Outcomes for children with recurrent/refractory atypical teratoid rhabdoid tumor: A single-institution study with molecular correlation.

BACKGROUND: Survival data for recurrent pediatric atypical teratoid rhabdoid tumor (ATRT) and its association to molecular groups are extremely limited. METHODS: Single-institution retrospective study of 64 children less than 21 years old with recurrent or treatment-refractory (progressive disease [PD]) ATRT treated at St. Jude Hospital from January 2000 to December 2020. Demographic, clinicopathologic, treatment, molecular grouping (SHH, TYR, and MYC) and germline data were collected. Progression-free survival (PFS2: time from PD to subsequent first progression) and overall survival (OSpostPD: time from PD to death/last follow-up) were estimated by Kaplan-Meier analysis. RESULTS: Median age at and time from initial diagnosis to PD were 2.1 years (range: 0.5-17.9 years) and 5.4 months (range: 0.5-125.6 months), respectively. Only five of 64 children (7.8%) are alive at median follow-up of 10.9 (range: 4.2-18.1) years from PD. The 2/5-year PFS2 and OSpostPD were 3.1% (±1.8%)/1.6% (±1.1%) and 20.3% (±4.8%)/7.3% (±3.5%), respectively. Children with TYR group (n = 10) had a better OSpostPD compared to those with MYC (n = 11) (2-year survival estimates: 60.0% ± 14.3% vs. 18.2% ± 9.5%; p = .019), or those with SHH (n = 21; 4.8% ± 3.3%; p = .014). In univariate analyses, OSpostPD was better with older age at diagnosis (p = .037), female gender (p = .008), and metastatic site of PD compared to local or combined sites of PD (p < .001). Two-year OSpostPD for patients receiving any salvage therapy (n = 39) post PD was 33.3% ± 7.3%. CONCLUSIONS: Children with recurrent/refractory ATRT have dismal outcomes. Older age at diagnosis, female gender, TYR group, and metastatic site of PD were associated with relatively longer survival in our study.

Reducing Wait Time in a High-volume Pediatric Neuro-oncology Clinic by Optimizing Process Flow: A Quality Improvement Project.

Introduction: Hospital wait time (WT) influences healthcare quality and patient satisfaction. Long WTs are distressful for patients and considered substandard healthcare delivery. Pediatric hematology/oncology patients with complex medical conditions frequently need multiple appointments in a day, making their scheduling very challenging. Here, we report a quality improvement (QI) project aimed to decrease the percentage of patients waiting >30 minutes before room placement in the neuro-oncology clinic. Methods: We measured WT from when the patient reported to the clinic (or, for those arriving early, from scheduled appointment) to when the patient got an exam room. We collected data by random sampling and collected baseline data over the initial 4 weeks; generated process mapping and Pareto charts to identify reasons for delayed patient placement in rooms; and used iterative Plan-Do-Study-Act (PDSA) cycles to test interventions. We used Run charts and Shewhart charts for data analysis. Results: Our baseline data analyses showed provider and room availability as critical reasons for delayed room placement (38.4% and 30%, respectively). We also completed related PDSA improvement cycles. The median percent of patients waiting >30 minutes decreased from 21% to 13%. The median average waiting time decreased from 21 to 11 minutes. Conclusion: Using structured QI methodology, we decreased the percent of patients waiting >30 minutes before room placement and overall WTs. We developed a strategy for continuous improvement and future interventions. Furthermore, our results suggest that QI projects, which account for the complexity of hospital systems, can improve patient flow throughout the hospital.