Background: Patients with relapsed or refractory neuroblastoma have a poor prognosis; there are limited effective and safe rescue chemotherapies for these patients. Development of new chemotherapy regimens for these patients is a key imperative. Procedure: We retrospectively analyzed patients with refractory or relapsed neuroblastoma who received irinotecan, etoposide, and carboplatin (IREC) as a second-line treatment for neuroblastoma. We evaluated the therapeutic response, toxicity, and survival outcomes. We also assessed the impact of UGT1A1 gene polymorphisms, which are involved in irinotecan metabolism, on the outcomes and toxicity. Results: A total of 131 cycles of IREC were administered to 43 patients with a median of two cycles per patient (range, 1–10). All patients were classified as high-risk (International Neuroblastoma Risk Group). Seven patients had relapsed before IREC. One patient (2%) showed partial response and 37 patients (86%) developed stable disease (disease control rate: 88%). Grade IV neutropenia was observed in 127 cycles (97%), while ≥ grade III gastrointestinal toxicity was observed in 3 cycles (2%). There was no IREC-related mortality. The one-year overall survival and progression-free survival rates were 65% and 52%, respectively. Patients with UGT1A1 polymorphisms showed a higher frequency of grade IV neutropenia; however, there was no increase in treatment-related mortality or nonhematological toxicity in these patients. Patients with UGT1A1 gene polymorphisms showed better one-year survival rate than the wild type (80% vs. 44%, p = 0.012). Conclusions: This study suggests that IREC is well-tolerated by patients with UGT1A1 polymorphisms and is a promising second-line chemotherapy for refractory/relapsed neuroblastoma.

Background: Central venous catheters (CVCs) have been essential devices for the treatment of children with hematological and oncological disorders. Only few studies investigated the complications and selections of different types of CVCs in these pediatric patients. This study aimed to compare risk factors for unplanned removal of two commonly used CVCs, i.e., peripherally inserted central catheters (PICCs) and tunneled CVCs, and propose better device selection for the patient. Procedure: This retrospective, single center cohort analysis was conducted on pediatric patients with hematological and oncological disorders inserted with either a PICC or a tunneled CVC. Results: Between January 1, 2013, and December 31, 2015, 89 patients inserted with tunneled CVCs (total 21,395 catheter-days) and 84 with PICCs (total 9,177 catheter-days) were followed up until the catheter removal. The median duration of catheterization was 88 days in PICCs and 186 days in tunneled CVCs (p = 1.24×10-9). PICCs at the 3-month cumulative incidence of catheter occlusion (5.2% vs. 0%, p = 4.08×10-3) and total unplanned removal (29.0% vs 7.0%, p = 0.0316) were significantly higher, whereas no significant difference was observed in the cumulative incidence of central line-associated bloodstream infection (11.8% vs. 2.3%, p = 0.664). Multivariable analysis identified younger age (<2 years) (subdistribution hazard ratio [SHR], 2.29; 95% confidence interval [CI], 1.27–4.14) and PICCs (SHR, 2.73; 95% CI, 1.48–5.02) were independent risk factors for unplanned removal. Conclusion: Our results suggest that tunnel CVCs would be a preferred device for children with hematological and oncological disorders requiring long-term, intensive treatment.