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 300 days), 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.

Unexpected Cause of Persistent Microcytosis and Neurological Symptoms in a Child: Niemann-Pick Disease Type C.

Atypical microcytic anemias are rare diseases of iron/heme metabolism that can be diagnostically challenging. We report the case of a 2-year-old twin boy with neurodevelopmental delay and persistent microcytosis in whom atypical microcytic anemias was initially suspected. He had low blood iron and transferrin saturation with normal/high ferritin despite iron therapy. Hemoglobinopathies were excluded by conventional/DNA studies. Hepcidin was high but iron-refractory-iron-deficiency anemia was ruled out by a genetic panel. Bone marrow aspiration revealed foamy cells and iron depletion. A genetic study confirmed the diagnosis of Niemann-Pick disease type C which was finally considered the origin of microcytosis through anemia of chronic disease.

Questions and answers in the management of children with medulloblastoma over the time. How did we get here? A systematic review.

Introduction: Treatment of children with medulloblastoma (MB) includes surgery, radiation therapy (RT) and chemotherapy (CT). Several treatment protocols and clinical trials have been developed over the time to maximize survival and minimize side effects. Methods: We performed a systematic literature search in May 2023 using PubMed. We selected all clinical trials articles and multicenter studies focusing on MB. We excluded studies focusing exclusively on infants, adults, supratentorial PNETs or refractory/relapsed tumors, studies involving different tumors or different types of PNETs without differentiating survival, studies including <10 cases of MB, solely retrospective studies and those without reference to outcome and/or side effects after a defined treatment. Results: 1. The main poor-prognosis factors are: metastatic disease, anaplasia, MYC amplification, age younger than 36 months and some molecular subgroups. The postoperative residual tumor size is controversial.2. MB is a collection of diseases.3. MB is a curable disease at diagnosis, but survival is scarce upon relapse.4. Children should be treated by experienced neurosurgeons and in advanced centers.5. RT is an essential treatment for MB. It should be administered craniospinal, early and without interruptions.6. Craniospinal RT dose could be lowered in some low-risk patients, but these reductions should be done with caution to avoid relapses.7. Irradiation of the tumor area instead of the entire posterior fossa is safe enough.8. Hyperfractionated RT is not superior to conventional RT9. Both photon and proton RT are effective.10. CT increases survival, especially in high-risk patients.11. There are multiple drugs effective in MB. The combination of different drugs is appropriate management.12. CT should be administered after RT.13. The specific benefit of concomitant CT to RT is unknown.14. Intensified CT with stem cell rescue has no benefit compared to standard CT regimens.15. The efficacy of intraventricular/intrathecal CT is controversial.16. We should start to think about incorporating targeted therapies in front-line treatment.17. Survivors of MB still have significant side effects. Conclusion: Survival rates of MB improved greatly from 1940-1970, but since then the improvement has been smaller. We should consider introducing targeted therapy as front-line therapy.

Clinical experience with immunotherapy in patients with diffuse intrinsic pontine glioma.

The objective of the article is to report the case of three patients with diffuse intrinsic pontine glioma (DIPG) treated with immunotherapy. In particular we report the data related to the treatments' efficacy and tolerance.To achieve this, we review the medical records in the Paediatric Oncology and Haematology Unit of HM Hospitales/Centro Integral Oncológico Clara Campal (CIOCC). We focused on patients diagnosed with DIPG who were administered oncolytic viruses followed by immune checkpoint inhibitors (ICI) (pembrolizumab, anti PD-1) plus a concomitant antiangiogenic agent (bevacizumab).The results we obtained showed the three paediatric DIPG patients studied presented good tolerance, with disease stabilisation for approximately 5 months after immunotherapy. However, subsequent clinical worsening required clinicians to change the patients' treatment.In conclusion, immunotherapy combined with other conventional antineoplastic treatments (chemotherapy, radiotherapy) is postulated as a very promising future therapeutic option. However, further research is warranted in the paediatric population to demonstrate safety and effectiveness.

Ten Reasons Why People With Down Syndrome are Protected From the Development of Most Solid Tumors -A Review.

People with Down syndrome have unique characteristics as a result of the presence of an extra chromosome 21. Regarding cancer, they present a unique pattern of tumors, which has not been fully explained to date. Globally, people with Down syndrome have a similar lifetime risk of developing cancer compared to the general population. However, they have a very increased risk of developing certain tumors (e.g., acute leukemia, germ cell tumors, testicular tumors and retinoblastoma) and, on the contrary, there are some other tumors which appear only exceptionally in this syndrome (e.g., breast cancer, prostate cancer, medulloblastoma, neuroblastoma and Wilms tumor). Various hypotheses have been developed to explain this situation. The genetic imbalance secondary to the presence of an extra chromosome 21 has molecular consequences at several levels, not only in chromosome 21 but also throughout the genome. In this review, we discuss the different proposed mechanisms that protect individuals with trisomy 21 from developing solid tumors: genetic dosage effect, tumor suppressor genes overexpression, disturbed metabolism, impaired neurogenesis and angiogenesis, increased apoptosis, immune system dysregulation, epigenetic aberrations and the effect of different microRNAs, among others. More research into the molecular pathways involved in this unique pattern of malignancies is still needed.

When a transfusion in an emergency service is not really urgent: hyperhaemolysis syndrome in a child with sickle cell disease.

A 13-month-old boy with sickle cell disease (SCD) from Equatorial Guinea, who had recently arrived in Spain, presented with fever. He had suffered from malaria and had received a blood transfusion. Following physical examination and complementary tests, intravenous antibiotics and a red blood cell (RBC) transfusion were administered. Soon after a second transfusion 5 days later, the haemoglobin level fell below pretransfusion levels, together with reticulocytopenia, and haematuria-the so-called hyperhaemolysis syndrome-requiring intensive care and treatment with intravenous immunoglobulins and corticosteroids, with resolution of the complication. We want to emphasise the importance of suspecting this rare, though severe complication that can appear after any RBC transfusion especially in patients with SCD, as the clinical syndrome can simulate other more common complications of these patients and a further transfusion is contraindicated. There is no standardised treatment, but intravenous immunoglobulin and corticosteroids are usually effective.

Radioterapia paliativa pediátrica / Pediatric palliative radiotherapy

Introducción: La radioterapia es un recurso infrautilizado en los cuidados paliativos pediátricos; sin embargo, tiene utilidad para aliviar diversos síntomas al final de la vida. Pacientes y métodos: Se muestra un estudio retrospectivo de 30 pacientes pediátricos que han recibido radioterapia por motivos paliativos. El objetivo fue revisar si la radioterapia resultó efectiva. Resultados: La radioterapia fue útil para aliviar los síntomas, mostrando una respuesta global en el 85 % de los casos y presentando toxicidades leves. Las respuestas más pobres fueron en gliomas con alivio sintomático en el 56 % de los casos, mostrando toxicidades en el 89 % de los mismos. La necesidad de anestesia supuso un factor de riesgo para el desarrollo de toxicidades. Conclusiones: El uso de la radioterapia paliativa es beneficiosa y debe incluirse en el manejo multidisciplinar del niño en cuidados paliativos. (AU)

Introduction: Radiotherapy is an underutilized resource in pediatric palliative care; however, it has utility in relieving various symptoms at the end of life. Patients and methods: A retrospective study of 30 pediatric patients who received radiotherapy for palliative reasons is reported. The objective was to review whether radiotherapy was effective. Results: Radiotherapy was useful in relieving symptoms, showing an overall response in 85 % of cases and presenting mild toxicities. The poorest responses were found in gliomas, with symptomatic relief in 56 % of cases, showing toxicities in 89 % of cases. The need for anesthesia represented risk factor for the development of toxicities. Conclusions: The use of palliative radiotherapy is beneficial and should be included in the multidisciplinary management of children in palliative care. (AU)

Pediculosis capitis: ¿es algo realmente banal? / Pediculosis capitis: is it really an insignificant disease?

La pediculosis capitis es la infestación pediátrica más frecuente. Generalmente se considera una infestación banal y no se comentan las posibles complicaciones clínicamente relevantes. Se presenta el caso de una adolescente con historia de astenia, anemia moderada, importante adenopatía cervical y eosinofilia, finalmente achacada a la infestación por el parásito, no sospechada de entrada (AU)

Pediculosis capitis is the most common pediatric infestation. Generally, it is considered a minor infestation, and scientific literature rarely reports possible relevant clinical complications. Our aim is to present the case of an adolescent with asthenia, moderate anaemia, significant cervical lymphadenopathy and eosinophilia, which was discovered later that the parasite was the cause (AU)