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IntroductionTreatment 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.MethodsWe 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

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.

El Colegio Montepríncipe trabaja en la educación de niños, niñas y adolescentes con diagnóstico de cáncer; apuesta por la no interrupción de su vida escolar en su aspecto académico y, cuando es posible, social. Cuenta, para ello, con un equipo multidisciplinar formado por profesionales de la docencia, psicología y musicoterapia y con personas voluntarias. Este equipo está integrado en el funcionamiento sanitario de la Unidad de Hematología y Oncología Pediátrica del Hospital Universitario Montepríncipe. En este artículo se describe el funcionamiento del colegio en el hospital y cómo este permite integrar la enfermedad en la vida normal de estos grupos afectados por tal diagnóstico. Para valorar dicho objetivo, se ha llevado a cabo un estudio experimental longitudinal cuantitativo, en el que se tuvo en cuenta la asistencia diaria al colegio. Se contó con un promedio de 36 estudiantes a lo largo de cinco cursos académicos (2012-2017) en los que se comprobó que la asistencia diaria al colegio era del 90 % (esta no es obligatoria), y se mantenía constante a lo largo de estos años. Además, se midió el rendimiento académico de una selección de estudiantes para comprobar que la asistencia al colegio era eficaz. En este caso, la muestra real la formaron 14 sujetos y se compararon las notas medias de las asignaturas de Lengua y Matemáticas antes del diagnóstico y después de terminar el curso en el hospital. Los resultados mostraron que el rendimiento aumentaba como promedio un punto por asignatura. De los resultados se podría concluir que la asistencia diaria al colegio en el hospital ayuda a integrar la enfermedad en la vida normal de cada paciente e incluso a mejorar sus resultados académicos.

Introduction: Diffuse intrinsic pontine glioma (DIPG) is the most common malignant brainstem tumour in children. Despite advances in understanding its biology, current treatments have shown minimal impact on overall survival in this fatal disease. Focal radiotherapy (RT) is the only treatment proven to improve symptoms and extend progression-free survival. Albeit palliative, re-irradiation (rRT) has emerged as the best alternative for progressive disease. This study presents the Spanish experience with re-irradiation in DIPG. Results: Between April 2015 and December 2023, 44 paediatric patients with progressive DIPG underwent rRT in 16 Spanish institutions. Median time from diagnosis to progression was 9.9 months (range, 4.2–24.3 months). Median dose of rRT was 20 Gy (range, 18–40 Gy) in 2 Gy fractions (range, 1.3–4 Gy). Twenty-two patients (50 %) received other treatments besides RT. Clinical improvement was seen in 77.3 %, and radiological improvement in 60 %. Treatment was well tolerated (1 case toxicity >grade 2 related to rRT). Median overall survival was 15.5 months (range, 8.2–63.2 months), with a median time from rRT to death of 4.2 months (range, 0.6–10.3 months). Longer time between diagnosis and rRT (>10 months) and dose of rRT >20 Gy were statistically significantly correlated with better overall survival. There was no survival benefit in patients receiving additional treatments. Conclusions: Re-irradiation is safe and effective in progressive DIPG patients, not only improving symptoms but also prolonging survival. However, the ideal candidates for rRT remain undefined, as well as the best irradiation scheme. Prospective studies are needed.