Society Of Pediatric Oncology Research Articles

Diagnosis and Treatment of Iron Deficiency and Iron Deficiency Anemia in Children and Adolescents: Recommendations of the Polish Pediatric Society, the Polish Society of Pediatric Oncology and Hematology, the Polish Society of Neonatology, and the Polish Society of Family Medicine

Background/Objectives. Iron deficiency is one of the most common nutritional deficiencies worldwide and is the leading cause of anemia in the pediatric population (microcytic, hypochromic anemia due to iron deficiency). Moreover, untreated iron deficiency can lead to various systemic consequences and can disrupt the child’s development. Methods/Results. Therefore, a team of experts from the Polish Pediatric Society, the Polish Society of Pediatric Oncology and Hematology, the Polish Neonatology Society, and the Polish Society of Family Medicine, based on a review of the current literature, their own clinical experience, and critical discussion, has developed updated guidelines for the diagnosis, prevention, and treatment of iron deficiency in children from birth to 18 years of age. These recommendations apply to the general population and do not take into account the specifics of individual conditions and diseases.

Perceptions of physicians caring for pediatric patients with cancer in Europe: insights into the use of palliative care, its timing, and barriers to early integration

BackgroundIntegrating pediatric palliative care (PPC) into pediatric oncology standard care is essential. Therefore, it is important to assess physicians’ knowledge and perceptions of PPC to optimize its practice.ObjectiveTo evaluate the knowledge, comfort levels, and perspectives of physicians regarding the timing and perceived barriers to integrating PPC into pediatric cancer care across Europe.DesignThe Assessing Doctors’ Attitudes on Palliative Treatment (ADAPT) survey, originally developed for other global regions, was culturally and contextually adapted for Europe.Setting/SubjectsThe survey was distributed via the European Society of Paediatric Oncology (SIOPE) membership listserv. Any physicians caring for children with cancer across Eastern, Southern, Central, and Northern Europe were invited to complete the survey.ResultsA total of 198 physicians from 29 European countries completed the ADAPT survey. Physicians demonstrated relative agreement with the World Health Organization’s guidance; median alignment was 83.4% (range 59.9%-94.1%). Although most respondents felt comfortable addressing physical (84.4%) and emotional (63.4%) needs, they felt less comfortable addressing spiritual needs (41.9%) and providing grief and bereavement support (48.5%). There were significant regional differences, such as physicians in Eastern and Southern Europe reporting a lack of PPC specialists, opioids, and home-based care, while those in Northern and Central Europe did not.ConclusionPhysicians caring for children with cancer throughout Europe have a good understanding of PPC. However, misconceptions about PPC persist, requiring educational and capacity-building efforts. Additionally, the regional differences in perceived barriers must be addressed to ensure equitable access to PPC for all European children with cancer.

Outcome of childhood ALK-positive anaplastic large cell lymphoma relapses: Real-life experience of the French Society of Pediatric Oncology (SFCE) cohort of 75 French children.

To describe treatments and outcomes of French children treated for relapsed/refractory anaplastic lymphoma kinase-positive anaplastic large cell lymphoma (ALK+ ALCL). We conducted the analysis of a series of 75 French children treated for a first relapsed/refractory ALK+ ALCL between 1999 and 2017. The median time to first relapse was 8.1months from initial diagnosis (2.9 after end of treatment), with 12 relapses during frontline treatment or within 1month of the end of treatment. Treatment of the first relapse varied according to the period of time and risk factors: 48 received multiagent chemotherapy, including 21 and 19 consolidated with allogeneic stem cell transplantation (SCT) and autologous-SCT, respectively. Twenty-one patients received weekly vinblastine, and six received ALK inhibitors (ALKi). Overall, 64/75 patients reached a second complete remission (CR2). Eight out of 11 patients who did not reach CR2 died and the other three were rescued with ALKi, vinblastine, and nivolumab. With a median follow-up of 8.2years, 60 patients are alive, 43 in CR2, 15 in CR3, two in CR4; and 15 patients died, six from toxicity and nine from disease progression. The 5-year event-free survival and overall survival after first relapse were 51.7% (95% confidence interval [CI]: 39.6%-62.6%) and 80.7% (95% CI: 69.6%-88.1%), respectively. Time to relapse greater than 12months from initial diagnosis was proven to be a prognostic factor in relapsed/refractory ALK+ ALCL. In relapsed ALK+ ALCL, high survival rate can be reached with various therapeutic strategies. The main challenge remains to prevent subsequent relapses, and to lower long-term morbidity.

Pediatric oncology nutritional practices in high-income countries: A survey from the International Society of Paediatric Oncology (SIOP).

Optimal nutrition in pediatric oncology can influence cancer-related outcomes. To establish an understanding of nutrition practice and perceptions of best practice, we queried nutrition providers practicing in pediatric oncology care centers in high-income countries. An electronic, multidisciplinary, cross-sectional survey of nutrition practices was conducted among pediatric oncology nutrition practitioners. Final analysis included 110 surveys from 71 unique institutions and included practitioners from Europe, the United States, Canada, Australia/New Zealand, South America, and the Middle East/Asia. The majority of institutions (97%) reported having dietitians; 72% had designated oncology dietitians. Approximately half of the practitioners (47%) reported feeling their institutions were inadequately staffed. The majority (78%) of institutions completed nutrition risk screening, but there was no consensus on specific screening practices. Half (50%) of the institutions that screened for nutrition risk did so in both inpatient and outpatient settings. The majority (80%) of institutions completed a nutrition assessment close to the time of diagnosis. Those that did not cite lack of staff and/or lack of time, lack of standardized approach, and consult only level of nutritional care as primary barriers. The most common topic of nutrition education provided to patients/families was nutrition-related symptom management (68%). While most institutions reported having pediatric oncology dietitians, we found a lack of standardized practice and perceived inadequate staffing. In addition, what providers perceived to be best practice did not always align with day-to-day clinical practice. Ongoing efforts are needed to develop evidence-based guidelines, including staffing recommendations, to support specialized care in this population.

Upregulation of Insulin-like Growth Factor-I in Response to Chemotherapy in Children with Acute Lymphoblastic Leukemia.

The treatment of childhood cancer is challenged by toxic side effects mainly due to chemotherapy-induced organ damage and infections, which are accompanied by severe systemic inflammation. Insulin-like growth factor I (IGF-I) is a key regulating factor in tissue repair. This study investigated associations between the circulating IGF-I levels and chemotherapy-related toxicity in pediatric acute lymphoblastic leukemia (ALL). In this prospective study, we included 114 patients (age: 1-17 years) with newly diagnosed ALL treated according to The Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL2008 protocol between 2013 and 2018. The patients' plasma levels of IGF-I, and the primary binding protein, IGFBP-3, were measured weekly during the first six weeks of treatment, including the induction therapy. The patients' systemic inflammation was monitored by their C-reactive protein (CRP) and interleukin (IL)-6 levels and their intestinal epithelial damage by their plasma citrulline levels. IGF-I and IGFBP-3 were converted into sex-and age-adjusted standard deviation scores (SDS) using 1621 healthy children as reference. At ALL diagnosis, IGF-I levels were decreased (median (quartiles): -1.2 SDS (-1.9 to -0.5), p = 0.001), but increased significantly following the initiation of chemotherapy, peaking on day 8 (0.0 SDS (from -0.8 to 0.7), p < 0.001). This increase correlated with the levels of CRP (rho = 0.37, p < 0.001) and IL-6 (rho = 0.39, p = 0.03) on day 15, when these markers reached maximum levels. A larger IGF-I increase from day 1 to 15 correlated with a slower recovery rate of the intestinal damage marker citrulline from day 15 to 29 (rho = -0.28, p = 0.01). Likewise, IGFBP-3 was reduced at diagnosis, followed by an increase after treatment initiation, and was highly correlated with same-day IGF-I levels. This study demonstrates a chemotherapy-induced increase in IGF-I, with a response that appears to reflect the severity of tissue damage and systemic inflammation, preceding CRP and IL-6 increases. IGF-I may have potential as an early reactive biomarker for acute toxicity in patients with ALL.

Paediatric oncology physiotherapy in Africa: International Society of Paediatric Oncology (SIOP) Global Mapping Programme.

Childhood cancer treatment in Africa has a dramatically increasing patient population resulting in greater rehabilitation needs. The International Society of Paediatric Oncology (SIOP) mapped childhood cancer services in Africa including access to physiotherapy. Irrespective of income classification, just over two-thirds of countries in Africa reported having access to physiotherapy services in paediatric oncology sites. There is a lack of knowledge about African childhood physiotherapy services. Research is needed to understand the rehabilitation needs of these children/adolescents and how to meet their needs in a globally equitable and sustainable way.

International Society of Paediatric Oncology (SIOP) Global Mapping Program: Analysis of healthcare centers in countries of the Latin American Society of Pediatric Oncology (SLAOP).

The International Society of Paediatric Oncology Society Global Mapping Program aims to describe the local pediatric oncology capacities. Here, we report the data from Latin America. A 10-question survey was distributed among chairs of pediatric oncology services. Centers were classified according to patient volume into high- (HVC; 100 or more new cases per year), medium- (MVC; 31-99 cases), and low-volume centers (LVC; 30 cases or less), respectively. National referral centers (NRC) were identified. Total 307 centers in 20 countries were identified (271 responded), and 264 responses were evaluable, accounting for 78% of the expected cases (21,359 cases per year). Seventy-seven percent of patients are treated in public centers, including additional support by civil society organizations. We found that 66% of the patients are treated in 70 centers of excellence, including 21 NRC. There was a median of one pediatric oncologist every 21 newly diagnosed patients (44 for NRC), and in 84% of the centers, nurses rotated to other services. A palliative care team was lacking in 25% of the centers. LVC with public funding have significantly lower probability of having a palliative care team or trained pediatric oncology surgeons. Psychosocial, pharmacy, and nutrition services were available in more than 93% of the centers. No radiotherapy facility was available on campus in nine of 21 NRC. Most children with cancer in Latin America are treated in public HVC. There is a scarcity of pediatric oncologists, specialized nurses and surgeons, and palliative care teams, especially in centers with public funding.

Outcome of Retinoblastoma After Implementation of National Retinoblastoma Treatment Guidelines in South Africa.

Retinoblastoma, a curable childhood cancer, has been identified as a tracer cancer in the WHO Global Initiative for Childhood Cancer. To document the outcomes of children with retinoblastoma in South Africa, treated as per the first prospective standard national treatment guidelines for childhood cancer in South Africa. All children diagnosed with retinoblastoma between 2012 and 2016 in five South African pediatric oncology units were treated with a standard treatment on the basis of the International Society of Pediatric Oncology-Pediatric Oncology in Developing Countries guidelines for high-income settings. Treatment included focal therapy with/without chemotherapy, or enucleation with/without chemotherapy, and orbital radiotherapy, depending on enucleated eye histology. The end point was survival at 24 months, using Kaplan-Meier curves with log-rank (Mantel-Cox) and chi-square (χ2) tests with respective P values reported. A total of 178 children were included in the study; 68% presented with unilateral disease. The median age was 27 months (range 0-118 months) with a male:female ratio of 1:0.75. The overall survival was 79% at 24 months with significant association with stage at diagnosis (P < .001) and older age over 2 years as opposed to younger than 2 years (P < .001). Causes of death were disease progression/relapses in 90% (34 of 38) and unknown in 2% (1 of 38), whereas treatment abandonment was 1.7% (3 of 178). Efficacy with national treatment guidelines was confirmed, and feasibility of implementing standard national childhood cancer treatment guidelines was documented, involving multidisciplinary teams in South Africa. Outcome was significantly associated with stage at diagnosis and age.

Indian Society of Medical and Paediatric Oncology (ISMPO)—Breast Cancer in Young Guidelines

AbstractBreast cancer (BC) is the most common type of cancer globally and in India. In India, BC is more common among younger women compared with Western counterparts. Younger women with BC tend to have a less favorable outcome as they are more likely to have aggressive tumors. Younger women are not well represented in BC management studies as the median age at diagnosis is in the late 50s to early 60s. This can lead to difficulty in using risk-stratification models and molecular tools among young BC patients and may result in overtreatment. Therefore, Indian Society of Medical and Pediatric Oncology gathers and organizes available evidence from published literature to create a guide specifically for young BC patients in low- and middle-income countries like India.

Cancer is not a risk factor for severe COVID-19 in children, except in patients with recent allogeneic hematopoietic stem cell transplantation or comorbidities.

The EPICO (Spanish general registry of COVID-19 in children)-SEHOP (Spanish Society of Pediatric Hematology and Oncology) platform gathers data from children with SARS-CoV-2 in Spain, allowing comparison between children with cancer or allogeneic hematopoietic stem cell transplantation (alloHSCT) and those without. The infection is milder in the cancer/alloHSCT group than in children without comorbidities (7.1% vs. 14.7%), except in children with recent alloHSCT (less than 300days), of which 35.7% experienced severe COVID-19. These data have been shared with the SEHOP members to support treatment and isolation policies akin to those for children without cancer, except for those with recent alloHSCT or additional comorbidities. This highlights the collaborative registries potential in managing pandemic emergencies.

Real-world experience of larotrectinib in children, adolescents and young adults with TRK fusion solid tumors in France.

10056 Background: Larotrectinib is an FDA and EMA approved TRK inhibitor for TRK fusion solid tumors in patients with locally advanced or metastatic disease, or where surgical resection is likely to result in severe morbidity. Here we report the real-world activity and safety data on compassionate use and post-marketing authorization of larotrectinib collected in the SACHA-France study (NCT04477681), following the French Health Technology Assessment institution (HAS) request. Methods: All larotrectinib prescriptions made in France outside clinical trials since April 2019 for patients’ &lt; 25 years-old were registered in the SACHA-France study. Safety and activity data were collected, with data cut-off of 23/01/2024. SACHA-France is open in all 31 French Society of Pediatric Oncology (SFCE) centers, it is approved as a real-world data source by the HAS and supported by the French National Agency for the Safety of Medicines and Health Products (ANSM). Results: 21 patients were included (4 compassionate use, 17 post-marketing authorization). Main cancer types were soft-tissue sarcomas (n=13, of them 7 infantile fibrosarcoma), followed by central nervous system (CNS) tumors (n= 7), and other solid extra-CNS tumors (n=1). All patients except one had a TRK fusion tumor: NTRK3 (n=10), NTRK2 (n=6, all CNS tumor) and NTRK1 (n=4). Median age at start of larotrectinib was 2.8 years (range: 0.2 -21.1). Ten patients had received no prior systemic therapy. Main reasons to start larotrectinib were to avoid mutilating surgery (n=9) and disease progression (n=9). Best objective response was reported as partial response in 13 out of 19 patients with evaluable disease (10/12 patients with soft tissue sarcomas, 2/6 patients with CNS tumors), with median time to response of 57 days. Of them, five patients with soft tissue sarcomas achieved a complete response after non mutilating surgery. Median treatment duration was 219 days (range: 10-1368, 5 patients still on therapy). Seven patients with soft tissue sarcomas stopped larotrectinib, after a median duration of 242 days (range: 122-1190), three of them after tumor surgery; none presented with tumor recurrence (follow-up: 350 days; range 40-636). At data cut-off, 8 patients had disease progression on-therapy (5/7 CNS tumors, 3/13 soft tissue sarcomas), with a NTRK resistance mutation identified in 2 out 3 patients with tumor biopsy. Adverse drug reaction (ADR) were reported in 2/21 patients (9%), including a grade 2 weight gain and a grade 3 neutrophil count decreased (1 patient each). Both ADRs were expected and required neither corrective treatment nor action on larotrectinib. Conclusions: Our real-world data confirm the favorable safety profile of larotrectinib with rapid and durable tumor-agnostic efficacy.

Current surgical approaches to pediatric renal tumors.

Pediatric renal tumors are among the most common pediatric solid malignancies. Surgical resection is a key component in the multidisciplinary therapy for children with kidney tumors. Therefore, it is imperative that surgeons caring for children with renal tumors fully understand the current standards of care in order to provide appropriate surgical expertise within this multimodal framework. Fortunately, the last 60years of international, multidisciplinary pediatric cancer cooperative group studies have enabled high rates of cure for these patients. This review will highlight the international surgical approaches to pediatric patients with kidney cancer to help surgeons understand the key differences and similarities between the European (International Society of Pediatric Oncology) and North American (Children's Oncology Group) recommendations.

Managing Undernutrition in Pediatric Oncology: A Consensus Statement Developed Using the Delphi Method by the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology.

"Managing Undernutrition in Pediatric Oncology" is a collaborative consensus statement of the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology. The early identification and accurate management of malnutrition in children receiving anticancer treatment are crucial components to integrate into comprehensive medical care. Given the scarcity of high-quality literature on this topic, a consensus statement process was chosen over other approaches, such as guidelines, to provide comprehensive recommendations. Nevertheless, an extensive literature review using the PubMed database was conducted. The following terms, namely pediatric, childhood, cancer, pediatric oncology, malnutrition, undernutrition, refeeding syndrome, nutritional support, and nutrition, were used. The consensus was reached through the Delphi method. Comprehensive recommendations aim to identify malnutrition early in children with cancer and optimize nutritional interventions in this group. The statement underscores the importance of baseline and ongoing assessments of nutritional status and the identification of the risk factors for malnutrition development, and it presents tools that can be used to achieve these goals. This consensus statement establishes a standardized approach to nutritional support, aiming to optimize outcomes in pediatric cancer patients.

Anaplasia in Wilms tumor: A critical review.

Anaplasia in Wilms tumor is recognized as the most important prognostically unfavorable histological feature. It is subtyped as focal anaplastic Wilms tumor (FAWT) and diffuse anaplastic Wilms tumor (DAWT). Outcomes of patients with DAWT remain poor in patients with stage III and IV tumors. Important issues relevant to anaplasia in Wilms tumor, including prevalence, treatment, outcomes, biomarkers, anaplasia, and chemotherapy, and the concept of tumor aggressiveness, are reviewed and discussed here. We also consider the differences in clinical approaches to anaplasia in Wilms tumor between the two major renal tumor clinical research groups: the International Society of Paediatric Oncology (SIOP) Renal Tumour Study Group and the Children's Oncology Group (COG) Renal Tumor Group. We emphasize the importance and implications of recognizing FAWT and DAWT as separate clinico-pathological entities.

International Society of Paediatric Oncology (SIOP) Global Mapping Programme: Latin American Society of Pediatric Oncology (SLAOP) country-level report.

Latin American countries are improving childhood cancer care, showing strong commitment to implement the Global Initiative for Childhood Cancer, but there are scant publications of the situation at a continental level. As part of the International Society of Paediatric Oncology Global Mapping project, delegates of each country participating in the Latin American Society of Pediatric Oncology (SLAOP) and chairs of national pediatric oncology societies and cooperative groups were invited to provide information regarding availability of national pediatric cancer control programs (NPCCP), pediatric oncology laws, pediatric oncology tumor registries, and training programs and support to diagnosis and treatment. Nineteen of the 20 countries participating in SLAOP responded. National delegates reported nine countries with NPCCP and four of them were launched in the past 5years. National pediatric tumor registries are available in eight countries, and three provided published survival results. Fellowship programs for training pediatric oncologists are available in 12 countries. National delegates reported that eight countries provide support to most essential diagnosis and treatments and 11 provide partial or minimal support that is supplemented by civil society organizations. Seven countries have a pediatric oncology law. There are three international cooperative groups and four national societies for pediatric oncology. Despite many challenges, there were dramatic advances in survivorship, access to treatment, and availability of NPCCP in Latin America. Countries with highest social development scores in general provide more complete support and are more likely to have NPCCP, training programs, and reported survival results.

Spanish registry of hemoglobinopathies and rare anemias (REHem-AR): demographics, complications, and management of patients with β-thalassemia

IntroductionThe increase in the number of patients with hemoglobinopathies in Europe in recent decades highlights the need for more detailed epidemiological information in Spain. To fulfil this need, the Spanish Society of Pediatric Hematology and Oncology (SEHOP) sponsored the creation of a national registry of hemoglobinopathies known as REHem-AR (Spanish Registry of Hemoglobinopathies and Rare Anemias). Data from the transfusion-dependent (TDT) and non–transfusion-dependent (NTDT) β-thalassemia cohorts are described and analyzed.MethodsWe performed an observational, multicenter, and ambispective study, which included patients of any age with TDT and NTDT, registered up to December 31, 2021.ResultsAmong the 1741 patients included, 168 cases of thalassemia were identified (103 TDT and 65 NTDT-patients). Survival at 18 years was 93% for TDT and 100% for NTDT. Regarding management, 80 patients with TDT (77.7%) and 23 patients with NTDT (35.4%) started chelation treatment during follow-up, with deferasirox being the most widely used. A total of 76 patients within the TDT cohort presented at least 1 complication (73.8%), the most frequent being hemosiderosis and osteopenia-osteoporosis. Comparison of both cohorts revealed significant differences in the diagnosis of hepatic hemosiderosis (p = 0.00024), although these were not observed in the case of cardiac iron overload (p = 0.27).DiscussionOur registry enabled us to describe the management of β thalassemia in Spain and to analyze the morbidity and mortality of the cohorts of patients with TDT and NTDT. Complications related to iron overload in TDT and NTDT account for most of the morbidity and mortality of the disease, which is associated with a considerable social, psychological, and economic impact, although cardiac, osteopathy and endocrinological complications requiring more attention. The convenience and simplicity of online registries make it possible to homogenize variables and periodically update data, thus providing valuable information on these diseases.

Chemo-immunotherapy with dinutuximab beta in patients with relapsed/progressive high-risk neuroblastoma: does chemotherapy backbone matter?

BackgroundAddition of anti-GD2 antibodies to temozolomide-based chemotherapy has demonstrated increased antitumor activity and progression-free survival in patients with relapsed/progressive high-risk neuroblastoma. However, chemo-immunotherapy is not yet approved for this indication. This study presents the chemo-immunotherapy experience in patients with relapsed/progressive high-risk neuroblastoma treated within the off-label use program of the Neuroblastoma Committee of the French Society of Pediatric Oncology (SFCE). MethodsDinutuximab beta (dB) was administered alongside temozolomide-topotecan (TOTEM) or temozolomide-irinotecan (TEMIRI) at first disease relapse/progression or topotecan-cyclophosphamide (TopoCyclo) at further relapse/progression. Real-world data on demographics, treatment, antitumor activity and safety was collected from all patients after inclusion in SACHA-France (NCT04477681), a prospective national registry, which documents safety and efficacy data on innovative anticancer therapies prescribed to patients ≤ 25 years old as compassionate or off-label use. ResultsBetween February 2021 and July 2023, 39 patients with confirmed relapsed/progressive high-risk neuroblastoma (median age 6 years, range 1–24) were treated with dB+TopoCyclo (n = 24) or dB+TOTEM/TEMIRI (n = 15) across 17 centers. In total, 163 chemo-immunotherapy cycles were administered, main toxicities were mild or moderate, with higher incidence of hematological adverse drug reactions with dB+TopoCyclo than dB+TOTEM/TEMIRI. Objective response rate was 42% for dB+TopoCyclo (CI95% 22–63%) and 40% for dB+TOTEM/TEMIRI (CI95% 16–68%). ConclusionSimilar objective response rates for dB+TopoCyclo and dB+TOTEM/TEMIRI in patients with relapsed/progressive high-risk neuroblastoma emphasize the importance of chemo-immunotherapy, irrespective of the chemotherapy backbone.

Assessment of complementary health approaches use in pediatric oncology: Modification and preliminary validation of the "Which Health Approaches and Treatments Are You Using?" (WHAT) questionnaires.

Complementary Health Approaches (CHA) are commonly used by children with cancer; however, a few health care providers (HCPs) inquire about the use of CHA. A standardized questionnaire could facilitate such clinical discussions. We aimed to adapt and determine the face and content validity of the "Which Health Approaches and Treatments are you using?" (WHAT) child and parent-report questionnaires in pediatric oncology. An electronic Delphi survey that included children with cancer (8-18 years), parents, and HCPs and CHA researchers was conducted to reach consensus on the content of the WHAT questionnaires in pediatric oncology. Children and parents from the Hospital for Sick Children (SickKids), and HCPs and researchers from the International Society of Pediatric Oncology and Pediatric Complementary and Alternative Medicine Research and Education Network completed the survey. To determine the face and content validity of the questionnaires, two iterative cycles of individual interviews were conducted with purposive samples of children (8-18 years), parents, and HCPs from SickKids. Consensus was reached on all domains and items of the original WHAT questionnaires after one Delphi cycle (n = 61). For face and content validity testing, the first cycle of interviews (n = 19) revealed that the questionnaires were mostly comprehensive and relevant. However, the paper-based format of the original WHAT was not user-friendly, and generic items were vague and not aimed at facilitating clinical dialogues about CHA use. The WHAT questionnaires were then modified into electronic cancer-specific self- and proxy-report questionnaires including 13 and 15 items, respectively. The second cycle (n = 21) showed no need for further changes. The modified electronic cancer-specific WHAT questionnaires showed adequate face and content validity. The next step is to determine inter-rater reliability, construct validity, and feasibility of administration of the modified WHAT questionnaires in pediatric oncology.

The role of International Society of Paediatric Oncology (SIOP) in advancing global childhood cancer care.

The Société Internationale d'Oncologie Pédiatrique [International Society of Paediatric Oncology] (SIOP), founded in 1969, aims to improve the lives of children and adolescents with cancer through global collaboration, education, training, research and advocacy. The annual congress provides the opportunity to share late-breaking research, clinical experiences and debate, with experts worldwide. SIOP's six Continental Branches represent their constituent members in North America, Oceania, Latin America, Africa, Europe and Asia and bring best practices and recent research findings of value to their specific patient populations. In 1990, the SIOP Board of Directors addressed the formerly predominantly European/North American society transforming into a global association by establishing a scholarship program to bring low- and middle-income country (LMIC) paediatric oncologists and nurses to SIOP meetings. A major achievement was SIOP's acceptance as a World Health Organisation (WHO) non-state actor in official relations in 2018, joining 220 non-governmental organisations, international business associations and philanthropic foundations with this privilege. SIOP supports advocacy with WHO member states and civil society to highlight the specific needs of cancer in this age-group through key programs especially supporting the WHO Global Initiative for Childhood Cancer. Sustained improvement in childhood cancer outcomes has paralleled the integration of research with care; thus, SIOP launched a Programme for Advancing Research Capacity for funding selected clinical trial groups in LMICs. SIOP supports south-south partnerships, and the principles elegantly expressed in SIOP Africa's checklist for co-branding projects, that include the prioritisation of local needs, cultivation of local expertise and commitment to equitable partnerships. SIOP now counts approximately 3,000 members from over 128 countries; 39% are from more than 60 LMICs. SIOP members have multidisciplinary expertise on all aspects of childhood cancer care working in collaboration with key stakeholders including governments, civil society organisations and funders to improve the lives of children/adolescents with cancer everywhere in all ways.

Wilms tumour in a two day old neonate: A rare and challenging case

Wilms tumor or nephroblastoma is the second most common renal tumor occurring in the neonatal age group next to congenital mesoblastic nephroma. The most common age group of presentation of Wilms tumor is between 3 and 4 years of age. Here, we report a case of right-sided Wilms tumor in a 2-day-old neonate. A term-born female baby presented on day 2 of birth with abdominal distension and physical examination revealed a large palpable mass in the right lumbar region crossing the midline. Imaging features were suggestive of a retroperitoneal mass of probable right renal origin. The patient was taken up for right radical nephroureterectomy and Per-operatively, there was anticipated tumor spillage. Post-operative histopathological examination was suggestive of a triphasic Wilms tumor with no anaplasia and favorable histology. The patient was diagnosed with Wilms tumor of the right kidney, COG stage III, SIOP (“International Society of Pediatric Oncology”) intermediate risk loss of heterozygosity testing for 1p and 16q was negative. She was started on chemotherapy with a DDA4 regimen. She completed whole abdominal radiotherapy and tolerated treatment well. The child is disease free and is under regular follow-up. This case of neonatal Wilms tumor was indeed a challenge to the pediatric surgeons and the oncologists but the final results were worth the challenge that was faced.