessary that the patient's general condition, including hemostasis, should be fairly good and that the results of hematological examinations should include a white blood cell (WBC) count of1,000/µL, hemoglobin level of10 g/dL and platelet count of5 × 10(4)/µL.The mean duration before WBC recovery after HSCT was 14.5 ± 1.4 days after auto-HSCT and 23.8 ± 1.2 days after allo-HSCT, respectively (p0.01). The mean duration before platelet recovery after HSCT was 46.5 ± 5.2 days for auto-HSCT and 48.6 ± 5.5 days for allo-HSCT (not significant [n.s.]). The mean interval between allo-HSCT and surgery was significantly longer (92.8 ± 6.2 days) than that between auto-HSCT and surgery (57.0 ± 3.9 days) (p0.01), likely because of the use of steroids and immunosuppressants after HSCT. The tumors were completely resected in all cases without severe complications. All the patients treated with allo-HSCT had an acute graft versus host (aGVH) reaction at 2 to 3 weeks after HSCT, and specifically required the administration of steroids and immunosuppressants to prevent aGVH. The postoperative complications included paralytic ileus in two cases and a tacrolimus-associated encephalopathy in one case involving allo-HSCT. In half of the patients, the WBC count was not elevated after surgery, whereas the postoperative serum C-reactive protein (CRP) level was elevated in all cases.Our data indicate that surgical treatment can be safely performed even after HDC with HSCT if attention is paid to myelosuppression and the adverse effects of both chemotherapeutic agents and immunosuppressants.

Background The introduction of multimodal therapy has improved the survival rate of bilateral Wilms tumors (BWT); however, the results are still not satisfactory in terms of the renal preservation. To establish a new treatment strategy for BWT, we reviewed the results of the cases registered in the Japan Wilms Tumor Study Group (JWiTS). Procedure This analysis concerned patients with synchronous BWT registered in the JWiTS between 1996 and 2011. In these patients, the management of BWT included initial tumor resection or biopsy followed by chemotherapy. The details of the treatments and outcomes were analyzed. Results Among the 355 cases registered in the JWiTS database, 31 (8.7%) had BWT. They were 16 males and 15 females with a mean age of 15.5 months. Preoperative chemotherapy was performed in 24 cases. Bilateral nephron-sparing surgery (NSS) was achieved in 10 of 28 cases (36%). All of the cases were of favorable nephroblastoma without anaplasia, and a WT1 mutation was detected in 21 of the 27 cases (78%) examined. The 5-year overall survival was 92.6%; however, 10 children (40%) developed impaired renal function and three of them developed renal failure. Conclusions The long-term survival rates for patients with synchronous BWT have improved. However, more than half of patients receive nephrectomy. The protocol should be changed to improve the rate of preservation of the renal parenchyma. Preoperative chemotherapy should be performed to shrink the tumors in every case, and subsequent NSS should be carried out after a central imaging evaluation. Pediatr Blood Cancer 2014;61:1184–1189. © 2014 Wiley Periodicals, Inc.

The Japanese Study Group for Pediatric Liver Tumor (JPLT) has conducted cooperative treatment studies on hepatoblastoma (HBL) since 1991. The JPLT2 protocol was launched in 1999 to evaluate the efficacy of cisplatin/pirarubicin (CITA) under risk stratification. European and North American groups showed the improvement of HBL patients by pre- and postoperative chemotherapeutic regimens. Therefore, we evaluated the results of JPLT study and considered the future aspect of JPLT.A total of 389 children with malignant hepatic tumors were enrolled in JPLT-2 until 2010. Data from 331 HBL cases were analyzed.Of the 331 patients enrolled, their 5-year overall survival and event-free survival rates were 83.3 and 68.0%, respectively. While outcomes of standard-risk cases (tumors involving 3 or fewer sectors of the liver) were excellent, those of high-risk cases (tumors involving 4 sectors of the liver or with distant metastases) remained poor. For 26 high-risk or relapse/refractory HBL cases, high-dose chemotherapy (HDC) with stem cell transplantation (SCT) was carried out. Among them, 6 of 12 relapse or refractory cases died. Compared with other regimens, the CITA regimen achieved similar or superior rates of survival among children with standard-risk HBL, while HDC with SCT was not effective in patients with high-risk HBL. Presently, a global Children's Hepatic Tumor International Consortium (CHIC) project is ongoing, with a focus on international cooperation and risk stratification in the field of rare liver cancers in children. More promising strategies, including liver transplantation and new targeting drugs under global risk stratification, are being proposed.

Background Survivin, an inhibitor of apoptosis, has been reported to be associated with a worse prognosis in some malignancies. However, its expression in hepatoblastoma (HB) remains to be elucidated. We assessed the survivin expression in HB specimens collected before and after chemotherapy to elucidate the impact of survivin on the outcome of HB therapy. Methods HB specimens were collected before and after 2 to 4 cycles of cisplatin-based chemotherapy from 16 patients. The survivin expression level was assessed by immunohistochemical staining and real-time polymerase chain reaction. Results Out of 16, 12 HB sections collected before chemotherapy were positive for survivin as determined by immunohistochemical staining. The intensity of survivin expression was found to significantly increase after chemotherapy. Surprisingly, all of the HB specimens obtained after chemotherapy were positive for survivin. The expression of survivin messenger ribonucleic acid from a human HB cell line, Huh-6 was significantly higher when the cells were cultured with cis-diamminedichloroplatinum(II) than when the cells were cultured without the drug. Conclusion Our results indicate that most of the primary HB tissue specimens express survivin, and its expression increased after chemotherapy, thus suggesting that survivin may concern with the survival of tumor cells, therefore be a candidate for the target of the treatment of HB.