Retroperitoneal liposarcoma (RLS) is a rare, biologically heterogeneous tumor that present considerable challenges due to its size and deep location. As a consequence, the majority of patients with high-grade RLS will develop locally recurrent disease following surgery, and this constitutes the cause of death in most patients. Here, we review current insights and controversies regarding histology, molecular biology, extent of surgery, (neo)adjuvant treatment, and systemic treatment including novel targeted agents in RLS.

PURPOSE To evaluate the impact of surgeon-assessed extent of primary tumor resection on local progression and survival in patients in the International Society of Pediatric Oncology Europe Neuroblastoma Group High-Risk Neuroblastoma 1 trial. PATIENTS AND METHODS Patients recruited between 2002 and 2015 with stage 4 disease > 1 year or stage 4/4S with MYCN amplification < 1 year who had completed induction without progression, achieved response criteria for high-dose therapy (HDT), and had no resection before induction were included. Data were collected on the extent of primary tumor excision, severe operative complications, and outcome. RESULTS A total of 1,531 patients were included (median observation time, 6.1 years). Surgeon-assessed extent of resection included complete macroscopic excision (CME) in 1,172 patients (77%) and incomplete macroscopic resection (IME) in 359 (23%). Surgical mortality was 7 (0.46%) of 1,531. Severe operative complications occurred in 142 patients (9.7%), and nephrectomy was performed in 124 (8.8%). Five-year event-free survival (EFS) ± SE (0.40 ± 0.01) and overall survival (OS; 0.45 ± 0.02) were significantly higher with CME compared with IME (5-year EFS, 0.33 ± 0.03; 5-year OS, 0.37 ± 0.03; P < .001 and P = .004). The cumulative incidence of local progression (CILP) was significantly lower after CME (0.17 ± 0.01) compared with IME (0.30 ± 0.02; P < .001). With immunotherapy, outcomes were still superior with CME versus IME (5-year EFS, 0.47 ± 0.02 v 0.39 ± 0.04; P = .038); CILP was 0.14 ± 0.01 after CME and 0.27 ± 0.03 after IME ( P < .002). A hazard ratio of 1.3 for EFS associated with IME compared with CME was observed before and after the introduction of immunotherapy ( P = .030 and P = .038). CONCLUSION In patients with stage 4 high-risk neuroblastoma who have responded to induction therapy, CME of the primary tumor is associated with improved survival and local control after HDT, local radiotherapy (21 Gy), and immunotherapy.

Purpose Intestinal malrotation is a congenital intestinal rotation anomaly, which can be treated by either laparotomy or laparoscopy. Our hypothesis is that laparoscopic treatment leads to less small bowel obstruction because of the fewer adhesions in comparison to laparotomy, without increasing the risk of recurrent volvulus. We analyzed the outcome of patients who had a correction for intestinal malrotation after the introduction of laparoscopy. Methods All patients between 0 and 18 years who underwent a surgical procedure for malrotation in the Radboudumc Amalia Children's Hospital, Nijmegen, the Netherlands, between January 2004 and December 2011 were retrospectively reviewed for duration of operation, perioperative complications, length of hospital stay, and rate of redo surgery for intestinal volvulus or obstruction. Results A total of 83 patients were included of which 33 had a laparoscopic procedure and 50 had a laparotomy for suspected malrotation. Operating time was 63 minutes for the laparoscopic procedure versus 76 minutes for laparotomy (p = 0.588). Significantly more complications were found in the laparotomy group (11 vs. 35%, p = 0.047). However, one patient (aged 4 months) in the laparoscopy group developed an early (< 24 hours) recurrent volvulus. Length of hospital stay was significantly longer after a laparotomy (9 vs. 16 days, p = 0.002). Three (17%) patients in the laparoscopy group needed redo surgery compared with six (9%) in the laparotomy group (p = 0.400). No late volvulus occurred in both groups. After laparotomy, redo surgery because of the small bowel obstruction was more frequent (5 vs. 0%), although this was not statistically significant. Conclusion In both the laparoscopy and laparotomy group, no cases of long-term recurrent volvulus were seen. After laparotomy, more patients developed a late small bowel obstruction because of the adhesions for which redo surgery was needed. In the laparotomy group, the number of complications was significantly higher and the length of hospital stay was significantly longer. Comparing laparoscopy and laparotomy for the treatment of malrotation, no difference exists for the long-term risk of recurrent volvulus. In children aged 6 months or older with suspicion of intestinal malrotation but not presenting with an acute abdomen or hemodynamically instability, laparoscopy should be considered as a first approach to diagnose and subsequently treat intestinal malrotation.

To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBLrelated surgical procedures and their outcomes. Summary of Background Data: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. Methods: After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie -German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie -German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. Results: The ''International Neuroblastoma Surgical Report Form'' provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications.

A congenital short bowel (CSB) is a rare entity in pediatric surgery. We present the case of a newborn boy with a total small intestinal length of 47 cm, malrotation and gastroesophageal reflux, who is 19 months old at the time of this report. Main treatment steps were Ladd's procedure, a fundoplication and long-term parenteral nutrition. We suggest that missing physiological herniation of the gut into the coelomic cavity may impair normal intestinal growth and rotation and lead to congenital short bowel. Review of all cases reported in the literature shows a considerable mortality of 88%. The limiting factor seems to be reduced motility of the short small bowel causing functional obstruction and liver failure.

Thirteen years of experience with a laparoscopic technique mimicking, as closely as possible, the classical open technique are presented in this report. Consecutive laparoscopic herniotomies in 385 children (178 girls and 207 boys; age 0.13 to 16.8 years) with a total of 525 hernia repairs were included into a retrospective review. Overall, 95% of children were treated on a day-care basis; all operations were completed laparoscopically. Mean operation time was 32.1 minutes (26.2 for unilateral hernias and 34.5 for bilateral). In total, 10.1% of children presented with clinically bilateral hernias, whereas 29.2% of clinical unilateral hernias had a patent contralateral processus vaginalis. We discovered femoral hernias in 2.6% and direct inguinal hernias in 0.5% of the cases. In 79 cases, a concomitant of umbilical herniorraphy was performed. There were no cases of testicular malposition or atrophy. We had a 1% hernia recurrence rate. Cosmetic outcome was excellent. Laparoscopy provides the surgeon with a superior diagnostic tool, facilitating the diagnosis of any kind of inguinal hernia or controlateral patent processus vaginalis and its repair in the same session; surgery- and anesthesia-related stress is minimized. This makes it an interesting alternative to the open repair of inguinal hernias in children, providing an excellent view on the cord structures and leaving them, as well as the testis, untouched.

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Background and study aims: Transanal irrigation (TAI) is used in children to treat constipation and incontinence. Belgium has 2 systems available: Colotip® (cheaper, however not designed for TAI) or Peristeen®. Patients and methods: This patient-control switch study is the first to compare 2 TAI systems. Children regularly using Colotip® for TAI were asked to participate, after consent, a visual analogue scale (VAS) rating the system and a 2-week diary (fecal continence, self-reliance, time spent on the toilet, pain, Bristol stool scale, irrigation volume and frequency of enema) were completed. Non-parametric statistics were used. Results: Out of 26 children using Colotip®, 18 (69%) children participated and 5 refused (fear n=1, satisfaction Colotip® system n=7). Of these 18 children (interquartile range: 3-18 years, median 12.5 years, 9 girls) 5 patients stopped Peristeen® (pain n=1, fear n=1 and balloon loss n=3) and 2 were lost from follow up. Dropouts and included patients showed no statistical difference. In the 11 remaining patients, pseudo-continence (p 0.015), independence (p 0.01) and VAS score (p 0.007) were significantly better with Peristeen®, no difference was found in time spent on the toilet (p 0.288) and presence of pain (p 0.785). Conclusions: In children Peristeen® offered significantly higher pseudo-continence and independency. 30% refused participation because of satisfaction with the Colotip® and 30% spina bifida patients reported rectal balloon loss due to sphincter hypotony. To diminish Peristeen® failure, a test-catheter could be of value. Considering Colotip® satisfaction, both systems should be available. Patient selection for Peristeen® needs further research.