| 1. |
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| 2. |
- Anastasopoulou, Stavroula, et al.
(author)
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Does minimal central nervous system involvement in childhood acute lymphoblastic leukemia increase the risk for central nervous system toxicity?
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:7
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Journal article (peer-reviewed)abstract
- Central nervous system (CNS) involvement in childhood acute lymphoblastic leukemia (ALL) implicates enhanced intrathecal chemotherapy, which is related to CNS toxicity. Whether CNS involvement alone contributes to CNS toxicity remains unclear. We studied the occurrence of all CNS toxicities, seizures, and posterior reversible encephalopathy syndrome (PRES) in children with ALL without enhanced intrathecal chemotherapy with CNS involvement (n = 64) or without CNS involvement (n = 256) by flow cytometry. CNS involvement increased the risk for all CNS toxicities, seizures, and PRES in univariate analysis and, after adjusting for induction therapy, for seizures (hazard ratio [HR] = 3.33; 95% confidence interval [CI]: 1.26-8.82; p = 0.016) and PRES (HR = 4.85; 95% CI: 1.71-13.75; p = 0.003).
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| 3. |
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| 4. |
- Anderzen-Carlsson, Agneta, 1966-, et al.
(author)
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CHILDREN'S NARRATIVES OF SUPPORT FROM PARENTS WHEN EXPERIENCING FEAR RELATED TO ACUTE LYMPHOBLASTIC LEUKEMIA
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:Suppl. 5, s. S528-S528
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Journal article (other academic/artistic)abstract
- Background and Aims: Children diagnosed with Acute Lymphoblastic Leukemia (ALL) typically undergo intense treatment with frequent hospitalizations. Medical, as well as existential fears have been identified. It has also been found that children's coping strategies develop during their illness trajectory. The literature on what children with ALL find to be valuable support from parents when experiencing fear is sparse. Thus, the aim of this presentation is to describe what young children find to be important support from their parents when experiencing fear related to ALL.Methods: The study had a longitudinal descriptive qualitative design. Thirteen children (3 girls and 10 boys), initially 5-9 years old were interviewed once to three times during their treatment period (approximately 2 months after the diagnosis, after 1 year, and at end of treatment). Data were analyzed using a matrix-based qualitative analysis method.Results: The parents’ physical and emotional closeness was the most frequently reported support. It eased the children's medical and existential fears. The children also found it supportive when the parents facilitated for them to participate in their care and when the parents acted as their advocate. Other supportive measures were offering distraction, talking to the child about their fears, assisting the professionals in alleviating pain and fear, being playful and encouraging. Five children also appreciated when their parents restricted them, during medical procedures. The experiences of support varied between children and between different time points during treatment.Conclusions: Although being quite young, the children were able to describe what they found to be supportive when experiencing fear, or for preventing fear. The parental support had an impact on the child's emotional, social and physical wellbeing. Professionals should encourage parents to stay with their child, and offer support to the parents, so that they in turn can support their child.
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| 5. |
- Anderzén Carlsson, Agneta, 1966-, et al.
(author)
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Children’s Narratives of Support From Professionals when Experiencing Fear Related to Acute Lymphoblastic Leukemia
- 2020
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 67:S4, s. 120-120
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Journal article (peer-reviewed)abstract
- Children diagnosed with Acute Lymphoblastic Leukemia (ALL) typically face 2.5 years treatment, which initially is intense and includes frequent hospitalizations. Previous research has identified various fears during treatment: fear of getting needles, removal of adhesive tapes, having a feeding tube, taking tablets and the physical changes related to ALL itself, as well as to treatments. The children’s coping strategies develop during the course of illness. The literature on what children with ALL find to be supportive when experiencing fear is sparse. The aim of this presentation is thus to describe what children 5-9 years old find to be important support from professionals when experiencing fear related to ALL.
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| 6. |
- Attarbaschi, A., et al.
(author)
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Rare non-Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric-type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T-cell lymphoma
- 2020
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 67:8
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Journal article (peer-reviewed)abstract
- Pediatric-type follicular (PTFL), marginal zone (MZL), and peripheral T-cell lymphoma (PTCL) account each for <2% of childhood non-Hodgkin lymphoma. We present clinical and histopathological features of PTFL, MZL, and few subtypes of PTCL and provide treatment recommendations. For localized PTFL and MZL, watchful waiting after complete resection is the therapy of choice. For PTCL, therapy is subtype-dependent and ranges from a block-like anaplastic large cell lymphoma (ALCL)-derived and, alternatively, leukemia-derived therapy in PTCL not otherwise specified and subcutaneous panniculitis-like T-cell lymphoma to a block-like mature B-NHL-derived or, preferentially, ALCL-derived treatment followed by hematopoietic stem cell transplantation in first remission in hepatosplenic and angioimmunoblastic T-cell lymphoma.
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| 7. |
- Banerjee, Joanna, et al.
(author)
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The spectrum of acute central nervous system symptoms during the treatment of childhood acute lymphoblastic leukaemia
- 2020
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In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017. ; 67:2
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Journal article (peer-reviewed)abstract
- Background: Children with central nervous system (CNS) toxicity during therapy for acute lymphoblastic leukaemia (ALL) are at risk for treatment modifications, long-term sequelae and even higher mortality. A better understanding of CNS symptoms and their complications improves the potential to prevent and treat them.Methods: Patient files from 649 children treated with Nordic Society of Pediatric Hematology and Oncology ALL92 and ALL2000 protocols in Finland were reviewed retrospectively for any acute CNS symptom. Detailed data on symptoms, examinations and treatment of the underlying CNS complications were collected from the medical records. Disease-related and outcome data were retrieved from the Nordic leukaemia registry.Results: Altogether, 13% (86) of patients with ALL had acute CNS symptoms. Most symptoms (64%) occurred during the first 2 months of therapy. Posterior reversible encephalopathy syndrome was the most frequent complication (4.5%). Cerebrovascular events were diagnosed in 10 cases (1.6%), while methotrexate-related stroke-like syndrome (SLS) was observed in only one patient (0.2%). CNS symptoms due to systemic or unclear conditions, especially sepsis, were important for differential diagnosis. CNS leukaemia was associated with CNS symptoms (hazard ratio [HR] = 4.03; P = .003), and epilepsy was a common sequel of CNS complications (19%).Conclusions: Acute CNS symptoms are common during ALL therapy, occurring mainly during the first 2 months of treatment. Patients with CNS leukaemia at diagnosis are at a higher risk for CNS toxicity. Despite intensive CNS-directed methotrexate treatment, SLS was diagnosed extremely rarely in our series.
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| 8. |
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| 9. |
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| 10. |
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| 11. |
- Giertz, Mia, et al.
(author)
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Symptomatic osteonecrosis in children treated for Hodgkin lymphoma: A population-based study in Sweden, Finland, and Denmark
- 2024
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In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017.
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Journal article (peer-reviewed)abstract
- Background: Osteonecrosis (ON) is a potentially disabling skeletal complication of cancer treatment. Although symptomatic osteonecrosis (sON) is well-known in acute lymphoblastic leukemia (ALL), with an incidence around 6%, studies on sON in pediatric Hodgkin lymphoma (HL) are scarce. The aim of this study was to examine the incidence, risk factors, and outcome of sON in children treated for HL. Procedure: A total of 490 children under 18, diagnosed with HL between 2005 and 2019 in Sweden, Finland, and Denmark were eligible for the study. Data on patient characteristics, HL treatment, and development of sON were collected from patients' medical records. Magnetic resonance imaging scans were used to establish ON diagnosis and grade ON according to the Niinim & auml;ki grading system. Results: Cumulative 2-year incidence of sON among the 489 included patients was 5.5% (n = 30). The risk for developing sON was higher for those with older age (odds ratio [OR] 1.25, 95% confidence interval [CI]: 1.05-1.49, p < .010), female sex (OR 4.45, CI 1.87-10.58, p < .001), high total cumulative glucocorticoid (GC) doses (OR 1.76, 95% CI: 1.21-2.56, p = 0.003), and advanced HL (OR 2.19, 95% CI: 1.03-4.65, p = .042). Four (13.3%) patients underwent major surgical procedures and 13 (43.3%) had persistent symptoms due to ON at follow-up. Conclusions: This study shows that sON is as common in pediatric HL as in pediatric ALL, with risk factors such as older age, female sex, high cumulative GC doses, and advanced HL. Future HL protocol development should aim to reduce the burden of ON by modifying GC treatment.
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| 12. |
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| 13. |
- Grundström, Albin, et al.
(author)
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Educational and occupational outcomes in Swedish children treated for sarcomas : A nationwide registry-based study
- 2024
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 71:1
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Journal article (peer-reviewed)abstract
- Background: Many children treated for cancer experience a negative impact on their academic performance; however, most studies of children treated for sarcomas have not investigated academic performance. Our aim was to explore how Swedish children treated for sarcomas perform academically, as well as how they adjust to life afterwards. Procedure: We compared 167 pediatric sarcoma survivors with 776 matched, non-sibling controls without a history of cancer, in a retrospective cohort study using data from nation wide registries. Primary outcomes were grades at the end of compulsory education, high school eligibility, post-compulsory education (i.e., education after school Year 9), employment, and sickness or activity compensation. Results: Pediatric sarcoma survivors were more likely to be ineligible for high school (odds ratio [OR] 1.76; p = .045) and more likely to fail Swedish (OR 2.12; p = .046), mathematics (OR 2.27; p = .011), and/or physical education (OR 2.24; p = .004), compared with controls. Survivors were less likely to have been employed (OR 0.58; p = .027) and received sickness or activity compensation more often (OR 2.49; p = .008) compared with controls. Conclusions: Pediatric sarcoma survivors have poorer academic performance compared to peers without cancer in multiple school subjects. Survivors seem to catch up during post-compulsory education, but might struggle to find employment.
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| 14. |
- Heinz, Amadeus, et al.
(author)
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Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification : Data of the European Soft Tissue Sarcoma Registry (SoTiSaR)
- 2023
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 70:7
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Journal article (peer-reviewed)abstract
- Background: Outcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance. Methods: Second-line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low- (LR), standard- (SR), and high-risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline-based regimen in the very high-risk (VHR) group. Data of patients with relapse (n = 68) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018) were retrospectively analyzed. Results: Patients of initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n = 9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC (n = 12), or other (n = 15). Resection was performed in 40/68 (59%) patients and/or radiotherapy in 47/68 (69%). Initial risk stratification, pattern/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (p =.17). The 5-year event-free survival (EFS) and overall survival (OS) were 26% (+/- 12%) and 31% (+/- 14%). The 5-year OS of patients with relapse of SR, HR, and VHR groups was 80% (+/- 21%), 20% (+/- 16%), and 13% (+/- 23%, p =.008), respectively. Conclusion: Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups.
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| 15. |
- Heinz, Amadeus T., et al.
(author)
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Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma : Data of the European Soft Tissue Sarcoma Registry SoTiSaR
- 2024
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 71:1
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Journal article (peer-reviewed)abstract
- Background: Outcome of primary metastatic rhabdomyosarcoma (RMS) is poor. Certain risk factors as fusion status, Oberlin score, and local treatment of primary tumor are known to influence prognosis.Procedure: Patients with metastatic RMS were treated according to Cooperative Weichteilsarkom Studiengruppe (CWS) guidance with chemotherapy (CHT), radiotherapy (RT) excluding total lung irradiation (TLI), complete resection of the primary tumor, and metastasectomy if possible. Kaplan-Meier estimators and Cox proportional hazard models were used to examine event-free survival (EFS) and overall survival (OS) involving also landmark analyses.Results: In the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018), 211 patients were analyzed. Many patients had fusion-positive alveolar RMS (n = 83; 39%). Median age was 9.4 years [0.1-19.7 years]. Treatment primarily consisted of CHT with CEVAIE (carboplatin, epirubicine, vincristine, actinomycin-D, ifosfamide, etoposide: 86%, other regimens: 14%), RT (71%), resection of primary tumor (37%), metastasectomy (19%), and lymph node sampling/dissection (21%). Maintenance treatment (MT) (oral trofosfamide, idarubicin, etoposide) was added in 74% of patients. Oberlin factors, fusion status, and MT were predictive for EFS and OS. MT with O-TIE was not improving outcome when adjusting for the immortal time bias. Local treatment of the primary tumor and radical irradiation (except TLI) improved EFS, not OS, when adjusting for the Oberlin score. Patients with fusion-negative alveolar RMS (n = 9) had an excellent outcome with a 5-year EFS and OS of 100%, compared to patients with embryonal RMS (49%/62%), PAX7- (22%/47%) and PAX3/FOXO1-positive ARMS (10/13%), respectively (p < .001).Conclusions: Prognosis of metastatic RMS primarily depends on fusion status and Oberlin score. Fusion status needs to be considered in future trials to optimize treatment outcome. The role of radical irradiation needs further investigation.
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| 16. |
- Helenius, Marianne, et al.
(author)
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Characteristics of white blood cell count in acute lymphoblastic leukemia : A COST LEGEND phenotype-genotype study
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:6
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Journal article (peer-reviewed)abstract
- Background White blood cell count (WBC) as a measure of extramedullary leukemic cell survival is a well-known prognostic factor in acute lymphoblastic leukemia (ALL), but its biology, including impact of host genome variants, is poorly understood.Methods We included patients treated with the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL-2008 protocol (N = 2347, 72% were genotyped by Illumina Omni2.5exome-8-Bead chip) aged 1-45 years, diagnosed with B-cell precursor (BCP-) or T-cell ALL (T-ALL) to investigate the variation in WBC. Spline functions of WBC were fitted correcting for association with age across ALL subgroups of immunophenotypes and karyotypes. The residuals between spline WBC and actual WBC were used to identify WBC-associated germline genetic variants in a genome-wide association study (GWAS) while adjusting for age and ALL subtype associations.Results We observed an overall inverse correlation between age and WBC, which was stronger for the selected patient subgroups of immunophenotype and karyotypes (rho(BCP-ALL )= -.17, rho(T-ALL )= -.19; p < 3 x 10(-4)). Spline functions fitted to age, immunophenotype, and karyotype explained WBC variation better than age alone (rho = .43, p << 2 x 10(-6)). However, when the spline-adjusted WBC residuals were used as phenotype, no GWAS significant associations were found. Based on available annotation, the top 50 genetic variants suggested effects on signal transduction, translation initiation, cell development, and proliferation.Conclusion These results indicate that host genome variants do not strongly influence WBC across ALL subsets, and future studies of why some patients are more prone to hyperleukocytosis should be performed within specific ALL subsets that apply more complex analyses to capture potential germline variant interactions and impact on WBC.
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| 17. |
- Helenius, Marianne, et al.
(author)
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Characteristics of white blood cell count in acute lymphoblastic leukemia: A COST LEGEND phenotype-genotype study.
- 2022
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In: Pediatric blood & cancer. - : Wiley. - 1545-5017 .- 1545-5009. ; 69:6
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Journal article (peer-reviewed)abstract
- White blood cell count (WBC) as a measure of extramedullary leukemic cell survival is a well-known prognostic factor in acute lymphoblastic leukemia (ALL), but its biology, including impact of host genome variants, is poorly understood.We included patients treated with the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL-2008 protocol (N = 2347, 72% were genotyped by Illumina Omni2.5exome-8-Bead chip) aged 1-45 years, diagnosed with B-cell precursor (BCP-) or T-cell ALL (T-ALL) to investigate the variation in WBC. Spline functions of WBC were fitted correcting for association with age across ALL subgroups of immunophenotypes and karyotypes. The residuals between spline WBC and actual WBC were used to identify WBC-associated germline genetic variants in a genome-wide association study (GWAS) while adjusting for age and ALL subtype associations.We observed an overall inverse correlation between age and WBC, which was stronger for the selected patient subgroups of immunophenotype and karyotypes (ρBCP-ALL = -.17, ρT-ALL = -.19; p < 3 × 10-4 ). Spline functions fitted to age, immunophenotype, and karyotype explained WBC variation better than age alone (ρ = .43, p << 2 × 10-6 ). However, when the spline-adjusted WBC residuals were used as phenotype, no GWAS significant associations were found. Based on available annotation, the top 50 genetic variants suggested effects on signal transduction, translation initiation, cell development, and proliferation.These results indicate that host genome variants do not strongly influence WBC across ALL subsets, and future studies of why some patients are more prone to hyperleukocytosis should be performed within specific ALL subsets that apply more complex analyses to capture potential germline variant interactions and impact on WBC.
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| 18. |
- Hjelmstedt, Sofia, et al.
(author)
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Facilitators and barriers to return to work and meet financial needs in parents of children with cancer
- 2021
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 68:10
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Journal article (peer-reviewed)abstract
- BACKGROUND: The aim of this study was to explore what facilitators and barriers parents of children with cancer identify for their ability to return to work and meet financial needs.PROCEDURE: Nine focus groups (21 mothers; 11 fathers) were performed across Sweden in 2015 and 2019. A deductive content analysis approach was used. A preconstructed matrix consisting of 12 codes based on previous literature was used to organize the data. The codes were grouped into subcategories, which were abstracted to four generic categories.RESULTS: Facilitators for a return to work were covered in the category "Flexibility and understanding from employers and social services," and barriers in the category "Pressure to return without consideration of the consequences." Facilitators to meeting financial needs were covered in the category "Available public, private, and employer support," and barriers in the category "Lack of organized and efficient support from employers and social services."CONCLUSIONS: The identified barriers suggest that there is room for improvement in the provision of psychosocial support, which relates to a perceived lack of organized support regarding practical, financial, and occupational matters. The results show a need for a coordinated support system that includes major stakeholders, such as the health care, welfare agencies, and employers. Moreover, employers should consider how to implement more workplace flexibility and involvement of occupational health services. Importantly, to be able to return to work and achieve a sustainable financial situation, it is necessary for all stakeholders to recognize the long-term impact of parenting a child with cancer.
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| 19. |
- Holm, Maja, 1982-, et al.
(author)
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Parenting a child with cancer and maintaining a healthy couple relationship : Findings from the Family Talk Intervention
- 2024
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 71:1
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Journal article (peer-reviewed)abstract
- Background Despite the challenges that caring for a child with cancer brings for parents, little knowledge is available concerning the effects on the parents’ couple relationship. Furthermore, few interventions have been designed to support parents in their couple relationship. The aim of this paper was, therefore, to explore parents’ experiences of their couple relationship while having a dependent child with cancer and the support they received from a family-based intervention, the Family Talk Intervention (FTI).Methods Data for this paper were taken from semi-structured interviews performed in a pilot study of the FTI in the context of pediatric oncology. In total, 22 couples were interviewed after participating in the FTI. The interviews were transcribed and analyzed using qualitative content analysis.Results Parents described how maintaining a couple relationship while living with childhood cancer could be very challenging and was not given the highest priority. The FTI was considered a way of providing important support to the couple and a chance for them to sit down together and listen to each other's perspectives on the situation. Parents described that the FTI had helped them gain an increased mutual understanding, sometimes also helping them to realize that they needed more extensive professional support in their relationship.Conclusions Living with childhood cancer and upholding a healthy couple relationship is challenging for parents. The FTI has the potential to support couples, mainly by providing opportunities for parents to communicate with each other. However, some couples may be in need of a tailored clinical intervention.
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| 20. |
- Høgsholt, Stine, et al.
(author)
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Disease-specific hospitalizations among 5-year survivors of Wilms tumor : A Nordic population-based cohort study
- 2021
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In: Pediatric Blood and Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 68:5
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Journal article (peer-reviewed)abstract
- Background: With modern therapy, over 90% of Wilms tumor patients can expect to become long-term survivors, and focus on morbidity and late effects become increasingly important. We provide a novel evaluation and insight to subsequent hospitalizations in 5-year survivors of Wilms tumor. Methods: As part of the Adult Life after Childhood Cancer in Scandinavia (ALiCCS) study, we identified 5-year survivors of Wilms tumor. Based on stratified random sampling, we constructed a population comparison cohort. Outcomes of interest were overall hospitalizations; hospitalizations for specific organ systems and disease-specific categories. Standardized hospitalization rate ratios (SHRR) and absolute excess risks (AER) were calculated. Results: We included 913, 5-year survivors of Wilms tumor and 152 231 population comparisons. Survivors of Wilms tumor had an increased overall risk of being hospitalized (SHRR 1.8; 95% confidence interval (CI) 1.7-2.0). The hospitalization risk was increased within all major organ systems: urinary and genital organs (SHRR 2.5; 95% CI 2.1-3.0), endocrine (SHRR 2.5; 95% CI 1.9-3.3), cardiovascular (SHRR 2.2; 95% CI 1.7-2.9), and gastrointestinal (SHRR 1.5; 95% CI 1.3-1.8). Risks for specific diseases are reported in the study. Conclusions: Survivors of Wilms tumor had higher risks than population comparisons for a wide range of diseases, with the highest risks seen for urinary, endocrine, and cardiovascular disorders. Five to 20 years after the Wilms tumor diagnosis, 43% of survivors had been hospitalized at least once versus 29% of population comparisons. The overall AER was 2.3, which translates into 0.2 extra hospitalizations in 10 years for every Wilms tumor survivor.
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| 21. |
- Ivéus, Kerstin, et al.
(author)
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Family bonding as a result of the family talk intervention in pediatric oncology : Siblings’ experiences
- 2021
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 69:3
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Journal article (peer-reviewed)abstract
- Background: Childhood cancer affects the whole family. Illness-related stressors increase the risk for poor family communication, affecting the family's well-being. Siblings describe worry and poor illness-related information. As there are few evaluated family interventions in pediatric oncology, this study aimed to pilot-test a family-centered intervention, the family talk intervention (FTI), in pediatric oncology. This paper examined the feasibility in terms of acceptability from the siblings' perspectives.Methods: This study derives from a pilot study of 26 families including 37 siblings recruited from one pediatric oncology center. Standard FTI comprises six meetings with the family, led by two interventionists, with the main goal to facilitate family communication on illness-related topics (e.g., prognosis, the invisibility of healthy siblings). This paper focuses on interview and survey data from siblings after participation in FTI. The study is registered at ClinicalTrials.gov (Identifier NCT03650530).Results: The siblings, aged 6 to 24 years, stated that the interventionists made the meetings feel like a safe environment and that it was a relief for the siblings to talk. They reported that FTI helped the family talk openly about illness-related topics, which they felt led to increased family understanding and improved relationships. The siblings described that FTI also helped them with their school situation. The majority of the siblings reported that FTI came at the right time and involved an appropriate number of meetings.Conclusion: According to the siblings, the timing, content, and structure of FTI were appropriate. FTI showed benefits for both the siblings and each family as a whole.
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| 22. |
- Jackmann, Natalja, 1968-, et al.
(author)
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Vitamin D status in children with leukemia, its predictors, and association with outcome
- 2020
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 67:4, s. e28163-
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Journal article (peer-reviewed)abstract
- BACKGROUND: Children and adolescents with leukemia are potentially at high risk of vitamin D inadequacy, which may have clinical relevance for skeletal morbidity, infections, and cancer outcome. This study aimed to evaluate vitamin D status at the time of diagnosis to investigate its predictors and association with overall survival in children with leukemia. PROCEDURE: We included all 295 children and adolescents diagnosed with leukemia at our institution between 1990 and 2016 who had available serum sample from the time of diagnosis. We analyzed serum 25-hydroxyvitamin D and parathyroid hormone levels and correlated them with clinical data. RESULTS: The 25-hydroxyvitamin D level was deficient (< 25 nmol/L), insufficient (25-50 nmol/L), sufficient (50-75 nmol/L), and optimal (> 75 nmol/L) in 6.4%, 26.8%, 39.7%, and 27.1% of the children, respectively. Older age and a more recent time of sampling (calendar year) predicted lower 25-hydroxyvitamin D level. In preschool children (age 6 years), the 25-hydroxyvitamin D level showed significant seasonal variation. CONCLUSION: It remains unclear whether vitamin D supplementation in pediatric leukemia patients will improve outcome.
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| 23. |
- Kieran, Mark W., et al.
(author)
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A global approach to long-term follow-up of targeted and immune-based therapy in childhood and adolescence
- 2021
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In: Pediatric Blood and Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 68:7
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Journal article (peer-reviewed)abstract
- While considerable efforts and progress in our understanding of the long-term toxicities of surgery, radiation and chemotherapy in children with cancer have been made over the last 5 decades, there continues to be a wide gap in our knowledge of the long-term health impact of most novel targeted and immunotherapy agents. To address this gap, ACCELERATE, a multi-stakeholder collaboration of clinical and translational academics, regulators from the EMA and FDA, patient/family advocates and members spanning small biotechnology through to large pharmaceutical companies have initiated the development of an international long-term follow-up data registry to collect this important information prospectively. Providing critical safety data on the long-term use of these approved and investigational therapies in children will support the regulatory requirements and labeling information. It will also provide the necessary insight to help guide physicians and families on the appropriateness of a targeted or immune therapy for their child and inform survivorship planning.
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| 24. |
- Koscielniak, Ewa, et al.
(author)
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Extraskeletal Ewing sarcoma in children, adolescents, and young adults. An analysis of three prospective studies of the Cooperative Weichteilsarkomstudiengruppe (CWS)
- 2021
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 68:10
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Journal article (peer-reviewed)abstract
- Background We have analyzed the outcome of patients with localized extraskeletal Ewing sarcoma (EES) treated in three consecutive Cooperative Weichteilsarkomstudiengruppe (CWS) soft tissue sarcoma (STS) studies: CWS-91, CWS-96, and CWS-2002P. Methods Patients were treated in CWS-91 with four- (vincristine, dactinomycin, doxorubicin, and ifosfamide [VAIA] or cyclophosphamide [VACA II]) or five-drug (+etoposide [EVAIA]) cycles, in CWS-96 they were randomly assigned to receive VAIA or CEVAIE (+carboplatin and etoposide), and in CWS-2002P with VAIA III plus optional maintenance therapy (MT) with cyclophosphamide and vinblastine. Local therapy consisted of resection and/or radiotherapy (RT). Results Two hundred forty-three patients fulfilled the eligibility criteria. The 5-year event-free survival (EFS) and overall survival (OS) were 63% (95% confidence interval [CI] 57-69) and 73% (95% CI 67-79), respectively. The 5-year EFS by study was 64% (95% CI 54-74) in CWS-91, 57% (95% CI 48-66) in CWS-96, and 79% (95% CI 67-91) in CWS-2002P (n.s.). The 5-year OS was 72% (95% CI 62-82) in CWS-91, 70% (95% CI 61-79) in CWS-96, and 86% (95% CI 76-96) in CWS-2002P (n.s.). In CWS-96, 5-year EFS and OS in the VAIA arm versus the CEVAIE were 65% (95% CI 52-81) versus 55% (95% CI 39-76) log-rank p = .13, and 85% (95% CI 75-96) versus 61% (95% CI 45-82), log-rank p = .09. Conclusion Our analysis provides interesting information on the treatment and specificities of EES, which can be useful for a better understanding of this rare entity and should be considered in the development of future clinical trials for Ewing sarcoma defined as FET-ETS fusion positive tumors.
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| 25. |
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| 26. |
- Lönnerblad, Malin, et al.
(author)
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A nationwide, population‐based study of school grades, delayed graduation, and qualification for school years 10‐12, in children with brain tumors in Sweden
- 2020
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 67:2
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Journal article (peer-reviewed)abstract
- BackgroundAs many as 95.7% of children diagnosed with a brain tumor will experience persistent late effects as adults. These include difficulties with general executive functions, lower IQ, and mental fatigue, which may negatively affect school performance.MethodsThrough the Swedish Childhood Cancer Registry, we identified 475 children born between 1988 and 1996, diagnosed with a brain tumor before their 15th birthday. School grades in “Swedish,” “mathematics,” and “English,” if their graduation was delayed, and qualification for school years 10‐12 were compared with 2197 matched controls. Furthermore, we checked for interaction effects between sex and age at diagnosis, and possible effects of tumor grade (high or low) as well as parents’ education.ResultsChildren treated for a brain tumor performed worse in the subjects compared to controls and also had delayed graduation to a greater extent. Fewer children treated for a brain tumor than controls qualified for school years 10‐12. Children treated at a young age, especially females, and children whose parents have low education seem to be at particular risk. Unexpectedly, there were no differences in outcomes between survivors with high‐ and low‐grade tumors.ConclusionsIt is important that schools provide regular pedagogical assessment and individualized support to meet the different needs of children treated for a brain tumor. Children treated for low‐grade tumors do not perform better than children treated for high‐grade tumors, despite the lighter treatment, and hence require the same attention and support.
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| 27. |
- Lövgren, Malin, 1980-, et al.
(author)
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Much is left unspoken : Self-reports from families in pediatric oncology
- 2020
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017.
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Journal article (peer-reviewed)abstract
- Background: Communication about illness-related subjects is complex and difficult. To support entire families in pediatric oncology, health care professionals need to know what family members think, but leave unspoken. The aim of this study was to explore how families in pediatric oncology experienced illness-related information and communication with professionals and within the family.Procedure: A cross-sectional web survey was used. Families were recruited from one pediatric oncology center in Sweden, 2-3 months after diagnosis. One hundred eighteen family members (ill children, siblings, and parents) representing 27 families filled out age-adapted surveys.Results: Eighty-six percent of the parents and 71% of the siblings reported that theyhad not received enough or any information about how the cancer and its treatment could affect the child’s psychological health. The families reported that they did not dare ask professionals questions about psychosocial issues and future-related subjects.Nor did they talk with one another, even though 55% of the parents and 24% ofthe children wanted to reveal more about how they felt to someone in the family. The parents reported the lowest family communication, and few families had all members reporting the same perception of family communication. Conclusions: Much is still left unspoken in pediatric oncology and the needs of the families are prominent. Assessments of each family member’s needs might form a basis for professionals to give each person adequate information and family support. An increased awareness in families about family members’ different needs might lead to mutual understanding.
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| 28. |
- Martinsson, U., et al.
(author)
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Why are not ALL Paediatric Patients Treated With Protons? : A Complete National Cohort From Sweden 2016-2019
- 2020
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 67:S4, s. S79-S79
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Journal article (other academic/artistic)abstract
- Background and Aims: A proton therapy (PT) facility – Skandion clinic- opened in Uppsala in august 2015. It was stated that all children inSweden, benefitting from PT, ought to be sent there. PT plans are prepared at six university-based radiotherapy (RT) centres and assessedby a national board. There are no additional costs for the families compared to other radiotherapy (RT) options, not even for travel and lodging.Methods: Since 2008 all children receiving RT with any modality areregistered in the Swedish Radtox registry. Inclusion is populationbased, prospective and complete. Radiation oncologists from the sixcentres performing paediatric RT retrospectively reviewed patientswho had not received PT.Results: 354 treatments were given, 252 (71%) were not PT. Thereasons for choosing conventional RT were dismal prognosis in 66patients, uncertainty due to internal movement and lack of motioncontrol technique at the PT centre in 63 patients, and lack of dosimetric advantage for protons in 47 patients. Infrequent reasons were lackof set up for CSI or very superficial treatment in the beginning [n=11],variations of air in the field [n=6], not robust treatment plan for otherreasons (mainly metal in the field) [n=6], gamma-knife/other stereotactic treatment [n= 5], brachytherapy [n=4], TBI [n=31], acute RT startnecessary [n=10], and social reasons [n=3].Conclusions: Even though proton therapy, due to less side effects,has been advocated to be the best treatment modality for children, this might not always be the case. Sometimes conventional RTmay be advantageous, despite the increased exit-dose, due to thewider penumbra of PT. Social needs and palliative situations may bemore important than an optimal dose-distribution. However, technical improvement of PT by application of gating and treatment planningsystems (TPS) handling dose-deposition uncertainties should make themodality available for a wider range of paediatric patients.
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| 29. |
- Mogensen, Nina, et al.
(author)
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Parental experiences of the informed consent process in randomized clinical trials-A Nordic study
- 2023
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 70:12
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Journal article (peer-reviewed)abstract
- BackgroundRandomized clinical trials (RCTs) are an essential part of improving acute lymphoblastic leukemia (ALL) treatment. This population-based questionnaire study investigated parents' experiences of the informed consent process in the RCTs within the Nordic NOPHO (Nordic Society of Paediatric Haematology and Oncology) ALL2008 trial.ProcedureParents in Sweden, Denmark, and Finland whose child was alive and in first remission after end of therapy and who were asked to participate in any RCT in the ALL2008 protocol, were asked to complete 15 questions/items regarding their experience of the RCT consent process.ResultsA total of 483 parents of 279 children met the inclusion criteria and answered the study questionnaire. Most (91%) agreed/strongly agreed to having received sufficient information to make a well-informed decision, felt confidence in the study design (86%), and thought that the process was satisfactory (86%). Those who did not consent reported a generally more negative experience of the process. More than a third of all parents and over half of parents who had refused participation felt that it was burdensome to decide. Most parents (66%) in general, and one-third of those with children 8 years or older, reported that their child was not involved in the process.ConclusionsParents were in general satisfied with the informed consent process, although many parents, particularly those who refused participation, reported it as burdensome to make the decision concerning RCT. Fewer than expected of the school-aged children were involved in the decision process, which calls for attention on how children are included in the consent procedure in clinical trials.
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| 30. |
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| 31. |
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| 32. |
- Norbäck, Kajsa, et al.
(author)
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Recruiting children with cancer to research : A qualitative interview study exploring ethical values and challenges among Swedish health care professionals and researchers
- 2022
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 69, s. s555-s555
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Journal article (other academic/artistic)abstract
- Background and Aims: Research remains crucial to improve treatment, survival, and quality of life for children with cancer. However, recruitment of children to research raises ethical challenges. This study aimed to explore ethical values and challenges in recruitment of children with cancer to research, among health care professionals and researchers in Sweden. Another aim was to explore health care professionals and researcher’s perceptions of ethical competence in the context of recruiting children to research.Methods: An explorative qualitative design, using semi-structured interviews with seven pediatric oncologists and ten nurses. Interviews were analyzed with inductive qualitative content analysis.Results: The analysis resulted in five categories: Establishing relationships and trust, Meeting informational needs, Acknowledging vulnerability, Balancing roles and interests, and Ensuring ethical competence. Health care professionals and researchers described care-based, research-based and children’s rights-based ethical values in recruitment. Further, they reported ethical challenges related to informed consent, vulnerability, and shared decision-making. They relied on research ethical principles and regulations but also reasoned from ethics of care and virtue ethics perspectives.Conclusions: Health care professionals and researchers are guided by care-and research ethical values, and report ethical challenges in recruitment. There is a need to highlight ethical aspects of pediatric research. Promoting research ethical competence among health care professionals and researchers may reduce moral distress and ensure ethical quality in pediatric research.
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| 33. |
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| 34. |
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| 35. |
- Rask, Olof, et al.
(author)
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Prospective registration of symptoms and times to diagnosis in children and adolescents with central nervous system tumors: A study of the Swedish Childhood Cancer Registry
- 2022
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 69:11
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Journal article (peer-reviewed)abstract
- Background The elapsed time taken to diagnose tumors of the central nervous system in children and adolescents varies widely. The aim of the present study was to investigate such diagnostic time intervals at a national level in Sweden as they correlate with clinical features. Methods Data prospectively accumulated over a 4-year period in the Swedish Childhood Cancer Registry from patients aged 0-18 years were pooled, and diagnostic time intervals were analyzed considering tumor location, tumor type, patient age and sex, initial symptoms, and clinical timelines. All six pediatric oncology centers in Sweden contributed to collection of data. Time points for calculating the total diagnostic interval (TDI) defined as the time from symptom onset to diagnosis were reported in 257 of 319 patients (81%). Results The time from symptom onset to the first healthcare consultation, median 2.6 weeks, did not vary significantly between patients categorized according to tumor type or location. The median TDI was 8.3 weeks for the 4-year study period. Patients with optic pathway glioma (TDI 26.6 weeks), those with tumors of the spinal cord (TDI 25.9 weeks), and those with midline tumors (TDI 24.6 weeks) had the longest lead times. Additionally, older age, too few initial symptoms, and seeking initial redress outside an emergency ward were factors associated with a longer time to diagnosis. Conclusion This study identified several factors associated with delayed diagnosis of central nervous system tumors among Swedish children and adolescents. These novel data ought to help direct future efforts toward clinical improvement.
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| 36. |
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| 37. |
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| 38. |
- Scheer, Monika, et al.
(author)
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Low-grade fibromyxoid sarcoma : A report of the Cooperative Weichteilsarkom Studiengruppe (CWS)
- 2020
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In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017. ; 67:2
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Journal article (peer-reviewed)abstract
- Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with benign histologic appearance, though fully malignant behavior is possible.Methods: Patients with LGFMS<21 years registered in Cooperative Weichteilsarkom Studiengruppe trials until 2017 were analyzed. Firstline treatment consisted of complete surgical resection whenever possible.Results: Median age of 31 patients was 10.9 years (first month to 17.1 years). Twenty-six tumors were confirmed to the tissue of origin (T1), four invaded contiguous structures (T2), one was TX. Eight were >5 cm. The best surgical result was resection with free margins (R0) in 24 and microscopic residuals (R1) in seven. Five-year event-free (EFS), 5-year local-relapse-free (LRFS), and 5-year overall-survival were 71 +/- 18.6% confidence interval (CI) 95%, 76 +/- 17.6% CI 95%, and 100%, respectively. Six patients suffered local relapse in a median of 1 year, one combined within 1.3 year and one metastatic relapse with lesions in the lung, back muscles, and thigh discovered in whole-body imaging 6 years after the first diagnosis. In univariate analysis, T status correlated with EFS (T1 79.6 +/- 18.6%, T2 50.0 +/- 49.0%, P = .038). Resection with free margins tends to be associated with better LRFS (R0 82.4 +/- 18.6%, R1 53.6 +/- 39.4%, P = .053). Among 24 patients with R0 resection, five (21%) suffered relapse, thereof three local, one metastatic, and one combined. Among seven patients with R1-resection, three (43%) suffered local relapse.Conclusion Special caution is advisable in T2 tumors. The metastatic potential with lesions in unusual sites indicates that affected patients need to be informed. If long-term follow-up with whole-body imaging is beneficial, it may be addressed in larger intergroup analyses. Further research in disease biology is essential for optimal treatment and follow-up care.
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| 39. |
- Sparber-Sauer, Monika, et al.
(author)
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Endothelial cell malignancies in infants, children and adolescents : Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
- 2020
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In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017. ; 67:3
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Journal article (peer-reviewed)abstract
- Background Endothelial cell malignancies are extremely rare in childhood. New identification of genetic abnormalities (WWTR1:CAMTA1 translocation) helps to recognize potential therapeutic targets. Little is known about treatment and outcome of these patients. Methods Clinical course, treatment, and outcome in patients with endothelial cell malignancies treated within the Cooperative Weichteilsarkom Studiengruppe (CWS) trials CWS-91, -96, -2002P, and the Soft-Tissue Sarcoma Registry (SoTiSaR) were analyzed (1991-2019). Results Patients had angiosarcoma (AS) (n = 12), malignant epithelioid hemangioendothelioma (EHE) (n = 16), and kaposiform hemangioendothelioma (KHE) (n = 13). The median age was 5.39 years (range, 0.8-17.34); 33 patients had localized disease (LD), and 8 patients had metastatic disease. Therapy consisted of chemotherapy (CHT) (AS n = 8, EHE n = 9, KHE n = 5), interferon or new agent therapy (EHE n = 5, 2 KHE n = 2), microscopically or macroscopically complete resection (AS n = 3, EHE n = 6, KHE n = 3), and radiotherapy (AS n = 6, EHE n = 2, KHE n = 1). Two patients (KHE) had watch-and-wait strategy resulting in stable disease. Complete remission (CR) was achieved in AS (10/12; 83%), EHE (10/16; 63%), and KHE (5/13; 38%). The five-year EFS and OS for patients with AS was 64% (+/- 29 CI 95%) and 80% (+/- 25, CI 95%), with EHE 62% (+/- 24, CI 95%) and 78% (+/- 23, CI 95%), with KHE 33% (+/- 34, CI 95%) and 92% (+/- 15, CI 95%), respectively. Complete resection was a significant prognostic factor for AS, LD for EHE. Conclusions Endothelial cell malignancies in childhood have a fair outcome with multimodal treatment. New treatment options are needed for metastic disease.
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| 40. |
- Sparber-Sauer, Monika, et al.
(author)
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Infantile myofibromatosis : Excellent prognosis but also rare fatal progressive disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:3
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Journal article (peer-reviewed)abstract
- Background: Infantile myofibromatosis (IM) is a rare benign soft tissue tumor and often a self-limiting disease but rarely includes life-threatening complications. Little is known about optimal treatment of primary localized (LD) and multifocal disease (MFD).Methods: Treatment and outcome of 95 children with IM registered within five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry (1981-2016) were evaluated.Results: LD was diagnosed in 71 patients at a median age of 0.4 years (range 0.0-17.7). MFD was present in 24 patients. The mainstay of treatment was watch-and-wait strategy (w&w) after initial biopsy or resection. Low-dose chemotherapy (CHT) was administered to 16/71 (23%) patients with LD and eight of 24 (33%) patients with MFD, imatinib was added in two. A delayed resection was possible in eight of 71 (11%) and five of 24 (21%) patients with LD and MFD, respectively. Overall, patients were alive in complete remission (n = 77) and partial remission (n = 10) at a median follow-up time of 3.4 years after diagnosis (range 0.01-19.4); no data available (n = 5). Three patients died of progressive disease (PD) despite CHT. Gender, tumor size, and location correlated with a favorable event-free survival (EFS) in patients with LD. The 5-year EFS and overall survival of patients with LD were 73% (±12, confidence interval [CI] 95%) and 95% (±6, CI 95%), respectively; for MFD 51% (±22, CI 95%) and 95% (±10, CI 95%).Cconclusion: Prognosis is excellent in patients with LD and MFD. Targeted treatment needs to be evaluated for rare fatal PD.
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| 41. |
- Sparber-Sauer, Monika, et al.
(author)
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Long-term results from the multicentric European randomized phase 3 trial CWS/RMS-96 for localized high-risk soft tissue sarcoma in children, adolescents, and young adults
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:9
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Journal article (peer-reviewed)abstract
- Background: CWS/RMS-96 was an international multicenter trial with randomization between two therapy arms of the standard four-drug therapy (vincristine, ifosfamide, adriamycin, dactinomycin [VAIA]) versus an intensified six-drug regimen (carboplatin, epirubicin, vincristine, dactinomycin, ifosfamide, and etoposide [CEVAIE]) for high-risk rhabdomyosarcoma (RMS), extraskeletal Ewing sarcoma (EES), and undifferentiated sarcoma (UDS) in children, adolescents, and young adults aiming to improve their survival. Intensified chemotherapy with CEVAIE did not improve outcome. Methods: Patients younger than 21 years with a previously untreated localized HR-RMS, EES, and UDS were enrolled from Cooperative Weichteilsarkom Studiengruppe (CWS) centers in Germany, Austria, Poland, Switzerland, and from Italian Soft Tissue Sarcoma Committee (STSC) centers. Randomization (1:1) to receive either 9 x 21 days cycles of VAIA or CEVAIE was performed separately in CWS and STSC. Hyperfractionated accelerated radiotherapy (32-44.8 Gy) was added at week 9-12 according to histology and response to chemotherapy. A secondary microscopically complete nonmutilating resection was performed if possible. Primary endpoints were response to chemotherapy, event-free (EFS) and overall survival (OS). Results: Five hundred fifty-seven patients (HR-RMS: n = 416, EES and UDS: n = 141) underwent randomization: VAIA (n = 273) or CEVAIE (n = 284). Radiotherapy was given to 70% of patients in both groups. A secondary resection was performed in 47% and 48% patients, respectively. The 5-year EFS and OS for the VAIA and CEVAIE treatment arms were 59.8% and 60.8% (p = .89), and 74.2% and 68.3% (p = .16), respectively. No differences in response, toxicity, or second malignancies emerged in the two groups. Conclusion: The use of an intensified regimen failed to show a significant improvement in tumor response and outcome of patients with localized HR-RMS, EES, and UDS.
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| 42. |
- Sparber-Sauer, Monika, et al.
(author)
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Radiotherapy and long-term sequelae in pediatric patients with parameningeal rhabdomyosarcoma : Results of two Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
- 2024
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 71:1
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Journal article (peer-reviewed)abstract
- BackgroundParameningeal location of rhabdomyosarcoma (PM RMS) is known to be an unfavorable prognostic factor. Scarce data are available on radiotherapy (RT) concepts with regard to outcome.MethodsTreatment and outcome of 395 children with PM RMS registered within two Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry (1995-2021) were evaluated.ResultsPatients were IRS group II (n = 15) and III (n = 380) and received systemic treatment according to the enrolled protocols: I2VA (n = 172), VAIA/CEVAIE (n = 223). Delayed resection was performed in 88/395 (22%) patients, and RT was additionally given in 79/88 (90%) resected patients. RT was the predominant local treatment in 355/395 (90%) patients: hyperfractionated accelerated photon (HART; n = 77), conventionally fractionated photon (n = 91) or proton beam (n = 126), brachytherapy (n = 4), heavy ions (n = 1), not available (n = 56). In the subgroup of RT as only local treatment (n = 278), no intracranial tumor extension and complete remission at end of treatment were significant positive prognostic factors. No significant difference on tumor outcome was seen between different radiotherapy concepts. Long-term toxicity with mostly endocrinological and visual deficiencies was reported in 161/279 (58%) surviving patients with a lower trend after proton beam RT (48%) when compared to HART or conventionally fractionated photon RT (71% and 72%, respectively). Ten-year event-free and overall survival in the overall group were 62% (+/- 5, 95% confidence interval [CI]) and 67% (+/- 5, 95% CI); in the RT-only group 67% (+/- 6, 95% CI) and 71% (+/- 6, 95% CI), respectively.ConclusionCWS data confirm the recent RT concept in PM RMS. Long-term sequelae as endocrinological and visual deficiencies need to be addressed.
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| 43. |
- Sundberg, Emil, 1990-, et al.
(author)
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COVID-19 seroprevalence and clinical picture in Swedish pediatric oncology and hematology patients
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:10
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Journal article (peer-reviewed)abstract
- BackgroundChildren develop symptomatic coronavirus disease 2019 (COVID-19) more rarely than adults upon infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Pediatric oncology and hematology patients may be at increased risk of severe COVID-19 due to their underlying disease or treatment. We investigated COVID-19 and seroprevalence of anti-SARS-CoV-2 antibodies, respectively, in a Swedish cohort of pediatric oncology and hematology patients.ProcedurePatients (n = 136) were recruited between June 2020 and September 2021 at Uppsala University Children's Hospital, Sweden. Up to six consecutive blood samples per patient were analyzed for wild-type anti-S1 IgM and IgG antibodies (including after vaccination, n = 4). Clinical data on COVID-19 (including polymerase chain reaction [PCR] test results) were collected from electronic medical records. A questionnaire was completed at recruitment.ResultsA cumulative seroprevalence (IgM and IgG) of 33% (45/136 patients, 95% confidence interval: 25%–41%) was observed in this patient cohort, of whom 66% (90/136 patients) were under severe immunosuppressive treatment during the study period. Increasing patient age (p = .037) and PCR test results (p < .002) were associated with seropositivity in nonvaccinated cases. Most seropositive, nonvaccinated cases (32/43, 74%) were never PCR-verified for SARS-CoV-2 infection. Of the 13 patients with PCR-verified infection, nine (69%) reported mild disease. A majority (63%) reported continued school attendance during the pandemic.ConclusionsSwedish pediatric oncology and hematology patients developed antibodies against SARS-CoV-2, despite their diagnosis and/or treatment, and the observed seroprevalence was similar to that in national pediatric outpatients. PCR-verified cases underestimate the true incidence of COVID-19 in this patient cohort.
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| 44. |
- Sundberg, Emil, 1990-, et al.
(author)
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Low numbers of COVID-19 in Swedish pediatric oncology patients during the first pandemic year despite an open society
- 2022
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 69:10
-
Journal article (peer-reviewed)abstract
- Background Sweden adopted a different strategy than many other countries to combat the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic and kept most schools open. Initial reports from China suggested that coronavirus disease 2019 (COVID-19) was milder in children compared to adults, but there was a lack of data from immunocompromised children. Therefore, we investigated the rate of verified SARS-CoV-2 infections in our Swedish pediatric oncology patients.Procedure This was a multicenter retrospective study. A questionnaire including patient data as well as SARS-CoV-2 data was sent to the six Swedish childhood cancer centers in May 2021.Results During the first pandemic year, 49 patients were identified as SARS-CoV-2 positive, and 22 (45%) children were hospitalized with COVID-19. Two children needed intensive care, but no COVID-19-related deaths were reported. Most patients (n = 36, 73%) were on active chemotherapy treatment and 23 children (49%) attended school or daycare at least part-time. Half of the SARS-CoV-2-positive patients experienced a delay in cancer treatment.Conclusions Despite the rapid spread of SARS-CoV-2 in Sweden, without a strict lockdown of the society, the number of nationally reported pediatric oncology patients with polymerase chain reaction (PCR)-verified infection was low, and the majority of children had mild disease. Our data show that treatment interruptions occurred frequently and this should clearly be avoided for the coming years.
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| 45. |
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| 46. |
- Sørensen, Gitte V., et al.
(author)
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Late mortality among survivors of childhood acute lymphoblastic leukemia diagnosed during 1971–2008 in Denmark, Finland, and Sweden : A population-based cohort study
- 2022
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In: Pediatric Blood and Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 69:1
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Journal article (peer-reviewed)abstract
- Objective: Investigate all-cause and cause-specific late mortality after childhood acute lymphoblastic leukemia (ALL) in a population-based Nordic cohort. Methods: From the cancer registries of Denmark, Finland, and Sweden, we identified 3765 five-year survivors of ALL, diagnosed before age 20 during 1971–2008. For each survivor, up to five matched comparison subjects were randomly selected from the general population (n = 18,323). Causes of death were classified as relapse related, health related, and external. Late mortality was evaluated by cumulative incidences of death from 5-year survival date. Mortality hazard ratios (HR) were evaluated with Cox proportional models. Results: Among the survivors, 315 deaths occurred during a median follow-up of 16 years from 5-year survival date (range 0–42). The majority were attributable to relapse (n = 224), followed by second neoplasm (n = 45). Cumulative incidence of all-cause late mortality at 15 years from diagnosis decreased gradually over treatment decades, from 14.4% (95% confidence interval [CI]: 11.6–17.2) for survivors diagnosed during 1971–1981, to 2.5% (95% CI: 1.3–3.7) for those diagnosed during 2002–2008. This was mainly attributable to a reduction in relapse-related deaths decreasing from 13.4% (95% CI: 10.7–16.1) for survivors diagnosed during 1971–1981 to 1.9% (95% CI: 0.9–2.8) for those diagnosed during 2002–2008. Health-related late mortality was low and did not change substantially across treatment decades. Compared to comparison subjects, all-cause mortality HR was 40 (95% CI: 26–61) 5–9 years from diagnosis, and 4.4 (95% CI: 3.4–5.6) ≥10 years from diagnosis. Conclusions: Survivors of ALL have higher late mortality than population comparison subjects. Among the survivors, there was a temporal reduction in risk of death from relapse, without increments in health-related death.
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| 47. |
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| 48. |
- Verbruggen, Lisanne C., et al.
(author)
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Guidance regarding COVID-19 for survivors of childhood, adolescent, and young adult cancer : A statement from the International Late Effects of Childhood Cancer Guideline Harmonization Group
- 2020
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In: Pediatric Blood and Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 67:12
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Journal article (peer-reviewed)abstract
- Childhood, adolescent, and young adult (CAYA) cancer survivors may be at risk for a severe course of COVID-19. Little is known about the clinical course of COVID-19 in CAYA cancer survivors, or if additional preventive measures are warranted. We established a working group within the International Late Effects of Childhood Cancer Guideline Harmonization Group (IGHG) to summarize existing evidence and worldwide recommendations regarding evidence about factors/conditions associated with risk for a severe course of COVID-19 in CAYA cancer survivors, and to develop a consensus statement to provide guidance for healthcare practitioners and CAYA cancer survivors regarding COVID-19.
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| 49. |
- Virgone, C., et al.
(author)
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Adrenocortical tumours in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations
- 2021
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 68:S4
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Journal article (peer-reviewed)abstract
- Adrenocortical tumours (ACTs) are rare during childhood. A complete surgical resection provides the best chance of cure, but the role and efficacy of the adjuvant therapy are still controversial. Various histologic criteria of malignancy for ACTs adopted in children do not facilitate comparative studies and are not completely shared. Therefore, a sharp demarcation between benign and malignant lesions has not been recognised, making it difficult to identify who potentially needs perioperative therapy. This manuscript presents the internationally harmonised recommendations for the diagnosis and treatment of ACTs in children and adolescents, established by the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) group within the EU-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).
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| 50. |
- Walz, Amy L., et al.
(author)
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Tumor biology, biomarkers, and liquid biopsy in pediatric renal tumors
- 2023
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In: Pediatric Blood and Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 70:S2
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Research review (peer-reviewed)abstract
- The expansion of knowledge regarding driver mutations for Wilms tumor (WT) and malignant rhabdoid tumor of the kidney (MRT) and various translocations for other pediatric renal tumors opens up new possibilities for diagnosis and treatment. In addition, there are growing data surrounding prognostic factors that can be used to stratify WT treatment to improve outcomes. Here, we review the molecular landscape of WT and other pediatric renal tumors as well as WT prognostic factors. We also review incorporation of circulating tumor DNA/liquid biopsies to leverage this molecular landscape, with potential use in the future for distinguishing renal tumors at the time of diagnosis and elucidating intratumor heterogeneity, which is not well evaluated with standard biopsies. Incorporation of liquid biopsies will require longitudinal collection of multiple biospecimens. Further preclinical research, identification and validation of biomarkers, molecular studies, and data sharing among investigators are crucial to inform therapeutic strategies that improve patient outcomes.
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