Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

Around 6% of all childhood malignancies represent renal tumors, of which a majority includes Wilms tumor (WT). Although survival rates have improved over the last decades, specific patients are still at risk for adverse outcome. In the Netherlands, since 2015, pediatric oncology care for renal tumors has been centralized in the Princess Máxima Center for Pediatric Oncology. Here, we describe experiences of the first 5 years of centralized care and explore whether this influences the epidemiological landscape by comparing data with the Netherlands Cancer Registry (NCR). We identified all patients <19 years with a renal mass diagnosed between 01-01-2015 and 31-12-2019 in the Princess Máxima Center. Epidemiology, characteristics and management were analyzed. We identified 164 patients (including 1 patient who refused consent for registration), in our center with a suspicion of a renal tumor. The remaining 163 cases included WT (n = 118)/cystic partially differentiated nephroblastoma (n = 2)/nephrogenic rests only (n = 6) and non-WT (n = 37). In this period, the NCR included 138 children, 1 17-year-old patient was not referred to the Princess Máxima Center. Central radiology review (before starting treatment) was performed in 121/163 patients, and central pathology review in 148/152 patients that underwent surgery. Treatment stratification, according to SIOP/EpSSG protocols was pursued based on multidisciplinary consensus. Preoperative chemotherapy was administered in 133 patients, whereas 19 patients underwent upfront surgery. Surgery was performed in 152 patients, and from 133 biomaterial was stored. Centralization of care for children with renal tumors led to referral of all but 1 new renal tumor cases in the Netherlands, and leads to referral of very rare subtypes not registered in the NCR, that benefit from high quality diagnostics and multidisciplinary decision making. National centralization of care led to enhanced development of molecular diagnostics and other innovation-based treatments for the future.

Outcome of esophageal atresia: inborn versus outborn patients

Summary Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume–outcome association in EA management remains unconfirmed. Furthermore, whether outcomes differ between outborn and inborn patients with EA has not been systematically investigated. Therefore, this single-center, retrospective study aimed to investigate EA management and outcomes with a special focus on inborn versus outborn patients. The following data were extracted from the medical records of infants with EA from 2009 to 2019: EA type, associated anomalies, complications, and long-term outcome. Patients were allocated into inborn and outborn groups. Altogether, 57 patients were included. Five patients were excluded (referral before surgery, loss of data, death before surgery [n = 1], and incorrect diagnosis [diverticulum, n = 1]). Among all patients, the overall survival rate was 96%, with no mortalities among outborn patients. The overall hospitalization period was shorter for outborn patients. The median follow-up durations were 3.8 years and 3.2 years for inborn and outborn patients, respectively. Overall, 15% of patients underwent delayed primary anastomosis (long-gap atresia [n = 4] and other reasons [n = 4]). Early complications included three anastomotic leakages and one post-operative fistula; 28% of patients developed strictures, which required dilatation, and 38% of patients showed relevant gastroesophageal reflux, which required fundoplication, without any differences between the groups. The two groups had comparable low mortality and expected high morbidity with no significant differences in outcome. The outborn group showed nonsignificant trends toward lower morbidity and shorter hospitalization periods, which might be explained by the overall better clinical status.

The value of autopsy in preterm infants at a Swedish tertiary neonatal intensive care unit 2002–2018

Reliable data on causes of death (COD) in preterm infants are needed to assess perinatal care and current clinical guidelines. In this retrospective observational analysis of all deceased preterm infants born < 37 weeks’ gestational age (n = 278) at a Swedish tertiary neonatal intensive care unit, we compared preliminary COD from Medical Death Certificates with autopsy defined COD (2002–2018), and assessed changes in COD between two periods (period 1:2002–2009 vs. period 2:2011–2018; 2010 excluded due to centralized care and seasonal variation in COD). Autopsy was performed in 73% of all cases and was more than twice as high compared to national infant autopsy rates (33%). Autopsy revised or confirmed a suspected preliminary COD in 34.9% of the cases (23.6% and 11.3%, respectively). Necrotizing enterocolitis (NEC) as COD increased between Period 1 and 2 (5% vs. 26%). The autopsy rate did not change between the two study periods (75% vs. 71%). We conclude that autopsy determined the final COD in a third of cases, while the incidence of NEC as COD increased markedly during the study period. Since there is a high risk to determine COD incorrectly based on clinical findings in preterm infants, autopsy remains a valuable method to obtain reliable COD.

POS0501 SUSTAINED 2-YEAR REMISSION OF THE DISEASE IN A CLINIC OF EXCELLENCE IN RHEUMATOID ARTHRITIS. EXPERIENCE OF THE CLINICAL REGISTRY IN COLOMBIA

Background:Rheumatoid arthritis (RA) is a chronic autoimmune disease with no cure, characterized by episodes of exacerbation and remission, which requires permanent use of medications. Clinics of excellence are multidisciplinary and centralized programs that improve adherence to treatments. Information on the benefits of these models of care has been published but is not definitive. In Colombia, the clinical registry of patients with RA is kept in the Cuenta de Alto Costo (CAC).Objectives:To demonstrate the difference in the percentages of sustained remission at 2 years, between an institution with non-centralized management or standard of care (Hospital Militar Central-HMC) compared to another institution with centralized management or clinic of excellence (BIOMAB-IPS) and determine if the results are determined by any of the intervention variables or by the program.Methods:The 2-year clinical records for the CAC were compared between an institution with non-centralized management (HMC) in comparison with another institution with centralized management (BIOMAB-IPS), performing a sociodemographic description, measuring control of the disease DAS28 clinimetry, Fisher’s test non-parametric bivariate analysis, multiple regression model, and population matching with Propensity score Matching (PSM).Results:Complete information was obtained from 2 years of follow-up, in centralized management 3457 patients and for the non-centralized unit 114 patients. Most of them corresponded to 2962 women (82%), with time of illness of 9.5 years and 10.2 years, respectively, without statistically significant differences. A difference was observed in the 2 programs to maintain remission at 2 years, in favor of the centralized program 54.7% vs 28.6.2% (p <0.00). With the binomial generalized linear regression model, it was confirmed that this difference was not explained by variables such as the use of biological therapy (RR = 0.77; 95% CI 0.69-0.86), use of DMARDs (RR = 0.71; 95% CI 0.62-0.82) and number of rheumatology consultations (RR = 0.97; 95% CI 0.92-1.02) in comparison with the centralized care model (RR = 2.32; 95% CI 1.58-3.35). Due to the biases between the groups due to the non-probability sampling, a PSM was performed, with a 1: 1 match, caliper of 0.065, obtaining a pseudo population with well-balanced covariates (see table 1). In the common support area, statistically significant differences were documented in sustained remission over 2 years, in favor of the centralized care group 45 vs 17.9% (p = 0.001).Conclusion:With the information from the clinical records, statistical strategies can be used to evaluate important differences in centralized care programs, observing favorable results of these types of care that are not related to isolated elements of the program, but to the overall effect of the program.References:[1]Austin PC. Double propensity-score adjustment: A solution to design bias or bias due to incomplete matching. Stat Methods Med Res. 2017;26(1):201–22.33333Disclosure of Interests:Juan Manuel Bello-Gualtero: None declared, Esperanza Peña: None declared, Pedro Iván Santos Moreno: None declared, Jasmin Vesga Gualdrón Employee of: Baxter, Ginna Saavedra: None declared, Clara Perez: None declared

Traveling for Pancreatic Cancer Care Is Worth the Trip

Background Centralized care for patients with pancreatic cancer is associated with longer survival. We hypothesized that increased travel distance from home is associated with increased survival for pancreatic cancer patients. Methods The National Cancer Database user file for all pancreatic cancer patients was investigated from 2004 through 2015. Distance from the patients’ zip code to the treating facility was determined. Survival was investigated using the Kaplan-Meier method. Cox hazard ratios (CoxHRs) were determined based on stage of disease, distance traveled for care, and clinical factors. Results 340 780 patients were identified. In the average age of 68 ± 12 years, 51% were male and 83% were Caucasian. For all stages of cancer, longer survival was associated with traveling farther ( P < .001). The survival advantage was longer for Caucasians than African Americans (3.7 months vs. 2.6 months, P < .001) Travel was associated with a 13% decrease in risk of death ( P < .001). Even controlling for the pathologic stage, traveling farther was associated with decreased risk of death (CoxHR = .91, P < .001). Discussion Traveling for care is associated with improved survival for pancreatic cancer patients. While a selection bias may exist, the fact that all stages of patients investigated benefited suggests that this is a real phenomenon.