Is fundoplication mandatory in children with neurological impairment undergoing gastrostomy?

AIM: Percutaneous endoscopic gastrostomy (PEG) and surgical gastrostomy (SG) are common procedures in children with neurological impairment (NI) with swallowing difficulties. Pulmonary aspirations are a major concern and performing concomitant or delayed fundoplication is still controversial, especially among these patients. The aim of our study was to review our experience with fundoplication performed concomitantly with gastrostomy or later and to evaluate patient outcomes. METHODS: This is a retrospective, cohort study including all paediatric patients who underwent SG or PEG with or without Nissen fundoplication at Schneider Children's Medical Center of Israel between the years 2007 and 2018. Patients' clinical and surgical data were recorded and analysed. RESULTS: Between 2007 and 2018, 345 patients underwent SG or PEG. Of these, 89 patients underwent fundoplication. Of the patients who underwent PEG/SG, 158 (45.8%) were neurologically impaired. Most of the patients who underwent fundoplication (n = 69, 77.5%) were NI patients (P = 0.0001). NI patients with refractory seizures showed almost no improvement in terms of relief of gastro-oesophageal reflux disease symptoms following fundoplication (P = 0.0001) compared to NI patients without refractory seizures. CONCLUSION: Our findings suggest that in NI patients a concomitant fundoplication is not mandatory and is not efficacious in preventing gastro-oesophageal reflux disease in patients with refractory seizures.

Blunt High-Grade Pancreatic Injury in Children: A 20-Year Experience in Two Pediatric Surgical Centers.

BACKGROUND: Pancreatic trauma is uncommon in pediatric patients and presents diagnostic and therapeutic challenges. While non-operative management (NOM) of minor pancreatic injuries is well accepted, the management of major pancreatic injuries remains controversial. OBJECTIVES: To evaluate management strategies for major blunt pancreatic injury in children. METHODS: Data were retrospectively collected for all children treated for grade III or higher pancreatic injury due to blunt abdominal trauma from 1992 to 2015 at two medical centers. Data included demographics, mechanism of injury, laboratory and imaging studies, management strategy, clinical course, operative findings, and outcome. RESULTS: The cohort included seven boys and four girls aged 4-15 years old (median 9). Six patients had associated abdominal (mainly liver, n=3) injuries. The main mechanism of injury was bicycle (handlebar) trauma (n=6). Five patients had grade III injury and six had grade IV. The highest mean amylase level was recorded at 48 hours after injury (1418 U/L). Management strategies included conservative (n=5) and operative treatment (n=6): distal (n=3) and central (n=1) pancreatectomy, drainage only (n=2) based on the computed tomography findings and patient hemodynamic stability. Pseudocyst developed in all NOM patients (n=5) and two OM cases, and one patient developed a pancreatic fistula. There were no differences in average length of hospital stay. CONCLUSIONS: NOM of high-grade blunt pancreatic injury in children may pose a higher risk of pseudocyst formation than OM, with a similar hospitalization time. However, pseudocyst is a relatively benign complication with a high rate of spontaneous resolution with no need for surgical intervention.

Does Maternal Omega 3 Supplementation Protect Against Infantile Hypertrophic Pyloric Stenosis?

OBJECTIVES: Infantile hypertrophic pyloric stenosis (IHPS) is potentially life threatening. The etiology of IHPS remains unknown and many risk factors have been reported. We aimed to assess the prevalence of known risk factors and investigate maternal nutrition and habits as possible additional risk factors for IHPS. METHODS: This case-control study includes mothers of infants diagnosed with IHPS and control mothers of infants, age 2 to 11 months, hospitalized in the pediatric department due to other conditions. Cases of IHPS were identified by review of all infants diagnosed with IHPS and operated upon in 2010 to 2016 at 2 major hospitals in central Israel. Data regarding potential risk factors were collected via questionnaires in both study groups. RESULTS: Sixty-six cases and 67 controls were included in the study. Maternal omega 3 supplement consumption during pregnancy was significantly less common among cases of IHPS as compared with controls (P = 0.031). Consumption of omega 3 supplement was defined as consumption of at least 1 to 2 per week during the pregnancy period. Following adjustment for known risk factors, including male sex and maternal smoking, maternal omega 3 supplement consumption remained associated with a significantly lower risk of developing IHPS (odds ratio = 0.303, 95% confidence interval 0.111-0.828, P = 0.02). CONCLUSIONS: Maternal omega 3 supplement consumption during pregnancy was associated with a significantly reduced risk of IHPS. Further studies are needed to support these results and investigate possible mechanisms of the effect of omega 3.

Novel extra cellular-like matrices to improve human ovarian grafting.

PURPOSE: To investigate if human ovarian grafting with pure virgin human recombinant collagen type-1 from bioengineered plant lines (CollPlant™) or small intestine submucosa (SIS) yields better implantation results for human ovarian tissue and which method benefits more when combined with the host melatonin treatment and graft incubation with biological glue + vitamin E + vascular endothelial growth factor-A. METHODS: Human ovarian tissue wrapped in CollPlant or SIS was transplanted into immunodeficient mice with/without host/graft treatment. The tissue was assessed by follicle counts (including atretic), for apoptosis evaluation by terminal deoxynucleotidyl transferase assay and for immunohistochemical evaluation of neovascularization by platelet endothelial cell adhesion molecule (PECAM) expression, and for identification of proliferating granulosa cells by Ki67 expression. RESULTS: Human ovarian tissue transplanted with CollPlant or SIS fused with the surrounding tissue and promoted neovascularization. In general, implantation with CollPlant even without additives promoted better results than with SIS: significantly higher number of recovered follicles, significantly fewer atretic follicles, and significantly more granulosa cell proliferation. Moreover, results with CollPlant alone seemed to be at least as good as those after host and graft treatments. CONCLUSIONS: CollPlant is a biomaterial without any potential risks, and grafting ovarian tissue with CollPlant is easy and the procedure may be easily modified, with limited or no foreseeable risks, for auto-transplantation in cancer survivors. Further studies are needed using other novel methods capable of enhancing neovascularization and reducing apoptosis and follicle atresia.

Parapet Along the Roof: Injury Prevention in the Context of an Ancient, But Still Relevant, Commandment.

BACKGROUND: Falling from a height accounts for 14.1% of all hospital admissions for traumatic injury. In 5% of cases, the injury is severe or critical, and in 1.5%, it is fatal. The dangers of falling have been recognized since time immemorial. Indeed, the Bible instructs us to build a parapet around the roof of our home so that, "…you may not bring the guilt of bloodshed on your house if someone falls from it" (Deuteronomy 22:8). This commandment highlights the relatively simple and practical means by which we can prevent falls. It is also one of a series of ethical laws that are presented to help us understand and obey the larger Biblical precepts of loving one's neighbor and guarding the sanctity of life. The concept teaches us that it is the responsibility of all individuals to be cognizant of others and to avoid harming people through negligence or carelessness. The aim of this article is to explain the commandment to build a parapet in the context of the risk of falling from a height and to expand on its wider implications. The present work was prompted in part by the alarming increase in fatal and near-fatal accidents in Israel in two particular populations.

Emergency or urgent splenectomy in children for non-traumatic reasons.

Emergency splenectomy is rarely performed since a widespread consensus exists towards conservative management of splenic injury. However, in selected conditions, mainly hematological, there is a role for emergency or urgent splenectomy. This study aims to retrospectively review these cases and discuss outcome in relation to the pre-existing splenic pathologies. Between 2000 and 2015, 12 patients, five girls, and seven boys, with a median age of six years (3 months-13.11 years), underwent emergency or urgent splenectomy for non-traumatic conditions. All patients had major associated disorders; mainly hematological (11 cases) including hemolytic anemia with pancytopenia (1), sickle cell anemia (1), AML (1), ALL (2), CML (1), T cell lymphoma (1), Burkitt lymphoma (1), and ITP (3). One patient had a microvillous inclusion disease. Indications for splenectomy included diffuse resistant splenic abscesses (4), intracranial hemorrhage (4) or hypersplenism (3) with refractory thrombocytopenia, and spontaneous splenic rapture (1). Nine patients improved following surgery but three died, owing to massive intracranial hemorrhage (1) and severe respiratory failure (2) despite aggressive management.Conclusions: Rarely, an emergency splenectomy is required in complex settings, mostly refractory hematological conditions, in a deteriorating patient when all other measurements have failed. A multidisciplinary team approach is mandatory in the treatment of these complex cases. What is known • Conservative treatment is advised for splenic injury. • Many hematological disorders are responsible of splenic pathology. What is new • Emergency splenectomy in children for reasons other than trauma is a treatment of last resort that should be performed in a multidisciplinary context. • The outcome of emergency splenectomy in children for reasons other than trauma depends on the underlying medical condition.

Perianal abscess in infants: Amenable to conservative treatment in selected cases.

BACKGROUND: Perianal abscess is a common surgical condition in daily pediatric practice. Management is a subject of controversy and a variety of approaches are practiced. While the most frequent approach is drainage with/without fistulotomy, the superiority of this approach and the place of conservative approach has not been established. The aim of this study was to evaluate the outcomes of conservative approach in selected cases of perianal abscesses in infants. METHODS: Data of 19 patients aged <24 months treated conservatively for perianal abscess at a tertiary hospital in 2014-2018 were retrospectively reviewed. RESULTS: Criteria for a conservative approach were: spontaneous drainage into the anal canal (n = 8) or perianal skin (n = 4), and phlegmonous infiltrate with fluid collection detected on ultrasound (n = 7). Mean age at symptom onset was 8.4 months. Twelve patients were managed for the first time. Previous care in seven patients included 1-4 drainage procedures (n = 4), spontaneous drainage (n = 1) and antibiotics (n = 2). Five patients were on oral antibiotics at presentation. After diagnosis, 18 patients received i.v. antibiotics and one, oral antibiotics. Three patients (15.7%) ultimately required surgical drainage; two were lost to follow up. During follow up (mean, 22.4 months) four patients (28.5%) had a single recurrent episode; abscess in three (managed conservatively in two and surgically in one) and fistula-in-ano in one patient that healed spontaneously. Thus, surgical intervention was prevented in 13/17 patients (76.4%) available for follow up. CONCLUSIONS: Perianal abscess in infants is amenable to conservative management in selected cases. Avoiding surgical intervention is advantageous, especially given the high recurrence rate.

Current management of lipoblastoma.

Lipoblastoma is a rare and benign tumor arising from embryonal fat cells. It is generally diagnosed in children younger than 3 years of age and can occur in the extremities or on the trunk. We present our series of 10 children with lipoblastoma treated at Schneider Children's Medical Center of Israel between 2011 and 2016. Six boys and four girls underwent tumor resection at a median age of 2 years and 3 months (range 5 months to 5.6 years). Locations were trunk (6), groin (2), perineum (1), and omentum (1). Follow up ranges from 1 to 5 years. Two patients had a local recurrence and required a second resection 2 years (perineal) and 6 years (trunk) after the first surgery without further recurrence at 1.9 and 2.9 years, respectively. CONCLUSION: Higher awareness of lipoblastoma enables optimal imaging strategies and resection. Long follow up is required due to local recurrences. The treatment of choice consists of complete, but non mutilating surgical resection. What is Known: • Lipoblastoma is a rare benign tumor of fatty tissue affecting children • Treatment consists of surgical resection What is New: • MRI is the modality of choice for follow up • Ten-year long-term follow up is required due to late recurrence.

Emergencies in the Treatment of Wandering Spleen.

BACKGROUND: Wandering spleen is a rare entity that may pose a surgical emergency following torsion of the splenic vessels, mainly because of a delayed diagnosis. Complications after surgery for wandering spleen may necessitate emergency treatment. OBJECTIVES: To describe the clinical course and treatment for children who underwent emergency surgeries for wandering spleen at a tertiary pediatric medical center over a 21 year period and to indicate the pitfalls in diagnosis and treatment as reflected by our experience and in the literature. METHODS: The database of a tertiary pediatric medical center was searched retrospectively for all children who underwent emergency treatment for wandering spleen between 1996 and 2017. Data were collected from the medical files. The relevant literature was reviewed. RESULTS: Of ten patients who underwent surgery for wandering spleen during the study period, five underwent seven emergency surgeries. One patient underwent surgery immediately at initial presentation. In the other four, surgical treatment was delayed either due to misdiagnosis or for repeated imaging studies to confirm the diagnosis. Emergency laparotomy revealed an ischemic spleen in all patients; splenectomy was performed in two and the spleen was preserved in three. Four of the seven emergency operations were performed as the primary surgery and three were performed to treat complications. CONCLUSIONS: Wandering spleen should ideally be treated on an elective or semi-elective basis. Surgical delays could be partially minimized by a high index of suspicion at diagnosis and by eliminating unnecessary and time-consuming repeated imaging studies.

Point-of-Care Ultrasound in a Department of Pediatric and Adolescent Surgery.

BACKGROUND: Point-of-care ultrasound (POCUS) is becoming a common tool for routine use in emergency medicine, anesthesiology and intensive care for diagnostic and interventional purposes. When a portable ultrasound device became available for the department of Pediatric and Adolescent Surgery at the Schneider's Children Medical Center of Israel, we added POCUS assessments to the physician's daily rounds. POCUS is performed by pediatric surgeons trained in basic ultrasonography skills. Starting September 2015 all POCUS examinations were documented. OBJECTIVES: To describe the current use, diagnostic and therapeutic impacts of POCUS in a department of pediatric and adolescent surgery. METHODS: We conducted an observational study of all the documented POCUS procedures performed during a half-year period. Data regarding patient condition and the POCUS procedures were collected, as well as data on the use of other diagnostic modalities, mainly formal ultrasound exams (by radiologists) and computed tomography scans and their correlation with the POCUS assessment. RESULTS: Fifty-one POCUS exams were performed during the study period, most of which served to define the presence and resolution of a collection - intraabdominal (34%) and subcutaneous (31%). Despite a high rate for formal diagnostic studies (65%), probably due to a relative lack of confidence of surgeons performing the POCUS exams during this initial period, most results (92%) were compatible. CONCLUSIONS: The ability and availability to perform multiple POCUS exams by the attending physician proved to be a valuable aide to the classical physical and laboratory examinations of surgical patients, and we predict its increasing use in quotidian practice.

Anorectal injuries in children: a 20-year experience in two centers.

INTRODUCTION: Anorectal injuries in children are not frequently reported and their management is challenging. This report reviews the experience in managing this type of injuries in two medical centers over 20 years. METHODS: An institutional database search for patients who were treated for anorectal injuries between 1994 and 2015 was undertaken. Twenty cases were located and medical records reviewed. This study was conducted with institutional review board approval (#572-14). RESULTS: There were 6 girls and 14 boys with ages ranging between 1 and 15 years (mean 7 years). Eleven patients sustained penetrating trauma, while nine sustained blunt trauma. The mechanism of injury was variable and associated injuries were more common in blunt trauma. Most common presenting symptoms were rectal bleeding (n = 12) and anal pain (n = 11), followed by abdominal pain in six patients. Eighteen anorectal injuries were extraperitoneal and two intraperitoneal. Among patients with extraperitoneal injuries, 12/18 were managed by primary repair with (6) or without (6) fecal diversion and 2/18 by wound irrigation and drainage with fecal diversion and delayed repair. Four patients had superficial anal and perineal injuries that were irrigated and left to heal by secondary intention. Two patients with intraperitoneal rectal injuries underwent primary repair with fecal diversion. Follow-up period ranged from 2 weeks to 8 years (mean 2 years). There were three cases of wound infection, one case of suture line leak requiring reoperation and one case of vesicorectal fistula in a patient with combined trauma of the rectum and urinary bladder. There was no mortality. Fecal continence was preserved in all patients available for follow-up evaluation. CONCLUSIONS: Primary repair of the perineal wound and anal sphincters can be performed safely in most cases given hemodynamic stability. Fecal diversion should be saved for cases with severe perineal involvement or cases with substantial associated injuries and concern of gross contamination.

Possible improvements in human ovarian grafting by various host and graft treatments.

BACKGROUND: Anticancer treatment poses a high risk of ovarian failure. In many cases cryopreservation of ovarian tissue is the only option for fertility preservation. Although autologous transplantation of cryopreserved-thawed ovarian tissue has resulted in live births, slow graft revascularization and ischemia after transplantation leads to substantial follicular loss. Therefore, methods to improve and hasten graft vascularization are needed. The aim of the study was to examine the benefits of host and graft treatments with melatonin, hyaluronan (HA), vascular endothelial growth factor A (VEGF-A) and vitamin E with regard to the outcome of human ovarian tissue grafting. METHODS: Five young cancer patients who underwent laparoscopic ovarian surgery for fertility preservation donated ovarian tissue. Thawed ovarian samples were transplanted into immunodeficient mice divided into seven groups: (A) no treatment; (B) host treatment with melatonin before and after grafting; (C) graft incubation with HA-rich biological glue before transplantation; (D) host as in (B), graft as in (C); (E) host as in (B), graft incubation with VEGF-A and vitamin E; (F) graft as in (C) combined with VEGF-A and vitamin E; (G) host as in (B), graft as in (F). Graft survival was assessed by follicle counts, apoptosis assay and immunohistochemical staining for proliferating cell nuclear antigen and VEGF-A expression. RESULTS: Only grafts implanted in melatonin-treated hosts and grafts incubated with HA-rich biological glue retained their original size. Apoptosis was significantly lower after host treatment with melatonin and graft incubation with HA-rich biological glue plus VEGF-A and vitamin E than in untreated grafts; apoptosis was specifically low in Group G. There were significantly more atretic follicles in the untreated group than in most treated groups. CONCLUSIONS: The findings suggest that host treatment with melatonin or graft incubation with HA-rich biological glue, especially when combined with VEGF-A and vitamin E improves graft survival. This protocol can be applied and holds promise in ovarian autotransplantation for fertility restoration.

Spinal anesthesia for noncardiac surgery in infants with congenital heart diseases.

OBJECTIVE/AIM: To compare hemodynamic parameters in infants with congenital heart disease (CHD) undergoing noncardiac surgery (NCS) under awake spinal anesthesia (SA) with controls without CHD also undergoing SA. BACKGROUND: NCS poses a twofold higher mortality risk in infants with CHD. SA might be a good alternative to general anesthesia (GA) in this setting. METHODS: The files of 84 infants were reviewed; 42 had CHD and 42 were controls without CHD. Primary outcome measures were percent decrease in mean arterial pressure (%MAP decrease) and heart rate (%HR decrease)from baseline to the lowest intraoperative value. One-way analysis of variance(ANOVA), ANOVA with repeated measures, Pearson chi-square test, Fisher's exact test, and Pearson correlation were used for statistical analysis.Time to discharge was analyzed with the nonparametric Mann­Whitney U-test. RESULTS: There were no significant between-group differences in %MAP decrease and %HR decrease; no significant associations and correlations between %MAP decrease or %HR decrease and other variables; and no correlation between %MAP decrease and %HR decrease. A %MAP decrease of >20% was documented in 11 patients with CHD (26.2%) and 10 controls (23.8%); a lowest intraoperative HR of <100 b.min)1 was recorded in two study patients (4.8%) and four controls (9.5%) (P = NS for both). There were no cases of high SA or conversion to GA and no need for mechanical ventilation or inotropic support intra/postoperatively. CONCLUSIONS: These preliminary findings show that hemodynamic parameters in infants with CHD undergoing NCS under awake SA are not different from controls without CHD and that SA appears to be safe in infants with CHD.

Embryonic pig pancreatic tissue for the treatment of diabetes in a nonhuman primate model.

Xenotransplantation of pig tissues has great potential to overcome the shortage of organ donors. One approach to address the vigorous immune rejection associated with xenotransplants is the use of embryonic precursor tissue, which induces and utilizes host vasculature upon its growth and development. Recently, we showed in mice that embryonic pig pancreatic tissue from embryonic day 42 (E42) exhibits optimal properties as a beta cell replacement therapy. We now demonstrate the proof of concept in 2 diabetic Cynomolgus monkeys, followed for 393 and 280 days, respectively. A marked reduction of exogenous insulin requirement was noted by the fourth month after transplantation, reaching complete independence from exogenous insulin during the fifth month after transplantation, with full physiological control of blood glucose levels. The porcine origin of insulin was documented by a radioimmunoassay specific for porcine C-peptide. Furthermore, the growing tissue was found to be predominantly vascularized with host blood vessels, thereby evading hyperacute or acute rejection, which could potentially be mediated by preexisting anti-pig antibodies. Durable graft protection was achieved, and most of the late complications could be attributed to the immunosuppressive protocol. While fine tuning of immune suppression, tissue dose, and implantation techniques are still required, our results demonstrate that porcine E-42 embryonic pancreatic tissue can normalize blood glucose levels in primates. Its long-term proliferative capacity, its revascularization by host endothelium, and its reduced immunogenicity, strongly suggest that this approach could offer an attractive replacement therapy for diabetes.

Laparoscopic-assisted retrieval of migrated intra-abdominal pacemaker generator.

A 5-year-old boy born with a congenital heart defect had insertion of an epicardial pacemaker that was found on routine evaluation to have been displaced into the rectovesical pouch. He underwent transvenous insertion of another pacemaker, and the displaced pacemaker was successfully retrieved by laparoscopy without incident.

Alginate scaffold for organ culture of cryopreserved-thawed human ovarian cortical follicles.

PURPOSE: To compare macroporous alginate scaffolds with Matrigel for culturing frozen-thawed human primordial follicles in organ culture. METHODS: Twelve girls/women donated ovarian tissue. One tissue sample was fixed immediately after thawing (uncultured samples). Slices were cultured for 2 weeks on either Matrigel or on alginate scaffolds with a serum-free culture medium. Growth evaluation consisted of follicular counts and classification, immunohistochemistry and measurement of 17ß-Estradiol (E(2)) production. RESULTS: The number of developing follicles was significantly higher in alginate scaffold-cultured samples than on Matrigel with a concomitant decrease in the number of primordial follicles in alginate scaffold-cultured samples than uncultured samples. The number of atretic follicles after 1 week was significantly higher in the Matrigel-cultured samples than in the alginate scaffold cultured samples. E(2) production was similar in both groups. CONCLUSIONS: Three dimensional alginate scaffolds are a promising putative in vitro technology for developing human primordial follicles.

A case study of virilizing adrenal tumor in an adolescent female elite tennis player--insight into the use of anabolic steroids in young athletes.

A 14-year-old Caucasian girl was referred to the endocrine clinic for evaluation of voice deepening, facial hirsutism, and acne starting 2 years previously. She had been a competitive tennis player since age 7 years, practicing for 4-6 hours daily. On physical examination she was noticed to have a masculine appearance with mild facial acne and moderate hirsutism. Tanner stage was 1 for breast tissue and 5 for pubic hair. Her androgen levels (testosterone, androstenedione, dehydroepiandrosterone sulfate) were extremely elevated. Adrenal ultrasonography revealed a round left 4.6 × 5.3-cm adrenal mass. Laparoscopic left adrenalectomy was performed. The histologic findings were compatible with a benign adrenocortical tumor. Postoperatively, androgen levels dropped to within the normal range. Breast development proceeded normally, menarche occurred 2 months after tumor resection, and menses has been regular since then. Muscle strength of the dominant and nondominant upper and lower extremities was measured 1 month before surgery and 1 year later, using an isokinetic dynamometer (Biodex Systems II, Biodex, Shirley, NY, USA). There was no significant decrease in overall muscle strength after removal of the virilizing tumor and the marked drop in circulating androgens. In addition, the patient maintained her age category, number 1, national tennis ranking. The results suggest that even extremely high levels of tumor-related circulating androgens had no evident effect on muscle strength and competitive performance in a female adolescent tennis player. The lack of beneficial effect on performance in adolescents, combined with the potentially hazardous side effects of anabolic steroids, suggests that teenage athletes should avoid their use.

Spinal anesthesia in a child with Brachmann-de Lange (Cornelia de Lange) syndrome.

Providing anesthesia to patients with Brachmann-de Lange syndrome (BdLS) may be challenging, mainly because of intubation difficulty, gastroesophageal reflux disease, and aspiration complications. The use of spinal anesthesia (SA) in this population has not been reported. We report the uneventful administration of awake SA to a 7-month-old girl with BdLS who was scheduled for rectal biopsy. The current literature is reviewed to discuss the indications for SA in those patients.

Pediatric and Adolescent Surgical Breast Clinic: Preliminary Experience.

STUDY OBJECTIVE: The diagnostic and treatment tools used in breast centers are largely geared to adults, and there is little consideration of the unique characteristics of breast diseases in younger age groups. Herein we report on the preliminary experience of a specialized breast clinic for children and adolescents. DESIGN: Retrospective, observational. SETTING: Pediatric Surgical Breast Clinic of Schneider Children's Medical Center of Israel. PARTICIPANTS: Patients referred to the clinic during the first 18 months of its establishment. INTERVENTIONS AND MAIN OUTCOME MEASURES: Rate of breast masses, rate of malignancy, and types of evaluation and treatment. RESULTS: Forty-seven patients aged 0-19 years were referred. Breast masses were suspected in 23/47 (48.9%) and confirmed using ultrasound in 14/47 (29.7%; mean age, 16.4 years), followed by needle core biopsy in 7. Seven patients had multiple masses. Breast Imaging and Reporting Data System scores ranged from 3 to 4b. All cases were treated as fibroadenomas. Four patients underwent surgery because of a large mass (>5 cm; n = 3) or patient's preference to remove the lump rather than follow-up (n = 1). There were no malignancies. The median time from patient identification of the mass until seeking medical help was 5 months. CONCLUSION: Several findings distinct to this age group were highlighted: high rate of multiple masses, lack of malignancy, and high risk of delayed diagnosis. Care should be taken when using Breast Imaging and Reporting Data System categorization because it was formulated for adults and might be very limited in the adolescent population. Longer-term studies of larger cohorts are planned.

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.