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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.

Purpose This study aims to clarify the relationship between changes in skeletal muscle mass during treatment and prognosis of pediatric malignant solid tumors. Methods Patients with pediatric malignant solid tumors who were treated at Kyushu University Hospital from 2007 to 2017 were divided into two groups: the progression-free survival (PFS) group and the relapse/death (R/D) group; the psoas major muscle volume (PMV) was then compared. We also measured the PMV and psoas muscle area (PMA) of pediatric patients with no complications who underwent surgery for acute appendicitis (control) and compared the values with those of patients with malignant tumors. Results No significant differences were observed in the PMV and PMA between patients with appendicitis and those with malignant tumors. Significant differences were found in the rate of change in PMV between the PFS (1.424) and R/D groups (1.071) ( P = 0.0024). When the cutoff value of the rate of change in the PMV was 1.20, patients whose rate of change in PMV was ≥ 1.20 had longer PFS ( P = 0.0231) and overall survival ( P = 0.0229) than those whose rate of change was Conclusion Pediatric patients with malignant solid tumors and increased skeletal muscle mass during treatment have a good prognosis.

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.

Aim: We evaluated a series of late-presenting congenital diaphragmatic hernia (Late-CDH) cases and assessed the reliability and risks of laparoscopic and thoracoscopic approaches for Late-CDH at a ...

Background: We actively use novel endoscopic surgical approaches with complete curability and good cosmetic outcomes to facilitate the removal of resected tumors from the body via a small incision....

Emerging data have suggested that sirolimus may be a treatment option for complicated vascular anomalies (VAs). The present study aimed to investigate the immunologic effects of sirolimus treatment for 6 months in patients with VAs. Blood samples obtained from the patients enrolled in 2 multicenter studies to investigate the efficacy of sirolimus for VAs before and after sirolimus treatment for 6 months were used. Data for total white blood cell count, absolute lymphocyte count, serum immunoglobulins (Igs) levels (IgG, IgA, IgM), lymphocyte proliferation assays with mitogens including phytohemagglutinin and concanavalin A, and flow cytometric analysis of lymphocyte subsets were evaluated. A total of 18 patients with VAs receiving sirolimus treatment were included in the study. Comparisons of white blood cell, absolute lymphocyte count, IgG, IgA, IgM, and reaction rates of phytohemagglutinin and concanavalin A revealed no significant differences before and after treatment. No significant differences were observed in the absolute counts of lymphocyte subtypes before and after treatment, except for regulatory T-cell counts, which were significantly decreased after treatment. Severe infections were not observed during sirolimus treatment. The immunologic parameters assessed in the present study were hardly affected by sirolimus treatment for 6 months in patients with VAs.

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

Introduction Lymphatic anomalies (LAs) refer to a group of diseases involving systemic dysplasia of lymphatic vessels. These lesions are classified as cystic lymphatic malformation (macrocystic, microcystic or mixed), generalized lymphatic anomaly, and Gorham–Stout disease. LAs occur mainly in childhood, and present with various symptoms including chronic airway problems, recurrent infection, and organ disorders. Individuals with LAs often experience progressively worsening symptoms with a deteriorating quality of life. Although limited treatment options are available, their efficacy has not been validated in prospective clinical trials, and are usually based on case reports. Thus, there are no validated standards of care for these patients because of the lack of prospective clinical trials. Methods This open-label, single-arm, multicenter, prospective study will assess the efficacy and safety of a mammalian target of the rapamycin inhibitor sirolimus in the treatment of intractable LAs. Participants will receive oral sirolimus once a day for 52 weeks. The dose is adjusted so that the nadir concentration remains within 5–15 ng/ml. The primary endpoint is the response rate of radiological volumetric change of the target lesion confirmed by central review at 52 weeks after treatment. The secondary endpoints are the response rates at 12 and 24 weeks, respiratory function, pleural effusion, ascites, blood coagulation parameters, bleeding, pain, quality of life, activities of daily living, adverse events, side effects, laboratory examinations, vital signs, and pharmacokinetic data. Results This is among the first multicenter studies to evaluate sirolimus treatment for intractable LAs, and few studies to date have focused on the standard assessment of the efficacy for LAs treatment. Our protocol uses novel, uncomplicated methods for radiological assessment, with reference to the results of our previous retrospective survey and historical control data from the literature. Conclusions We propose a multicenter study to investigate the efficacy and safety of sirolimus for intractable LAs (SILA study; trial registration UMIN000028905). Our results will provide pivotal data to support the approval of sirolimus for the treatment of intractable LAs., Highlights • This is among the first multicenter studies to evaluate sirolimus for intractable LAs. • The study design is useful for evaluating sirolimus treatment in LAs. • Our study protocol uses novel, uncomplicated methods of radiological assessment.

When a tumor and trauma coexist, the treatment strategy must be established while considering their interaction. We herein report a 5-month-old girl with Wilms tumor complicated by blunt renal trauma. She was involved in a traffic accident and had hemorrhagic shock due to renal bleeding. We performed hemostasis by transcatheter arterial embolization. Ten days later, we extirpated the potential malignant tumor and left kidney. We were able to complete the surgery without rupture or major bleeding. Postoperative histopathology confirmed Wilms tumor. In the year since she received postoperative chemotherapy, there has been no recurrence. When we were deciding the treatment strategy, we first had to determine how much the renal trauma had affected the tumor staging. The second issue was when to extirpate the tumor after managing the trauma. There are no standard criteria for such situations at present, so we referred to the criteria concerning the bed rest period in cases of traumatic kidney injury and previous case reports and decided to wait over a week from the injury treatment to perform surgery. As a result, we were able to remove the tumor completely without any rupture or major bleeding.

The mechanism underlying the spontaneous regression of neuroblastoma is unclear. Although it was hypothesized that this regression occurs via an immunological mechanism, there is no clinical evidence, and no animal models have been developed to investigate the involvement of immune systems, especially natural antibodies, against neuroblastoma. We performed an immunological analysis of homo- and heterozygous TH-MYCN transgenic mice as a model of aggressive neuroblastoma. Mice with no or small (5 mm) tumors showed higher antibody titers in plasma than mice with large (5 mm) tumors. A significant negative correlation was observed between the tumor diameter and the titer of antitumor antibody. This antibody had complement-dependent cytotoxicity but not antibody-dependent cellular cytotoxicity against neuroblastoma cells. Moreover, B-cell depletion had no effect on the tumor incidence in vivo. We revealed that TH-MYCN transgenic mice have a natural antibody against neuroblastoma that correlate with tumor size. However, this antibody does not correlate with the spontaneous regression of neuroblastoma. Thus, the function of the natural antibody is limited.

Although hepatoblastoma is the most common pediatric liver cancer, its genetic heterogeneity and therapeutic targets are not well elucidated. Therefore, we conducted a multiomics analysis, including mutatome, DNA methylome, and transcriptome analyses, of 59 hepatoblastoma samples. Based on DNA methylation patterns, hepatoblastoma was classified into three clusters exhibiting remarkable correlation with clinical, histological, and genetic features. Cluster F was largely composed of cases with fetal histology and good outcomes, whereas clusters E1 and E2 corresponded primarily to embryonal/combined histology and poor outcomes. E1 and E2, albeit distinguishable by different patient age distributions, were genetically characterized by hypermethylation of the HNF4A/CEBPA-binding regions, fetal liver-like expression patterns, upregulation of the cell cycle pathway, and overexpression of NQO1 and ODC1. Inhibition of NQO1 and ODC1 in hepatoblastoma cells induced chemosensitization and growth suppression, respectively. Our results provide a comprehensive description of the molecular basis of hepatoblastoma and rational therapeutic strategies for high-risk cases.

Purpose: This study aimed to evaluate the learning effects of continuous training with a disease-specific endoscopic surgical simulator for young pediatric surgeons. Materials and Methods:...

Case We report a 2-year-old boy with duodenal ulcer with active bleeding that occurred as a result of norovirus gastroenteritis. On admission, the patient presented with shock accompanied with vomiting and melena. Abdominal contrast enhanced computed tomography scan showed signs of duodenal bleeding. Outcome He was successfully treated with emergent transcatheter arterial embolization. After the treatment, endoscopic examination revealed duodenal ulcer and the stool norovirus antigen test was found to be positive. The patient recovered completely without any sequelae. Conclusion Life-threatening duodenal ulcer bleeding in children can be caused by viral gastroenteritis. When endoscopic therapy is unsuccessful or difficult, in cases of small children, angiographic intervention can be a safe alternative treatment option of gastrointestinal bleeding.

We developed and validated a specific laparoscopic fundoplication simulator for use with the objective endoscopic surgical skills evaluation system. The aim of this study was to verify the quality of skills of surgeons. We developed a 1-year-old infant body model based on computed tomography data and reproduced pneumoperitoneum model based on the clinical situation. The examinees were divided into three groups: fifteen pediatric surgery experts (PSE), twenty-four pediatric surgery trainees (PSN), and ten general surgeons (GS). They each had to perform three sutures ligatures for construction of Nissen wrap. Evaluate points are time for task, the symmetry of the placement of the sutures, and the uniformity of the interval of suture ligatures in making wrap. And the total path length and velocity of forceps were measured to assess bi-hand coordination. PSE were significantly superior to PSN regarding total time spent (p

This study evaluated the stability and risk of single-incision laparoscopic percutaneous extraperitoneal closure (SILPEC) for pediatric inguinal hernia performed by inexperienced pediatric surgeons versus conventional LPEC procedure.Between 2011 and 2012, a randomized prospective study was performed comparing SILPEC (n = 37, 16 uni- and 21 bilateral patent processus vaginalis [PPV]) to LPEC (n = 72, 39 uni- and 33 bilateral PPV). The procedures were performed in girls with inguinal hernia by inexperienced pediatric surgeons with the assistance of an expert pediatric surgeon. In SILPEC, a laparoscope was placed through a transumbilical incision. A 2-mm trocar for the grasper was inserted through the same incision and introduced into the extraperitoneal cavity. The tip of the trocar was inserted in the abdominal cavity distant from the umbilical incision by the expert surgeon to avoid any complications caused by the in-line view. Using a special needle, the hernial sac was closed extraperitoneally by the inexperienced surgeon. A statistical survey of the mean age at operation, mean operative time, intra- and postoperative complications, and recurrence in the SILPEC and LPEC groups was performed.There were no significant differences in the mean age or operative time. There were fewer total number of postoperative complications in the SILPEC group compared with the LPEC group (P = .0707). No intraoperative complications or recurrence occurred.Considering the risks and need to improve endoscopic surgical skills with useful instruments specialized for SILPEC, inexperienced surgeons can successfully perform SILPEC safely under expert pediatric surgeons.

Background Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in neonates and infants. Although most cases of infantile SCT are benign tumors by nature, some develop into extremely large lesions, leading to massive bleeding, high-output heart failure, disseminated intravascular coagulation, and even fatal outcomes during the neonatal period. In addition, some patients may present with tumor recurrence, malignant transformation, long-term sequelae (including bladder and bowel dysfunction) and lower leg palsy during the long-term follow up. SCT, however, is very rare, and there are few opportunities to encounter this disease, therefore general physicians without expert credentials currently lack information relevant to clinical practice. For this reason, the research project committee has compiled guidelines concerning SCT. Methods The purpose of these guidelines was to share information concerning the treatment and follow up of infantile SCT. The guidelines were developed using the methodologies in the Medical Information Network Distribution System. A comprehensive search of the English- and Japanese-language articles in PubMed and Ichu-Shi Web identified only case reports or case series, and the recommendations were developed through a process of informal consensus. Results The clinical questions addressed the risk factors, the efficacy of cesarean section, the initial devascularization of tumor feeding vessels, interventional radiology, recommended clinical studies for follow up and possible long-term complications. Conclusions These are the first guidelines for SCT to be established in Japan, and they may have huge clinical value and significance in terms of developing therapeutic strategies and follow up, potentially contributing to the improvement of the prognosis and quality of life of SCT patients.

The RNA-binding protein HuC/D displays a neuron-specific expression and is involved in neuronal differentiation and the maintenance of the nervous system. Here we investigated the diagnostic value of HuC/D in neuroblastomas. We evaluated 85 neuroblastic tumors: 81 neuroblastomas; 3 ganglioneuroblastomas, intermixed; 1 ganglioneuroma, maturing; and 101 other tumors consisting of 34 Ewing sarcomas, 14 nephroblastomas, 11 rhabdomyosarcomas, 15 pulmonary small cell carcinomas, 18 pancreatic neuroendocrine tumors, and 9 pheochromocytomas. Immunohistochemistry for HuC/D, PHOX2B, and tyrosine hydroxylase was performed. The immunoreactivity for HuC/D was semiquantified using the total score (TS; range, 0-8). HuC/D positivity was defined as a TS ≥6. The TS of the neuroblastic tumors (mean TS, 7.94) was significantly higher than those of the other small round cell tumors and neuroendocrine tumors (P < .001) except for the pheochromocytomas (mean TS, 6.89; P = .074). HuC/D was positive in all 85 neuroblastic tumors, 1 (2.9%) Ewing sarcoma, 1 (6.7%) pulmonary small cell carcinoma, and 8 (89%) pheochromocytomas. PHOX2B was positive in all of the neuroblastic tumors and pheochromocytomas. Tyrosine hydroxylase was positive in 80 (94%) neuroblastic tumors, 1 (9.1%) rhabdomyosarcoma, and all of the pheochromocytomas. Therefore, HuC/D serves as a highly sensitive diagnostic marker to distinguish neuroblastomas from other small round cell tumors. The combination of HuC/D and PHOX2B staining may be valuable for the diagnosis of neuroblastic tumors, especially in the assessment of small sections. HuC/D expression in tumors may be related to catecholamine production or a neural crest-derived cell origin.

The occupational exposure to hazardous drugs(HD)has already been investigated; however, the actual exposure of the attendant family members of patients with childhood cancer has remained unknown. Here, we analyzed cyclophosphamide (CPM)exposure in attendant family members and the environment after the administration of CPM to patients with pediatric cancer. CPM of 320(8.39-1,510)ng from infant-families and 0(0-58.4)ng from adolescent-families were detected(p= 0.01). The exposure of infant-families was significantly greater than those of adolescent-families. In addition, CPM were detected in the hot water after bathing the infant, underwear, and sheets. We elucidated that the exposures take place through body fluid and excretions of the children. In the field of childhood cancer, HD exposure measures should be taken according to the age of the child to minimize health damage to medical personnel, family members, and other children who share the room. Nurses are recommended to educate the patients and their family members about preventing exposure to HD in pediatric medical centers.

Purpose Sarcopenia is a syndrome that is defined by the loss of skeletal muscle mass, quality, and strength. In adult patients with malignancies, the presence of sarcopenia is known to be correlated with a poor prognosis; however, there have been no reports on the influence of sarcopenia on malignant tumors in pediatric patients. In the present study, we investigated whether or not sarcopenia affects the prognosis of high-risk neuroblastoma. Materials and methods Thirteen patients with high-risk neuroblastoma who were treated according to the standard protocol at our hospital from 2007 to 2016 were divided into a progression-free survival group (n = 8) and a relapse/death group (n = 5). The rate of change in sarcopenia was calculated by comparing the psoas muscle area (PMA) of the L3-level lumbar spine on computed tomography before and after treatment with the standard protocol. The rate of change in the PMA, Kaup index, and serum albumin level were compared. Furthermore, we determined the cutoff rate of change in the PMA and compared the overall and progression-free survival. Results The rates of change in the PMA were 1.24 and 0.84 in the progression-free survival and relapse/death groups, respectively (P = 0.0472). There were no significant differences in the rates of change in the Kaup index or the serum albumin level of the two groups. The patients whose rate of change in the PMA was >1.00 showed a prolonged overall (P = 0.0078) and progression-free survival (P = 0.006). Conclusions A decrease in the skeletal muscle mass was suggested to be a significant prognostic factor for high-risk neuroblastoma.

Thoracoscopic surgery for pediatric benign tumors is a common procedure. However, a large incision is needed to remove large tumors from the thoracic cavity. And, for intrapulmonary sequestration in lower lobe, it sometimes needs a large incision to ligate the aberrant vessels. A muscle-sparing axillar skin crease incision (MSASCI) has been introduced for thoracic open surgery, resulting in excellent aesthetic outcomes compared with a standard incision. We herein report the utility of this MSASCI technique in thoracoscopic surgery to remove large tumors from the thoracic cavity and to ligate the aberrant vessels in intrapulmonary sequestration in lower lobe.From 2014 April to 2016 March, we performed the MSASCI technique in thoracoscopic surgeries for 5 children.Five cases were diagnosed as mediastinal masses (mature teratoma for 1 case, ganglioneuroblastoma for 2 cases, and extrapulmonary sequestration and intrapulmonary sequestration for 1 case each). The age at surgery was 32.0 ± 25.0 months (range 5-58 months). The size of the mediastinal mass was 9 × 5 × 5 cm, 4 × 3 × 3 cm, 5 × 5 × 2.5 cm, and 3 × 2.5 × 2 cm. For 4 other cases, except for the intrapulmonary sequestration case, the mass was resected under thoracoscopic surgery using only three or four 5-mm trocars and the mass was removed from the thoracic cavity using the MSASCI technique. For the intrapulmonary sequestration case, the aberrant vessels were resected under thoracoscopic surgery using only two 5-mm and one 12-mm trocars and the left lower lobectomy was performed using the MSASCI technique. All lesions were resected completely. No cases had surgical complications, none showed recurrence, and all cases demonstrated good cosmetic outcomes.Performing thoracoscopic surgery using a MSASCI technique is associated with good cosmetic outcome.

Background Three-dimensional (3D) printed models based on computed tomography (CT) images facilitate the visualization of complex structures and are useful for understanding the surgical anatomy preoperatively. We developed a preoperative surgical simulation method using a 3D printed model based on CT images obtained prior to laparoscopic adrenalectomy for adrenal neuroblastomas (NBs). Materials and methods The multi-detector CT images were transferred to a 3D workstation, and 3D volume data were obtained by reconstructing the sections. A model was made with a 3D printer using acrylic ultraviolet curable resin. The adrenal tumor, kidney, renal vein and artery, inferior vena cava, aorta, and outer body were fabricated. The pneumoperitoneum, insertion of trocars, and laparoscopic view were all attainable in this model. We used this model for three cases with adrenal NB. Results We used this model to discuss the port layout before the operation and to simulate the laparoscopic view and range of forceps movement. All three cases with NB were completely resected without any surgical complications. Conclusions The surgical simulation using 3D printed models based on preoperative CT images for adrenal NB was very useful for understanding the patient's surgical anatomy and for planning the surgical procedures, especially for determining the optimal port layout.

Pediatric surgeons require highly advanced skills when performing endoscopic surgery; however, their experience is often limited in comparison to general surgeons. The aim of this study was to evaluate the effectiveness of endoscopic surgery training for less-experienced pediatric surgeons and then compare their skills before and after training. Young pediatric surgeons (n = 7) who participated in this study underwent a 2-day endoscopic skill training program, consisting of lectures, box training and live tissue training. The trainees performed the Nissen construction tasks before and after training using our objective evaluation system. A statistical analysis was conducted using the two-tailed paired Student’s t tests. The time for task was 984 ± 220 s before training and 645 ± 92.8 s after training (p

The patient is a 3-year-old female diagnosed with PRETEXT IV hepatoblastoma (HB). Although the tumor was decreased after the neoadjuvant chemotherapy, HB still located at the porta hepatis. The patient underwent extended left lobectomy successfully after surgical simulation using three-dimensional (3D) printing liver model based on preoperative CT.

This study investigated whether parameters derived from hand motions of expert and novice surgeons accurately and objectively reflect laparoscopic surgical skill levels using an artificial intelligence system consisting of a three-layer chaos neural network. Sixty-seven surgeons (23 experts and 44 novices) performed a laparoscopic skill assessment task while their hand motions were recorded using a magnetic tracking sensor. Eight parameters evaluated as measures of skill in a previous study were used as inputs to the neural network. Optimization of the neural network was achieved after seven trials with a training dataset of 38 surgeons, with a correct judgment ratio of 0.99. The neural network that prospectively worked with the remaining 29 surgeons had a correct judgment rate of 79% for distinguishing between expert and novice surgeons. In conclusion, our artificial intelligence system distinguished between expert and novice surgeons among surgeons with unknown skill levels.

We report 2 infantile cases of pulmonary tumor carrying a chimeric A2M-ALK gene. A2M-ALK is a newly identified anaplastic lymphoma kinase (ALK)-related chimeric gene from a tumor diagnosed as fetal lung interstitial tumor (FLIT). FLIT is a recently recognized infantile pulmonary lesion defined as a mass-like lesion that morphologically resembles the fetal lung. Grossly, FLIT characteristically appears as a well-circumscribed spongy mass, whereas the tumors in these patients were solid and firm. Histologically, the tumors showed intrapulmonary lesions composed of densely proliferating polygonal or spindle-shaped mesenchymal cells with diffuse and dense infiltrations of inflammatory cells forming microcystic or micropapillary structures lined by thyroid transcription factor 1-positive pneumocytes, favoring inflammatory myofibroblastic tumor rather than FLIT. The proliferating cells were immunoreactive for ALK, and A2M-ALK was identified in both tumors with reverse-transcription polymerase chain reaction. The dense infiltration of inflammatory cells, immunoreactivity for ALK, and identification of an ALK-related chimeric gene suggested a diagnosis of inflammatory myofibroblastic tumor. Histologically, most reported FLITs show sparse inflammatory infiltrates and a relatively low density of interstitial cells in the septa, although prominent infiltration of inflammatory cells and high cellularity of interstitial cells are seen in some FLITs. The present cases suggest that ALK rearrangements, including the chimeric A2M-ALK gene, may be present in these infantile pulmonary lesions, especially those with inflammatory cell infiltration. We propose that these infantile pulmonary lesions containing a chimeric A2M-ALK gene be categorized as a specific type of inflammatory myofibroblastic tumor that develops exclusively in neonates and infants.

The purpose of this study was to develop a new objective assessment system for the suture ligature method employed in the laparoscopic intestinal anastomosis model. Suturing skills were evaluated objectively using this system. This study compared 17 expert surgeons, each of whom had performed >500 laparoscopic procedures, with 36 novice surgeons, each of whom had performed

Purpose An endoscopic system is needed that presents informative images irrespective of the surgical situation and the number of degrees of freedom in endoscopic manipulation. This goal may be achieved with a virtual reality view for a region of interest from an arbitrary viewpoint. An endoscopic pseudo-viewpoint alternation system for this purpose was developed and tested. Method Surgical experts and trainees from an endoscopic surgery training course at the minimally invasive surgery training center of Kyushu University were enrolled in a trial of a virtual reality system. The initial viewpoint was positioned to approximate the horizontal view often seen in laparoscopic surgery, with \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$20^{\circ }$$\end{document}20∘ between the optical axis of the endoscope and the task surface. A right-to-left suturing task with right hand, based on a task from the endoscopic surgery training course, was selected for testing. We compared task outcomes with and without use of a new virtual reality-viewing system. Result There was a 0.37 mm reduction in total error (\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$p = 0.02$$\end{document}p=0.02) with use of the proposed system. Error reduction was composed of 0.1 mm reduction on the y-axis and 0.27 mm reduction on the x-axis. Experts benefited more than novices from use of the proposed system. Most subjects worked at a pseudo-viewpoint of around 34\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$^\circ $$\end{document}∘. Discussion Suturing performance improved with the new virtual reality endoscopic display system. Viewpoint alternation resulted in an overview that improved depth perception and allowed subjects to better aim the marker. This suggests the proposed method offers users better visualization and control in endoscopic surgery.

The number of operations performed by a surgeon may be an indicator of surgical skill. The hand motions made by a surgeon also reflect skill and level of expertise. We hypothesized that the hand motions of expert and novice surgeons differ significantly, regardless of whether they are familiar with specific tasks during an operation.This study compared 11 expert surgeons, each of whom had performed100 laparoscopic procedures, and 27 young surgeons, each of whom had performed15 laparoscopic procedures. Each examinee performed a specific skill assessment task, in which instrument motion was monitored using magnetic tracking system. We analyzed the paths of the centers of gravity of the tips of the needle holders and the relative paths of the tips using two mathematical methods of detrended fluctuation analysis and unstable periodic orbit analysis.Detrended fluctuation analysis showed that the exponent in the function describing the initial scaling exponent (α1) differed significantly for experts and novices, being close to 1.0 and 1.5, respectively (P 0.01). This indicated that the expert group had a greater long-range coherence with an intrinsic sequence and smooth continuity among a series of motions. Likewise, unstable periodic orbit analysis showed that the second period of unstable orbit was significantly longer for experts in comparison with novices (P 0.01). This demonstrates mathematically that the hands of experts are more stable when performing laparoscopic procedures.Objective evaluation of hand motion during a simulated laparoscopic procedure showed a significant difference between experts and novices.

Retroperitoneal enteric duplication (ED) without any communications to the alimentary tract is extremely rare. We herein report a laparoscopic resection of an isolated retroperitoneal ED in an infant. The patient was a 7-month-old girl, who was found shortly after birth to have a complex, 2 cm mass in the region of the right adrenal gland. The size and imaging appearance of the mass did not change over a 7-month observation period. We performed a laparoscopic resection of the mass, and pathology revealed an isolated ED. To our knowledge, this is the first report of a laparoscopic resection of a retroperitoneal duplication cyst with the imaging findings of a polycystic lesion in an infant.

In neuroblastoma (NB), one of the most common paediatric solid tumours, activation of anaplastic lymphoma kinase (ALK) is often associated with poor outcomes. Although genetic studies have identified copy number alteration and nonsynonymous mutations of ALK, the regulatory mechanism of ALK signalling at protein levels is largely elusive. Neuronal leucine-rich repeat 1 (NLRR1) is a type 1 transmembrane protein that is highly expressed in unfavourable NB and potentially influences receptor tyrosine kinase signalling. Here, we showed that NLRR1 and ALK exhibited a mutually exclusive expression pattern in primary NB tissues by immunohistochemistry. Moreover, dorsal root ganglia of Nlrr1+/+ and Nlrr1−/− mice displayed the opposite expression patterns of Nlrr1 and Alk. Of interest, NLRR1 physically interacted with ALK in vitro through its extracellular region. Notably, the NLRR1 ectodomain impaired ALK phosphorylation and proliferation of ALK-mutated NB cells. A newly identified cleavage of the NLRR1 ectodomain also supported NLRR1-mediated ALK signal regulation in trans. Thus, we conclude that NLRR1 appears to be an extracellular negative regulator of ALK signalling in NB and neuronal development. Our findings may be beneficial to comprehend NB heterogeneity and to develop a novel therapy against unfavourable NB.

We verified the appropriate port location for laparoscopic hepaticojejunostomy using a comprehensive laparoscopic training simulator. We developed a hepaticojejunostomy model, consist of common hepatic duct and intestine and participants required to place two sutures precisely using two different port locations (A: standard port location, B: modified port location). The order of tasks was randomly determined using the permuted block method (Group I: Task A → Task B, Group II: Task B → Task A). The time for task completion and total number of errors were recorded. In addition, we evaluated the spatial paths and velocity of both forceps. Statistical analyses were performed using a statistical software program. The time for the task, the total error score, and the spatial paths and velocity of both forceps were not significantly different between groups I and II. Furthermore, the port location and order of tasks (group I or group II) did not significantly affect the results. In contrast, there were significant differences in the performance between experts and novices, who were classified as such based on the total number of experienced endoscopic surgeries. Preoperative port simulation in advanced surgery using our artificial simulator is feasible and may facilitate minimally invasive surgery for children.

The implications of surgical intervention for neuroblastomas were assessed in one institution. We analyzed the clinical characteristics and extension of resection in 123 pediatric patients with neuroblastoma diagnosed between 1985 and 2004. The 5-year survival rate of the 82 patients under 12 months of age, 59 of whom were treated with complete resection of the primary tumor, was 97%. The 5-year survival rate of the 41 patients over 12 months of age did not differ significantly according to whether complete (n = 19) or incomplete resection (n = 22) was performed (46 vs. 38%, respectively). No local recurrence was observed in ten patients over 12 months of age with stage 4 disease who underwent complete resection of the primary tumor; however, four of these ten patients died of metastatic recurrence. Considering that the majority of infantile neuroblastomas in this study had favorable biology, complete resection might be unnecessary for patients under 12 years of age. For advanced neuroblastomas in patients over 12 months of age, the main treatment for metastasis is systemic chemotherapy, although extirpation of the primary tumor without extensive surgery might prevent local recurrence when combined with radiation therapy.

MYCN amplification (MYCN-A) is a strong prognostic factor in neuroblastoma (NB). MYCN gain which is a low level of MYCN-A as determined by FISH. It is unclear whether the MYCN gain is the pre-status of MYCN-A. This study assessed the status of MYCN gene and chromosome 2p of MYCN-A, MYCN gain and no MYCN amplification using a single nucleotide polymorphism (SNP) array, and the clinical implication of MYCN gain in NB. The status of the MYCN gene was determined by FISH in 47 primary NB samples and the status of chromosome 2p in all cases was analyzed using an SNP array. 8 of the 47 cases analyzed using FISH showed MYCN-A, 7 cases showed MYCN gain and 32 cases showed no MYCN amplification. An SNP array analysis showed that only 2 of 8 cases with MYCN-A by FISH had both amplification of MYCN region and distal 2p gain and other 6 cases had amplification of the MYCN region without distal 2p gain. All 7 cases with MYCN gain by FISH had distal 2p gain without amplification of the MYCN region, and all 32 cases with no MYCN amplification by FISH demonstrated neither the amplification of the MYCN region nor the 2p gain. 5-year overall survival rate of patients with MYCN gain (n = 7, 71.4%) was not significant different from that of patients with no MYCN amplification (n = 32, 90.6%) by FISH (p = 0.11). These results suggested that the MYCN gain detected by FISH represents the 2p gain, and the MYCN gain is not considered to represent the pre-status of MYCN amplification.

BACKGROUND AND AIMS: The standard palliative chemotherapy for pancreatic ductal adenocarcinoma (PDAC) is gemcitabine-based chemotherapy; however, PDAC still presents a major therapeutic challenge. The aims of this study were to investigate the expression pattern of genes involved in gemcitabine sensitivity in resected PDAC tissues and to determine correlations of gene expression with treatment outcome. MATERIALS AND METHODS: We obtained formalin-fixed paraffin-embedded (FFPE) tissue samples from 70 patients with PDAC. Of the 70 patients, 40 received gemcitabine-based adjuvant chemotherapy (AC). We measured hENT1, dCK, CDA, RRM1, and RRM2 messenger RNA (mRNA) levels by quantitative real-time reverse transcription-polymerase chain reaction and determined the combined score (GEM score), based on the expression levels of hENT1, dCK, RRM1, and RRM2, to investigate the association with survival time. By determining the expression levels of these genes in endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) cytologic specimens, we investigated the feasibility of individualized chemotherapy. RESULTS: High dCK (P = .0067), low RRM2 (P = .003), and high GEM score (P = .0003) groups had a significantly longer disease-free survival in the gemcitabine-treated group. A low GEM score (

Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43–50% and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient’s recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values. This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.

To select the optimal treatment according to the grade of malignancy of neuroblastoma (NB), it is essential to accurately and rapidly identify genetic abnormalities associated with the prognosis. We have identified BIN1 and neuronatin beta as the novel prognosis-related genes for NBs. This study aims to assess the correlation between the combination of the expression level of prognosis-related genes and the outcome of NB.In 44 NB samples, the expression levels of TrkA, BIN1, and neuronatin beta were determined using quantitative reverse transcriptase-polymerase chain reaction; furthermore, the correlation between the expression of these genes' expression levels and the clinical progression of NB were assessed.It was possible to classify 44 NBs into 4 groups regarding the grade of malignancy of NB. These 4 groups were all significantly associated with the clinical stages international NB staging system as well as the outcomes of the patients (P.001, according to the trend test by Kruskal-Wallis exact test).The combination of the expression levels of these genes using quantitative reverse transcriptase-polymerase chain reaction is indicated as the effective method to quickly and accurately evaluate the grade of malignancy of NBs.