International Comparisons of Clinical Demographics and Outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study.

PURPOSE: International comparisons of patient demographics, tumor characteristics, and survival can shed light on areas for health care system improvement. The International Society of Pediatric Oncology Wilms Tumor 2001 trial/study registered patients through national clinical study groups in Western Europe and Brazil. This retrospective post hoc analysis of the International Society of Pediatric Oncology Wilms Tumor 2001 database aims to make visible and suggest reasons for any variations in outcomes. METHODS: All patients with unilateral Wilms tumor (WT), age > 6 months, treated with preoperative chemotherapy as per protocol, and registered between 2001 and 2011 were eligible. Countries were grouped to give comparable case numbers and geographical representation. Cox univariable and multivariable (MVA) statistics were applied, with the German collaborative group (Gesellschaft für Pädiatrische Onkologie und Hämatologie-Austria, Germany, and Switzerland) as reference for hazard ratios for event-free survival (EFS) and overall survival (OS). RESULTS: A total of 3,176 eligible patients were registered from 24 countries assigned into six groups. Age and histologic risk group distribution were similar across all groupings. The distribution of WT stage varied by country grouping, with 14.9% (range, 11.1%-18.2%) metastatic at diagnosis. Median follow-up was 78.9 months. For localized WT, 5-year EFS varied from 80% (Brazilian group) to 91% (French group; P < .0001), retaining significance only for Brazil in MVA (P = .001). Five-year OS varied from 89% (Brazilian group) to 98% (French group; P < .0001). In MVA, only superior OS in France was significant (P = .001). Five-year EFS/OS for stage IV did not vary significantly. High-risk histology and tumor volume at surgery were significantly associated with increased risk of death in MVA for metastatic disease. CONCLUSION: International benchmarking of survival rates from WT within a large trial/study database has demonstrated statistically significant differences. Clinical interpretation should take account of variation in tumor stage but also treatment factors.

Talking about Death with Children with Incurable Cancer: Perspectives from Parents.

OBJECTIVE: To investigate the rationale and consequences associated with a parent's decision to discuss death with a child with incurable cancer. STUDY DESIGN: We present data from a larger retrospective study involving bereaved parents of a child who died of cancer. Parents were asked whether they had discussed the impending death with their child, whether they reflected on this discussion positively, their reasons for not discussing death with their child, and the manner in which the conversation regarding death occurred. The data were analyzed qualitatively using a framework approach. RESULTS: Of the 86 parents of 56 children who answered the questions regarding discussing death with their child, 55 parents of 35 children did not discuss the impending death with their child. The following themes were identified: the parents' inability to discuss the impending death; the parents' desire to protect their child; views regarding talking with children; parents' views of child characteristics; the child's unwillingness to discuss the subject; lack of opportunity to talk; and the child's disability. The parents who did discuss death with their child generally used symbolic and/or religious narratives, or they had brief, direct conversations regarding death. The majority of parents felt positive regarding their decision about whether to talk with their child about his/her impending death. CONCLUSION: Most parents in this study cited several reasons for not discussing death with their child. Our findings highlight the sensitive and complex issues surrounding these conversations, indicating that there may be a role for clinicians in supporting parents.

Altered bone mineral density and body composition, and increased fracture risk in childhood acute lymphoblastic leukemia.

OBJECTIVE: To evaluate fracture rate and bone mineral density (BMD) and body composition in children with acute lymphoblastic leukemia (ALL) treated with dexamethasone-based chemotherapy. STUDY DESIGN: Children with ALL (n = 61) participated. At diagnosis, during therapy, and one year after cessation of therapy, BMD and body composition were measured using dual energy X-ray absorptiometry of lumbar spine (LS) and total body (TB). Serum markers of bone turnover were assessed. RESULTS: BMD(LS) was significantly reduced at diagnosis, and remained low during therapy. BMD(TB) was normal at diagnosis, with a fast decrease in the first 32 weeks, in which chemotherapy was relatively intensive. Apparent ("volumetric") BMD(LS) was also reduced, but this did not reach significance at diagnosis and follow-up. Bone formation markers were reduced at diagnosis; formation as well as resorption markers increased during treatment. Fracture rate was 6 times higher in ALL patients compared with healthy controls. Lean body mass was decreased at baseline. Percentage of body fat increased significantly during therapy. After ALL treatment was completed, BMD and body composition tended to improve. CONCLUSIONS: Children with ALL are at risk for osteopenia because of the disease itself and the intensive chemotherapy. Fracture rate increases substantially, not only during but also shortly after treatment.