Symptom management care pathway adaptation process and specific adaptation decisions.

BACKGROUND: There is substantial heterogeneity in symptom management provided to pediatric patients with cancer. The primary objective was to describe the adaptation process and specific adaptation decisions related to symptom management care pathways based on clinical practice guidelines. The secondary objective evaluated if institutional factors were associated with adaptation decisions. METHODS: Fourteen previously developed symptom management care pathway templates were reviewed by an institutional adaptation team composed of two clinicians at each of 10 institutions. They worked through each statement for all care pathway templates sequentially. The institutional adaptation team made the decision to adopt, adapt or reject each statement, resulting in institution-specific symptom management care pathway drafts. Institutional adaption teams distributed the 14 care pathway drafts to their respective teams; their feedback led to care pathway modifications. RESULTS: Initial care pathway adaptation decision making was completed over a median of 4.2 (interquartile range 2.0-5.3) weeks per institution. Across all institutions and among 1350 statements, 551 (40.8%) were adopted, 657 (48.7%) were adapted, 86 (6.4%) were rejected and 56 (4.1%) were no longer applicable because of a previous decision. Most commonly, the reason for rejection was not agreeing with the statement (70/86, 81.4%). Institutional-level factors were not significantly associated with statement rejection. CONCLUSIONS: Acceptability of the 14 care pathways was evident by most statements being adopted or adapted. The adaptation process was accomplished over a relatively short timeframe. Future work should focus on evaluation of care pathway compliance and determination of the impact of care pathway-consistent care on patient outcomes. TRIAL REGISTRATION: clinicaltrials.gov, NCT04614662. Registered 04/11/2020, https://clinicaltrials.gov/ct2/show/NCT04614662?term=NCT04614662&draw=2&rank=1 .

Barriers to Pediatric Oncologist Enrollment of Adolescents and Young Adults on a Cross-Network National Clinical Trials Network Supportive Care Cancer Clinical Trial.

Few studies have explored interventions to improve adolescent and young adult (AYA) cancer care delivery. While many AYAs receive cancer care at NCI Community Oncology Research Program (NCORP) sites, few enroll on clinical trials. Barriers and facilitators to pediatric oncologist activation of and enrollment on an AYA cross-network National Clinical Trials Network (NCTN) supportive care trial were assessed using a survey that was administered to 162 stakeholders representing all 47 children's oncology group (COG) institutions affiliated to an NCORP. Fifty-eight stakeholders participated representing 62% of all sites surveyed. Approximately half of participants (45%) were unaware of the trial. Seven sites had the study open and one enrolled a patient. Reasons for not opening and enrolling on the trial included limited research staff and resources, low anticipated accrual, and lower prioritization of the trial. Enrollment facilitators included having a local "AYA champion," improving communication between pediatric and medical oncology, and having site education on available AYA trials. Interventions focused on increasing site and provider awareness of AYA trials and decreasing local barriers to AYA enrollment are needed.

First pediatric experience of SL-401, a CD123-targeted therapy, in patients with blastic plasmacytoid dendritic cell neoplasm: report of three cases.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly aggressive hematological malignancy with extremely poor outcome. The median overall survival for adult patients is 9-13 months. Pediatric patients are exceedingly rare with an unclear clinical course. Currently, no standardized therapy has been established, although an acute lymphoblastic leukemia type of treatment appears to be more effective in those patients who are able to tolerate aggressive chemotherapy. SL-401 is a targeted therapy directed to CD123, a protein ubiquitously expressed at high level on the surface of BPDCN blasts. In adult phase 2 trials, it has demonstrated efficacy with 90% overall response rate. No pediatric patients with BPDCN using SL-401 have been reported. CASE PRESENTATION: Here, we report the first pediatric experience of three children with BPDCN treated with SL-401 at our institution. All patients tolerated SL-401 without significant toxicities. One patient with multiply relapsed and refractory disease had no response. The other two cases had significant and rapid clinical improvement after the two courses of treatment. However, the response was transient, and growth of soft tissue mass was observed in-between cycles in both patients with large tumor burden. CONCLUSIONS: This is the first report of SL-401 in pediatric patients with BPDCN. Sl-401 was well tolerated and can produce a promising response. Further testing this agent in children is warranted.

Suprasellar Germinoma Presenting with Slipped Capital Femoral Epiphysis: Case Report.

Slipped capital femoral epiphysis (SCFE) is a fracture that results from displacement of the proximal femoral epiphysis from the femoral neck. SCFE can be caused by various endocrinopathies that lead to bone weakening in both adult and pediatric patients. We report a rare case of suprasellar germinoma presenting with SCFE in an 11-year-old female patient. The findings of this case further support the need to consider pituitary lesions as the underlying cause of endocrine deficiences leading to SCFE.

Chronic osteomyelitis in children: Shriners Hospital Honolulu experience.

Chronic osteomyelitis incidence and severity in 55 hospitalized Pacific Island children between 1990 and 2002 were compared with the expected incidence and reports in the literature. Of these 55 cases, 87% were from Polynesia/Micronesia. The average length of hospital stay was 104 days. Staphylococcus aureus was cultured in 64% of the cases with 43% of those being methicillin resistant S. aureus. A total of 111 bones were involved. Average antibiotic treatment was 135 days. Each case required an average of 1.3 irrigations/debridements and 45% required a sequestrectomy. Ninety-two percent had elevated erythrocyte sedimentation rate on admission. Sixty-nine percent of the cases involved metaphyseal, diaphyseal and epiphyseal segments of the bone and 29% were multifocal. Results indicate that Pacific Island children have a higher incidence and increased severity of osteomyelitis when compared with non-Pacific Island children in the literature, requiring a high suspicion for multifocal osteomyelitis, extensive bone involvement, S. aureus positive cultures and a longer period of antibiotic treatment.

A practical approach to neutrophil disorders.

This review discusses disorders of altered neutrophil number and function and provide a basic framework for patient evaluation and management. The sections begin with neutropenia, neutrophilia and neutrophil dysfunction with a general screening approach to differentiate common, more benign syndromes from rare, often more serious disorders. Also included is a detailed discussion of some specific primary neutrophil syndromes at the end of each section. Focus is placed on specific disorders that are clinically common or particularly instructive.