Comparative retrospective study on the modalities of biopsying peripheral neuroblastic tumors: a report from the Italian Pediatric Surgical Oncology Group (GICOP)

Background: Peripheral neuroblastic tumors are the most common extracranial solid neoplasms in children. Early and adequate tissue sampling may speed up the diagnostic process and ensure a prompt start of optimal treatment whenever needed. Different biopsy techniques have been described. The purpose of this multi-center study is to evaluate the accuracy and safety of the various examined techniques and to determine whether a preferential procedure exists. Methods: All children who underwent a biopsy, from January 2010 to December 2014, as a result of being diagnosed with a peripheral neuroblastic tumor, were retrospectively reviewed. Data collected included patients’ demographics, clinical presentation, intraoperative technical details, postoperative parameters, complications, and histology reports. The Mann–Whitney U and Fisher's exact tests were used for statistical analysis. Results: The cohort included 100 patients, 32 of whom underwent an incisional biopsy (performed through open or minimally invasive access) (Group A), and the remaining 68 underwent multiple needle-core biopsies (either imaging-guided or laparoscopy/thoracoscopy-assisted) (Group B). Comparing the two groups revealed that Group A patients had a higher rate of complications, a greater need for postoperative analgesia, and required red blood cell transfusion more often. Overall adequacy rate was 94%, without significant differences between the two groups (100% vs. 91.2% for Group A and Group B, respectively, P = 0.0933). Conclusions: Both incision and needle-core biopsying methods provided sub-optimal to optimal sampling adequacy rates in children affected by peripheral neuroblastic tumors. However, the former method was associated with a higher risk of both intraoperative and postoperative complications compared with the latter

Mature and immature teratoma: A report from the second Italian pediatric study

none16noBACKGROUND: Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. PROCEDURE: Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. RESULTS: The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. CONCLUSIONS: Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.noneTerenziani M;D'Angelo P;Inserra A;Boldrini R;Bisogno G;Babbo GL;Conte M;Dall' Igna P;De Pasquale MD;Indolfi P;Piva L;Riccipetitoni G;Siracusa F;Spreafico F;Tamaro P;Cecchetto GTerenziani, M; D'Angelo, P; Inserra, A; Bisogno, Gianni; Bisogno, G; Babbo, Gl; Conte, M; Dall' Igna, P; De Pasquale, Md; Indolfi, P; Piva, L; Riccipetitoni, G; Siracusa, F; Spreafico, F; Tamaro, P; Cecchetto, Giovann

Mature and Immature Teratoma: A Report From the Second Italian Pediatric Study

Background. Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the ®rst Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. Procedure. Patients with teratoma were collected from 2004 to 2014. Teratomas were classi®ed according to the WHO classi®cations, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. Results. The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. Conclusions. Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses

Transitional Care for Patients with Congenital Colorectal Diseases: An EUPSA Network Office, ERNICA, and eUROGEN Joint Venture

Background: Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD. Methods: A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs. Recruitment of centres was done by the ERNs and EUPSA, using mailing lists and social media accounts. Only descriptive statistics were reported. Results: In total, 82 centres from 21 different countries entered the survey. Approximately half of them were ERN network members. Seventy-two centres (87.8%) had a self-reported area of expertise for both ARM and HD. Specific TOC programs were installed in 44% of the centres and AC programs in 31% of these centres. When comparing centres, wide variation was observed in the content of the programs. Conclusion: Despite the awareness of the importance of TOC and AC programs, these programs were installed in less than 50% of the participating centres. Various transition and AC programs were applied, with considerable heterogeneity in implementation, content and responsible caregivers involved. Sharing best practice examples and taking into account local and National Health Care Programs might lead to a better continuation of care in the future. Level of Evidence: III

Long term follow-up and transition of care in anorectal malformations: An international survey

Background: The aim of the study was to assess current international practice in the long termfollow-up, in managing active problems and transition of care for teenagers born with anorectal malformations (ARM).Methods: An original survey was administered to delegates attending two large colorectal surgical meetings in 2015. The 21 questions covered long term follow-up, specific issues for teenagers and transition of care.Results: 96/236 delegates completed the survey. Follow-up was routinely suspended before 10 year of age by 33% of respondents. 90% of them did not use a scoring system to assess or risks stratify patients, despite 81% stating that an objective score would be beneficial. 40% of respondents felt that > 30% of their teenagers had ongoing active medical or psychosocial issues. 42% thought their patients were not ready to be transitioned. The process of transition should start around 13-16 years according to 54% of respondents. 72% had no protocol for transition and 82% did not hold multidisciplinary meetings with adult practitioners before transition.Conclusions: International consensus on the following aspects of the care in ARM is needed: structured long term follow-up, objective assessment and risk stratification scores, pathways of transition and methods to prepare patients, parents and adult practitioners. (C) 2016 Elsevier Inc. All rights reserved

The Role of Video-Assisted Thoracoscopic Surgery in Pediatric Oncology: Single-Center Experience and Review of the Literature

Aim: Video-assisted thoracoscopic surgery (VATS) has been widely used in the last decades. Nevertheless, the pros and cons of thoracoscopy vs. open surgery in pediatric oncology are still under debate. In literature, VATS has been applied for both diagnostic and ablative surgery to treat neurogenic tumors, thymic neoplasms, lung tumors and metastases, germ cell tumors, lymphoproliferative diseases, and other rare tumors. Recent reviews described excellent outcomes in pediatric oncology as well as in the treatment of adult lung cancer, with a significantly higher rate of mortality and complication in thoracotomy compared to VATS. We reviewed our experience on thoracoscopy in pediatric malignancy and compared it to the literature. Materials and Methods: This was a retrospective cohort-study of pediatric oncological patients who underwent VATS at our institution from 2007 to 2020, and a review of the recent literature on the topic. Results: A total of 43 procedures were performed on 38 oncological patients (18 males, 20 females). Median age was years 7.72 (0.35–18.6). Diagnosis: 10 neurogenic tumors, nine hematological diseases, five metastases, four lypoblastomas, three thymic pathologies, three germ cell tumors, two pleuropneumoblastomas, two myofibroblastic tumors, one myoepithelial carcinoma, one liposarcoma, and three suspected oncological mass. In three cases, a 3D model was elaborated to better plan the surgical approach. Diagnostic biopsies were 22 (51.1%), and ablative surgeries, 21 (48.9%). One neurogenic tumor was resected with the Da Vinci Robot. Median operative time was 120 min (30–420). A drain was left in place in 20 (46.5%) for a median of 4 days. Median length of hospitalization was 5 days (1–18). One case (2.3%) was converted (intraoperative bleeding). There were three post-operative complications (7.0%): one pneumonia, one pleural effusion, and one diaphragmatic paralysis (need for plication). Results were compared to recent literature, and morbidity and conversion rate were comparable to reviewed publications. Conclusion: VATS represents a valuable tool for diagnostic and therapeutic procedures in pediatric oncology. Nonetheless, it is a challenging technique that should be performed by expert surgeons on oncological and mini-invasive surgery. Three-dimensional reconstruction can optimize the pre-operative planning and guarantee a safer and more targeted treatment. Finally, the advent of robotics-assisted surgery represents a new challenge that may further implement the advantages of VATS