Influence of Surgical Excision on the Survival of Patients With Stage 4 High-Risk Neuroblastoma: A Report From the HR-NBL1/SIOPEN Study.

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.

[Rare diagnostically difficult causes of acute abdomen in children]. / Rzadsze lub trudne diagnostycznie przyczyny ostrego brzucha u dzieci.

Abdominal pain is a very nonspecific sign that may be encountered in many diseases in children. The differentiation between acute abdomen and other pathologies may cause problems in many situations. The paper summarizes atypical causes of the acute abdomen which are rare but may be seen in the big, working hospital.

[Gastrografin challenge test for the management of subileus in children]. / PaSaz gastrografiny w ocenie watpliwej niedroznosci jelit u dzieci.

BACKGROUND: The appearance of gastrografin in colon within 6 hours after gastric administration rules out the need of surgery in abdominal subileus. This diagnostic management is not routinely applied in children. We present a one-year experience from the department of pediatric surgery. MATERIAL AND METHODS: Between April 2006 and September 2007 children with symptoms of ileus without clear indications for surgery were subjected to the study. Naso-gastric tube was inserted and 20-100 cc of gastrografin was administered. Abdominal radiograph was taken within 4-6 hours. The presence of contrast in colon allowed for conservative management. All remaining children were subjected to surgery. Newborns and children with intussusception or incarcerated hernia were ruled out of the study. RESULTS: The study was implemented in 8 girls and 7 boys ranking in the age between 1 and 17 (mean 11) years. Thirteen children had postoperative obstruction (8 after appendectomy, 5 after other laparotomy). Two children (with Crohn disease and Schoenlein-Henoch purpura) were not operated before. Four children without appearance of contrast in colon were operated. None of the remaining eleven children required surgical intervention. CONCLUSIONS: This management is safe and effective. It brings forward decision for surgery and shortens observation in children who don't require surgical intervention.

[Can intensive induction chemotherapy eradicate retroperitoneal lymph node metastases in children over 1 year of age with stage 4 neuroblastoma?]. / Czy intensywna chemioterapia indukcyjna moze spowodowac eradykacje zmian w pozaotrzewnowych wezlach chlonnych u dzieci powyzej 1 r.z. z neuroblastoma w 4 stopniu zaawansowania?

The prognosis in patients over 1 year of age with stage 4 neuroblastoma (NBL) is poor in spite of intensive treatment. The choice of intensive chemotherapy and extension of the surgery, especially in the case when the tumor is localized in the abdomen, is still controversial. Between 1991-2001 there were 61 patients with NBL treated in our Department; 28 of them were older than 1 year and had stage 4 with the tumor localized in the abdomen. All children received intensive chemotherapy according to Japanese protocol. Three children with disease progression during intensive treatment where excluded from further analysis. In 22 children with good or very good response to chemotherapy, the surgery was performed. In 4 patients it was local surgery. In 18 patients the tumor was removed with all visible retroperitoneal lymph nodes (so called Tsuchida surgery). The histopathological evaluation was performed in 17 patients with Tsuchida surgery and in 13 cases metastases in retroperitoneal lymph nodes were found. The 5-year overall survival was significantly better in the group with Tsuchida surgery performed (0.66 vs 0.21, p=0.044) for the patients in stage 4. After the intensive induction of chemotherapy the metastases in retroperitoneal lymph nodes can still be found. The removal of the tumor with all retroperitoneal lymph nodes can improve treatment results in patients with advanced abdominal neuroblastoma, but it is necessary to investigate more patients and to prolong the observation period.

[Peritoneal drainage as an alternative to laparotomy in premature infants with complicated necrotizing enterocolitis]. / Drenaz otrzewnowy u wczesniaków z ciezkimi powiklaniami martwiczego zapalenia jelita jako alternatywa agresywnego leczenia chirurgicznego.

UNLABELLED: The highest mortality due to necrotizing enterocolitis (NEC) in noted among low birth weight infants. Poor general medical condition of those children does not allow for major surgery despite obvious symptoms of perforation that usually require laparotomy. The aim of this study was assessment of the outcome of peritoneal drainage in complicated NEC in low birth weight infants. MATERIAL AND METHODS: Peritoneal drainage was employed in the treatment of fifteen children with perforated NEC between 1995 and 2002. Mean gestational age of studied newborns was 28.2 weeks, mean birth weight 1178 g (670-2540 g). RESULTS: Eight children survived. Their mean gestational age was 26.8 weeks, mean birth weight--876 g. Seven children died 1-15 days after the perforation. Their mean gestational age was 29.9 weeks, mean birth weight 1227 g. CONCLUSIONS: Survival of low birth weight infants with perforated NEC treated by the peritoneal drainage was 53.3%. The result doesn't seem to be very good unless we remember that before introduction of peritoneal drainage those children would probably die during surgery. Taking in account positive opinions about this method we may conclude that the use of peritoneal drainage could be extended to more patients with complications of NEC and should not be reserved for "hopeless" cases only.