Background and Aims

This is a nationwide study aiming to describe trends in cancer survival and mortality for children and young adolescents in the Netherlands including the unique information on stage at diagnosis.

Methods

All neoplasms in patients younger than 18 years, diagnosed between 1990-2015 (N=14,100), were derived from the Netherlands Cancer Registry. Traditional cohort-based and period-based analysis were used to estimate 5-year and 10-year observed survival (OS). OS was estimated overall and for the time periods 1990-99, 2000-09, and 2010-15. Changes over time were evaluated by parametric survival models adjusted for follow-up time. Time trends in mortality rates were evaluated by average annual percentage change.

Results

Since the 1990s, 5-year and 10-year OS of childhood and young adolescent cancer have improved significantly by 9 and 8 percent-points, reaching survival rates of 81% and 77%, respectively. Favourable trends in survival were observed for both genders, all age groups, and most diagnostic (sub)groups, and were particularly pronounced for advanced disease. Compared to 1990-99, the risk of dying within five years of diagnosis was significantly reduced in the most recent periods (2000-09: HR=0.8; 2010-15: HR=0.6) after adjustment for age, gender, and follow-up time. Nonetheless, the prognosis of children and young adolescents suffering from central nervous system tumours, neuroblastoma, and osteosarcoma remained poor, with 5-year OS <70% and/or 10-year OS <65%. Childhood and young adolescent cancer mortality in the Netherlands decreased by an average of 3% annually between 1990 and 2018.

Conclusions

Significant progress has been realized in the prognosis of childhood and young adolescent cancer in the Netherlands since the 1990s. Survival improvements were especially evident for patients with advanced stages and were also reflected by declining mortality rates.

Background and Aims

Birth defects have been consistently associated with elevated childhood cancer risk; however, the relationship between congenital heart disease (CHD) and childhood cancer remains conflicting and poorly understood. Considering the increasing patient population with CHD after improvements in their life expectancies, insights into this relationship are particularly compelling. Thus, we aimed to elucidate the relationship between CHD and cancer in Swedish children and adolescents.

Methods

All individuals registered in the Swedish Medical Birth Register (MBR) between 1973 and 2014 were included in this study (n=4,170,980). Individuals with CHD (n=68,776) were identified from the MBR and National Patient Register, whereas cancer diagnoses were retrieved from the Swedish Cancer Register. The relationship between CHD and childhood cancer (<20 years at diagnosis) was evaluated using Cox proportional hazards regression models.

Results

Among children with CHD, 221 had a cancer diagnosis during childhood. We observed increased risks of childhood cancer overall, leukemia, lymphoma, and hepatoblastoma in children with CHD, but after adjustment for Down syndrome, only the increased lymphoma (HR=1.55, 95% CI 1.05–2.31) and hepatoblastoma (HR=3.77, 95% CI 1.76–8.11) risk remained. However, when restricting to individuals with CHD diagnosed from the MBR only, i.e. those diagnosed near the time of birth, the risk for childhood cancer overall (HR=1.45, 95% CI 1.23–1.71) and leukemia (HR=1.41, 95% CI 1.08–1.84) was more pronounced, even after controlling for Down syndrome. Finally, a substantially elevated lymphoma risk (HR=8.05, 95% CI 4.02–16.14) was observed in children with complex CHD.

Conclusions

We found associations between CHD and childhood lymphomas and hepatoblastomas, not explained by a diagnosis of Down syndrome. Stronger associations were observed in complex CHD. The findings can help inform both the medical monitoring of children with CHD and future research aiming to elucidate the causal mechanisms underlying the relationships between CHD and childhood carcinogenesis.

Background and Aims

The “Assessing Doctors’ Attitudes on Palliative Treatment” (ADAPT) study was conducted in 11 Eurasian countries as an evidence-based, comprehensive, and culturally-relevant assessment of physician knowledge regarding the early integration of palliative care into pediatric oncology practice and perceptions of structural barriers affecting access to consultation. While study findings were published in 2020, regional stakeholders identified a demand for an advocacy tool to deliver country-specific results and policy recommendations to inform local stakeholders and implement policy change.

Methods

The ADAPT advocacy tool was developed based on study findings and delivers country-level data, reviewed by Eurasian and American pediatric palliative care and oncology experts to appeal to local health and educational stakeholders. In parallel, a survey was developed to understand the clinical, educational, and policy goals of those requesting reports and how they will be used to advocate for the indicated goals. The country report and survey were translated to English, Russian, and Mongolian.

Results

After iterative rounds of review, we created country-specific 2-page reports which display data and figures describing data on pediatric palliative care education, access, barriers to and timing of integration with childhood cancer care, alongside clinical and policy recommendations. These reports were distributed to regional collaborators in 11 countries. Survey results (n=31) demonstrated that the primary goals of advocacy included increasing access to pediatric palliative care services (77%), establishing a community-based palliative care and hospice network (70%), and increasing opportunities for palliative care specialization (70%).

Conclusions

We describe the development of an evidence-based advocacy tool utilizing published data to create local health, education, and policy change on-the-ground in Eurasia. By increasing accessibility of published findings through visual summary, translation to the local language, and adaptation to the knowledge of a broader audience, this work can improve the access to and quality of palliative care delivered to children with cancer.

Background and Aims

The experience of pediatric cancer is a life-altering event for all the family. Siblings must adapt to additional responsibilities, reduced availability of their parents and various conflicting emotions. Although psychological problems have been investigated and may persist over time, literature concerning long-term impact on siblings, especially on family functioning (FF), is scarce.

We aimed (1) to explore the link between the FF perceived by siblings of childhood leukemia survivors and the time since diagnosis and (2) to specify its determinants.

Methods

Recruited through the LEA cohort, a French multicenter long-term follow-up program, siblings over 11 years old of childhood leukemia survivors were asked to complete the Family Assessment Device (FAD). In accordance with authors’ threshold values, a score equal to or above 2.00 defined a problematic FF. Univariate and multivariate analyses were used to identify sibling's and survivor's sociodemographic, psycho-behavioral, environmental, health-related and cancer-related characteristics likely to be associated with siblings’ FF.

Results

We included 605 siblings (mean age 22.2 ±8.0 years, mean follow-up time from diagnosis 14.1 ±6.8 years). A first logistic regression showed FF did not differ according to the time since diagnosis but siblings of survivors with ≥2 sequela were significantly more likely to perceive impaired family functioning (OR=1.49, p=0.04). A second model, including familial economic status, showed siblings with household financial difficulties reported familial dysfunction (OR=1.49, p=0.04). Depending on siblings’ age, univariate analysis on psycho-behavioral characteristics showed a weak social support and emotional state seemed to be linked with a poorer perceived FF.

Conclusions

Our study contributed to identify profiles of siblings who may encounter difficulties within the family. To avoid late consequences, more consideration and support should benefit to siblings, also frequently considered as the “forgotten children”. Further research using Structural Equation Modeling is needed to explore links between FF and psycho-behavioral factors, such as coping strategies.

Background and Aims

Approximately 0.5-2% of the population identify as transgender; however, few studies have examined outcomes for transgender patients with cancer. Moreover, little is known about the outcomes for transgender and gender diverse pediatric patients with cancer.

Methods

A retrospective cohort study of 13 patients who were evaluated at Dana-Farber Cancer Institute/Boston Children’s Hospital between 1978-2020 with a cancer diagnosis before the age of 21 years and self-identified as transgender or gender diverse. Clinical and treatment characteristics, gender-affirming care (GAC), and long-term outcomes were recorded.

Results

Seven patients (54%) identified as transmasculine, three (23%) as transfeminine, two (15%) as non-binary, and one (8%) as gender fluid. Five patients (38%) were designated male at birth. Median follow-up time was 11.4 years (range: 1.2-41.3). Six (46%) patients had hematologic malignancies and seven (54%) had solid tumors. Ten (77%), nine (69%), and eight (62%) received chemotherapy, surgery, and radiotherapy, respectively. Five (38%) patients’ long-term survivorship care notes had mixed gender-specific pronoun usage. Eight (62%) patients received gender-affirming hormone therapy (GAHT) and five (38%) patients received gender-affirming surgeries after cancer diagnosis. One patient (8%) received GAHT before diagnosis of an androgen receptor-positive serous borderline ovarian tumor. After cancer diagnosis, the patient had a brief break from GAHT and restarted after multidisciplinary discussion regarding risks and benefits with the patient and family. Eleven (85%) patients’ oncology notes reported discussion with other specialty providers, including endocrinology, regarding the intersection of GAC, particularly safety of GAHT, and cancer care or associated toxicities.

Conclusions

Oncology providers will benefit from more education regarding caring for transgender patients, including correct pronoun usage. Transgender pediatric patients with cancer undergo treatments or suffer from toxicities that may affect GAC, highlighting the need for multidisciplinary care. Future studies need to incorporate experiences of transgender pediatric patients with cancer to further improve care.

Background and Aims

Non-chromosomal birth defects are an important risk factor for several childhood cancers. However, these associations are less clear for Hodgkin lymphoma (HL). Therefore, we sought to more fully elucidate the association between non-chromosomal birth defects and HL risk by leveraging data from a large study of childhood and adolescent HL.

Methods

Information on cases (n=517) diagnosed with HL between the ages of 0-14 at Children’s Oncology Group Institutions for the period of 1989-2003 was obtained. Healthy control children (n=784) were identified and individually matched to cases on sex, race/ethnicity, age and geographic location. Parents completed comprehensive interviews. To test the association between birth defects and HL risk, conditional logistic regression was applied to generate adjusted odds ratios (aORs) and 95% confidence intervals (CIs), adjusted for maternal age at index child’s birth, level of maternal education and annual household income level. We conducted these adjusted conditional logistic regression analyses overall and stratified by cofactors of interest (tumor Epstein-Barr viral status, histologic subtype and age group).

Results

Children born with non-chromosomal birth defects were not more likely to be diagnosed with HL at 0-14 years of age (aOR: 0.95; 95% CI: 0.73-1.23). A nonsignificant inverse association was detected for those children born with any birth defect and EBV-positive HL (aOR: 0.52; 95% CI: 0.25-1.08). Those born with two or more birth defects had a suggested increased risk of developing HL between the ages of 10-14 years (aOR: 2.09; 95% CI: 0.97-4.50).

Conclusions

Previous assessments of being born with a non-chromosomal birth defect and developing childhood HL have been limited. Using data from the largest case-control study of HL in those <15 years of age, we did not observe strong associations between being born with a birth defect and HL risk.