Comparison of Pediatric and Adult Solid Pseudopapillary Neoplasms of the Pancreas.

BACKGROUND: Solid pseudopapillary neoplasms (SPPNs) comprise the majority of pediatric pancreatic neoplasms. We queried the National Cancer Database to compare pediatric and adult patients with SSPNs to examine differences in demographics, tumor characteristics, treatment, and overall survival. We aimed to determine if survival differences existed between adult and pediatric patients with SPPN. METHODS: The National Cancer Database (2004-2014) was reviewed, and patients were stratified by age at diagnosis: pediatric (≤21 y) and adult (≥22 y). Demographics, comorbidities, tumor characteristics, diagnostic periods, treatments, and survival rates were compared using pooled variance t-tests and chi-square, followed by multivariate Cox proportional hazard model (α = 0.05). Log-rank test was used to compare survival. RESULTS: A total of 468 patients were analyzed and categorized according to age group. Four hundred and fourteen patients were included in the survival analysis. The pediatric patients were primarily female, Caucasian, had no comorbidities, and presented with stage I disease. Race/ethnicity, gender, socioeconomic status, comorbidities, and disease stage at presentation were similar between the groups. There was no difference in time to initiation of therapy or to surgical intervention. No significant difference was found in type of surgical resection, chemotherapy, or radiotherapy utilization. Despite the similarities between groups, comparison of overall survival demonstrated improved survival of pediatric SPPN compared with adult SPPN in every pathologic stage. CONCLUSIONS: These results suggest that pediatric and adult SPPNs are similar with regards to demographics, tumor characteristics, and treatment modalities. However, survival was better in children with SPPNs, which may be due to differences in tumor biology and may serve for risk stratification of prognosis.

Pancreatic islet cell tumors in adolescents and young adults.

BACKGROUND: Pancreatic islet cell tumors are rare in adolescents, and most studies published to date focus on older patients. We utilized a national database to describe the histology and clinical pattern of pancreatic islet cell tumors in adolescent and young adult (AYA) patients, and to compare AYAs to older adults. We hypothesized that AYAs with pancreatic islet cell tumors would have better overall survival. METHODS: The National Cancer Data Base (NCDB, 1998-2012) was queried for AYA patients (15-39 years) with a pancreatic islet cell tumor diagnosis. Demographics, tumor characteristics, treatment modalities, and outcomes were abstracted and compared to adults (≥40 years). RESULTS: 383 patients (56.4% female, 65% non-Hispanic Whites) were identified, with a median age of 27 (IQR 16-34) years. Islet cell carcinoma was the most common histology. Of patients with known stage of disease, 49% presented with early stage (I or II). Seventy percent of patients underwent surgical resection, including local excision 44%, Whipple procedure 37.5%, or total pancreatectomy 19%. Chemotherapy was utilized in 27% and radiotherapy in 7%. All-cause mortality was 36%. AYA patients underwent more extensive resections (p = 0.001) and had lower mortality rates (p < 0.001), with no differences in tumor stage or use of adjuvant therapies, when compared to adults. CONCLUSIONS: AYA patients with pancreatic islet cell tumors had comparable utilization of adjuvant therapies but underwent more extensive resections and demonstrated a higher overall survival rate than adult counterparts. Further investigation into approaches to earlier diagnosis and tailoring of multimodality therapy of these neoplasms in the AYA population is needed. LEVELS OF EVIDENCE: Prognostic Study, Level II - retrospective study.

Comparison of diagnostic imaging modalities for the evaluation of pancreatic duct injury in children: a multi-institutional analysis from the Pancreatic Trauma Study Group.

PURPOSE: Determining the integrity of the pancreatic duct is important in high-grade pancreatic trauma to guide decision making for operative vs non-operative management. Computed tomography (CT) is generally an inadequate study for this purpose, and magnetic resonance cholangiopancreatography (MRCP) is sometimes obtained to gain additional information regarding the duct. The purpose of this multi-institutional study was to directly compare the results from CT and MRCP for evaluating pancreatic duct disruption in children with these rare injuries. METHODS: Retrospective study of data obtained from eleven pediatric trauma centers from 2010 to 2015. Children up to age 18 with suspected blunt pancreatic duct injury who had both CT and MRCP within 1 week of injury were included. Imaging findings of both studies were directly compared and analyzed using descriptive statistics, Chi square, Wilcoxon rank-sum, and McNemar's tests. RESULTS: Data were collected for 21 patients (mean age 7.8 years). The duct was visualized more often on MRCP than CT (48 vs 5%, p < 0.05). Duct disruption was confirmed more often on MRCP than CT (24 vs 0%), suspected based on secondary findings equally (38 vs 38%), and more often indeterminate on CT (62 vs 38%). Overall, MRCP was not superior to CT for determining duct integrity (62 vs 38%, p = 0.28). CONCLUSIONS: In children with blunt pancreatic injury, MRCP is more useful than CT for identifying the pancreatic duct but may not be superior for confirmation of duct integrity. Endoscopic retrograde cholangiogram (ERCP) may be necessary to confirm duct disruption when considering pancreatic resection. LEVEL OF EVIDENCE: III.

Disparities in fertility-sparing surgery in adolescent and young women with stage I ovarian dysgerminoma.

BACKGROUND: In many cancers, racial and socioeconomic disparities exist regarding the extent of surgery. For ovarian dysgerminoma, fertility-sparing (FS) surgery is recommended whenever possible. The aim of this study was to investigate rates of FS versus non-fertility-sparing (NFS) procedures for stage I ovarian dysgerminoma in adolescents and young adults (AYAs) by ethnicity/race and socioeconomic status. MATERIALS AND METHODS: The National Cancer Data Base was queried for patients with ovarian dysgerminoma from 1998 to 2012. After selecting patients aged 15-39 y with stage I disease, a multivariate regression analysis was performed, and rates of FS and NFS procedures were compared, first according to ethnicity/race, and then by socioeconomic surrogate variables. RESULTS: Among the 687 AYAs with stage I ovarian dysgerminoma, there was no significant difference in rates of FS and NFS procedures based on ethnicity/race alone (P = 0.17), but there was a significant difference in procedure type for all three socioeconomic surrogates. The uninsured had higher NFS rates (30%) than those with government (21%) or private (19%) insurance (P = 0.036). Those in the poorest ZIP codes had almost twice the rate of NFS procedures (31%) compared with those in the most affluent ZIP codes (17%). For those in the least-educated regions, 24% underwent NFS procedures compared to 14% in the most-educated areas (P = 0.027). CONCLUSIONS: AYAs with stage I ovarian dysgerminoma in lower socioeconomic groups were more likely to undergo NFS procedures than those in higher socioeconomic groups, but there was no difference in rates of FS versus NFS procedures by ethnicity/race. Approaches aimed at reducing socioeconomic disparities require further examination.

Testicular tumors in prepubescent patients.

BACKGROUND/PURPOSE: Pediatric testicular tumors are rare, constituting only 1% of all pediatric solid tumors. Single-institution studies addressing pediatric testicular tumors published to date have been limited in the number of patients. METHODS: We utilized the National Cancer Data Base (1998-2012) to review all prepubescent patients (≤12 years old) with testicular neoplasms. Demographics, tumor characteristics, treatment modalities, and outcomes were abstracted. RESULTS: A total of 479 patients were identified, with a median age of 3 years (IQR 0-4) at diagnosis. 67% of cases were diagnosed by 3 years of age. Yolk sac tumors were the most common histology (202 patients, 42.2%). Most tumors were diagnosed at a low stage. Resection was performed in 465 boys, with 75% having undergone radical orchiectomies. Chemotherapy was utilized in 28% of cases and radiotherapy in 7%. With mean follow-up of 5.6 years, mortality rate was 3%. No difference in mortality was noted based on histology or extent of surgical resection. CONCLUSIONS: This series of prepubertal testicular tumors is the largest yet reported and highlights the patient demographics, tumor characteristics, treatment modalities and outcomes for these tumors. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: II.

Socioeconomic disparities affect survival in malignant ovarian germ cell tumors in AYA population.

BACKGROUND: Malignant ovarian germ cell tumors (MOGCTs) are a rare form of ovarian malignancy. Socioeconomic status (SES) has been shown to affect survival in several gynecologic cancers. We examined whether SES impacted survival in adolescent and young adults (AYAs) with MOGCT. MATERIALS AND METHODS: The National Cancer Data Base was used to identify AYAs (aged 15-39 years) with MOGCT from 1998 to 2012. Three SES surrogate variables identified were as follows: insurance type, income quartile, and education quartile. Pooled variance t-tests and chi-square tests were used to compare tumor characteristics, the time from diagnosis to staging/treatment, and clinical outcome variables for each SES surrogate variable, while controlling for age and race/ethnicity in a multivariate model. Kaplan-Meier survival estimates were calculated using the log-rank test. RESULTS: A total of 3125 AYAs with MOGCT were identified. Subjects with lower SES measures had higher overall stage and T-stage MOGCTs at presentation. There was no significant difference in the time to staging/treatment, extent of surgery, or use of chemotherapy by SES. Subjects from a lower education background, from a lower income quartile, and without insurance had decreased survival (P ≤ 0.02 for all). Controlling for overall stage and T-stage, the difference in survival was no longer significant. CONCLUSIONS: AYAs with MOGCT from lower SES backgrounds presented with more advanced stage disease. Further studies that focus on the underlying reasons for this difference are needed to address these disparities.

Open versus laparoscopic approach to gastric fundoplication in children with cardiac risk factors.

BACKGROUND: Gastric fundoplication is the most common noncardiac operation in children with congenital cardiac disease. While prior studies validated safety of laparoscopy in this population, we hypothesize that children with cardiac risk factors (CRFs) are likelier to undergo open fundoplication (OF) but experience greater morbidity than after laparoscopic fundoplication (LF). MATERIALS AND METHODS: Utilizing 2013 National Surgical Quality Improvement Program-Pediatrics Public-Use-File, pediatric patients undergoing LF and OF were stratified to none, minor, major, or severe CRFs. Multivariate logistic regression determined preoperative variables and postoperative outcomes associated with LF or OF. RESULTS: A total of 1501 fundoplication patients were identified with 92% undergoing LF. OF patients were likelier to have minor (odds ratio [OR]: 2.36, P < 0.001), major (OR: 2.41, P = 0.003), and severe CRFs (OR: 4.36, P < 0.001). Children ≤ 1 y (OR: 3.38, P = 0.048) and those with tracheostomy were likelier to have OF (OR: 2.3, P = 0.006). Overall, the OF group had higher postoperative morbidity (OR: 2.41, P < 0.001). Specifically, children with minor or major CRFs experienced more complications following OF compared to LF. CONCLUSIONS: OF is more common in patients ≤1 y old; patients with minor, major, or severe CRFs; and those with tracheostomy. LF should be considered in children with minor and major CRFs, as OF in those patients results in greater pulmonary, infectious, and hematological sequelae.

Proposed clinical pathway for nonoperative management of high-grade pediatric pancreatic injuries based on a multicenter analysis: A pediatric trauma society collaborative.

BACKGROUND: Guidelines for nonoperative management (NOM) of high-grade pancreatic injuries in children have not been established, and wide practice variability exists. The purpose of this study was to evaluate common clinical strategies across multiple pediatric trauma centers to develop a consensus-based standard clinical pathway. METHODS: A multicenter, retrospective review was conducted of children with high-grade (American Association of Surgeons for Trauma grade III-V) pancreatic injuries treated with NOM between 2010 and 2015. Data were collected on demographics, clinical management, and outcomes. RESULTS: Eighty-six patients were treated at 20 pediatric trauma centers. Median age was 9 years (range, 1-18 years). The majority (73%) of injuries were American Association of Surgeons for Trauma grade III, 24% were grade IV, and 3% were grade V. Median time from injury to presentation was 12 hours and median ISS was 16 (range, 4-66). All patients had computed tomography scan and serum pancreatic enzyme levels at presentation, but serial enzyme level monitoring was variable. Pancreatic enzyme levels did not correlate with injury grade or pseudocyst development. Parenteral nutrition was used in 68% and jejunal feeds in 31%. 3Endoscopic retrograde cholangiopancreatogram was obtained in 25%. An organized peripancreatic fluid collection present for at least 7 days after injury was identified in 59% (42 of 71). Initial management of these included: observation 64%, percutaneous drain 24%, and endoscopic drainage 10% and needle aspiration 2%. Clear liquids were started at a median of 6 days (IQR, 3-13 days) and regular diet at a median of 8 days (IQR 4-20 days). Median hospitalization length was 13 days (IQR, 7-24 days). Injury grade did not account for prolonged time to initiating oral diet or hospital length; indicating that the variability in these outcomes was largely due to different surgeon preferences. CONCLUSION: High-grade pancreatic injuries in children are rare and significant variability exists in NOM strategies, which may affect outcomes and effective resource utilization. A standard clinical pathway is proposed. LEVEL OF EVIDENCE: Therapeutic/care management, level V (case series).

Effects of socioeconomic status on children with well-differentiated thyroid cancer.

BACKGROUND: Well-differentiated thyroid cancer is the most common endocrine malignancy in children. Adult literature has demonstrated socioeconomic disparities in patients undergoing thyroidectomy, but the effects of socioeconomic status on the management of pediatric well-differentiated thyroid cancer remains poorly understood. METHODS: Patients ≤21 years of age with well-differentiated thyroid cancer remains were reviewed from the National Cancer Data Base. Three socioeconomic surrogate variables were identified: insurance type, median income, and educational quartile. Tumor characteristics, diagnostic intervals, and clinical outcomes were compared within each socioeconomic surrogate variable. RESULTS: A total of 9,585 children with well-differentiated thyroid cancer remains were reviewed. In multivariate analysis, lower income, lower educational quartile, and insurance status were associated with higher stage at diagnosis. Furthermore, lower income quartile was associated with a longer time from diagnosis to treatment (P < .002). Similarly, uninsured children had a longer time from diagnosis to treatment (28 days) compared with those with government (19 days) or private (18 days) insurance (P < .001). Despite being diagnosed at a higher stage and having a longer time interval between diagnosis and treatment, there was no significant difference in either overall survival or rates of unplanned readmissions based on any of the socioeconomic surrogate variables. CONCLUSION: Children from lower income families and those lacking insurance experienced a longer period from diagnosis to treatment of their well-differentiated thyroid cancer remains. These patients also presented with higher stage disease. These data suggest a delay in care for children from low-income families. Although these findings did not translate into worse outcomes for well-differentiated thyroid cancer remains, future efforts should focus on reducing these differences.

Survey on Robot-Assisted Surgical Techniques Utilization in US Pediatric Surgery Fellowships.

INTRODUCTION: Robotic technology has transformed both practice and education in many adult surgical specialties; no standardized training guidelines in pediatric surgery currently exist. The purpose of our study was to assess the prevalence of robotic procedures and extent of robotic surgery education in US pediatric surgery fellowships. MATERIALS AND METHODS: A deidentified survey measured utilization of the robot, perception on the utility of the robot, and its incorporation in training among the program directors of Accreditation Council for Graduate Medical Education (ACGME) pediatric surgery fellowships in the United States. RESULTS: Forty-one of the 47 fellowship programs (87%) responded to the survey. While 67% of respondents indicated the presence of a robot in their facility, only 26% reported its utilizing in their surgical practice. Among programs not utilizing the robot, most common reasons provided were lack of clear supportive evidence, increased intraoperative time, and incompatibility of instrument size to pediatric patients. While 58% of program directors believe that there is a future role for robotic surgery in children, only 18% indicated that robotic training should play a part in pediatric surgery education. Consequently, while over 66% of survey respondents received training in robot-assisted surgical technique, only 29% of fellows receive robot-assisted training during their fellowship. CONCLUSIONS: A majority of fellowships have access to a robot, but few utilize the technology in their current practice or as part of training. Further investigation is required into both the technology's potential benefits in the pediatric population and its role in pediatric surgery training.

Closure of a Traumatic Esophagomediastinal Fistula in a Child by Endoscopic Fulguration and Fibrin Injection.

Posttraumatic esophagomediastinal fistula is an uncommon clinical entity that warrants surgical awareness due to its life-threatening potential. Its management, especially in previously operated field, is controversial and several endoscopic methods are being proposed as alternatives. Ours is the first report of endoscopic fulguration and fibrin injection in successful closure of such fistula. A 9-year-old female sustained complete tracheoesophageal transection from a gunshot wound to the neck and underwent immediate primary repair. She presented nine months later with fevers and swelling over anterior neck. CT revealed air tracking posteriorly to the dorsal neck and inferiorly to the mediastinum. Considering difficulty of open surgical approach, endoscopic intervention was attempted. Posterior wall fistula was identified via microlaryngoscopy above the esophageal anastomosis. The fistula tract was de-epithelialized via a Bugbee fulgurating electrode and then sealed with fibrin glue. Consequent imaging studies demonstrated complete occlusion of the fistula. Posterior posttraumatic esophagomediastinal fistula presents a challenging scenario from a surgical standpoint, as it combines difficulty of safe approach, high rate of injury to surrounding structures, and significant postoperative recurrence rate. Endoscopic Bugbee fulguration and fibrin glue injection are a safe and effective alternative to the traditional approach.

Ileocolic and Jejunal Intussusceptions Requiring Surgical Intervention in a Pediatric Patient with Posttransplant Lymphoproliferative Disorder.

Posttransplant lymphoproliferative disorder (PTLD) is not uncommon in pediatrics. This case report describes a case of intestinal intussusception that occurred secondary to lymphoid hyperplasia in a child with PTLD following a cardiac transplant. This case was unique in its presentation with multiple areas of intussusception and with need for surgical intervention. The diagnosis of intussusception secondary to lead points from PTLD should be strongly considered in pediatric transplant patients presenting with abdominal complaints.

Difficult extraction of long-term central venous catheters in children--case report.

Although many publications address the complications associated with insertion of long-term, indwelling central venous catheters, there are only scattered reports of problems related to their removal. We report 4 cases of pediatric patients in whom the lines could not be pulled from the vein by standard methods, requiring 2 intraperiosteal clavicle resections with venorrhaphy, 1 femoral vein exploration and reconstruction, and 1 jugular venorrhaphy. Despite their relative biocompatibility, long-term silicone lines may become fixed in the vein. The rare and unpredictable occurrence of this problem may present the surgeon with unexpected intraoperative dilemmas. Before attempting localized extraction, surgeons should consider what venue, equipment, and anesthesia support may be required to successfully remove these catheters.

Anal canal duplication: case reviews and summary of the world literature.

Anal canal duplication (ACD) is a rare entity that is difficult to recognize. After the presentation of two patients, a review of the literature found 45 reported patients with ACD. This article presents the largest collection of known patients ACD in the literature. Of the 47 patients, 43 were female. The mean age at presentation was 28 months (range 0-24 years). The majority of the duplications were discovered incidentally (n = 21). Several presented with infectious complications such as epidural abscess with sepsis. Forty-two patients manifested an opening in the midline posterior to the native anus. Eighteen patients had associated anomalies, the majority of which were midline. Thirty-five of the patients underwent successful resection with rare complications. ACDs characteristically appear in females as incidental findings of an extra perineal orifice. They are frequently associated with additional congenital anomalies. Generally, ACDs are resectable with excellent outcomes.