[External radiation therapy of primary central nervous system tumors - current trends and practice]. / A primer központi idegrendszeri tumorok konvencionális külso sugárkezelése ­ aktuális trendek és gyakorlat.

The family of primary central nervous system (CNS) tumors is a highly heterogeneous group of diseases. In the complex care of CNS tumors, in addition to surgical and systemic treatments, modern external beam radiation therapy (EBRT) plays a prominent role. This summary article provides an overview of the current indications related to EBRT, diagnostic procedures, contouring, planning, and radiation therapy techniques and applications that can be used in daily routine for adults' most common primary CNS tumors.

The effectiveness and safety of proton beam radiation therapy in children and young adults with Central Nervous System (CNS) tumours: a systematic review.

BACKGROUND: Central nervous system (CNS) tumours account for around 25% of childhood neoplasms. With multi-modal therapy, 5-year survival is at around 75% in the UK. Conventional photon radiotherapy has made significant contributions to survival, but can be associated with long-term side effects. Proton beam radiotherapy (PBT) reduces the volume of irradiated tissue outside the tumour target volume which may potentially reduce toxicity. Our aim was to assess the effectiveness and safety of PBT and make recommendations for future research for this evolving treatment. METHODS: A systematic review assessing the effects of PBT for treating CNS tumours in children/young adults was undertaken using methods recommended by Cochrane and reported using PRISMA guidelines. Any study design was included where clinical and toxicity outcomes were reported. Searches were to May 2021, with a narrative synthesis employed. RESULTS: Thirty-one case series studies involving 1731 patients from 10 PBT centres were included. Eleven studies involved children with medulloblastoma / primitive neuroectodermal tumours (n = 712), five ependymoma (n = 398), four atypical teratoid/rhabdoid tumour (n = 72), six craniopharyngioma (n = 272), three low-grade gliomas (n = 233), one germ cell tumours (n = 22) and one pineoblastoma (n = 22). Clinical outcomes were the most frequently reported with overall survival values ranging from 100 to 28% depending on the tumour type. Endocrine outcomes were the most frequently reported toxicity outcomes with quality of life the least reported. CONCLUSIONS: This review highlights areas of uncertainty in this research area. A well-defined, well-funded research agenda is needed to best maximise the potential of PBT. SYSTEMATIC REVIEW REGISTRATION: PROSPERO-CRD42016036802.

Craniospinal irradiation for CNS leukemia: rates of response and durability of CNS control.

PURPOSE: Management of CNS involvement in leukemia may include craniospinal irradiation (CSI), though data on CSI efficacy are limited. METHODS: We retrospectively reviewed leukemia patients who underwent CSI at our institution between 2009 and 2021 for CNS involvement. CNS local recurrence (CNS-LR), any recurrence, progression-free survival (PFS), CNS PFS, and overall survival (OS) were estimated. RESULTS: Of thirty-nine eligible patients treated with CSI, most were male (59%) and treated as young adults (median 31 years). The median dose was 18 Gy to the brain and 12 Gy to the spine. Twenty-five (64%) patients received CSI immediately prior to allogeneic hematopoietic cell transplant, of which 21 (84%) underwent total body irradiation conditioning (median 12 Gy). Among 15 patients with CSF-positive disease immediately prior to CSI, all 14 assessed patients had pathologic clearance of blasts (CNS-response rate 100%) at a median of 23 days from CSI start. With a median follow-up of 48 months among survivors, 2-year PFS and OS were 32% (95% CI 18-48%) and 43% (95% CI 27-58%), respectively. Only 5 CNS relapses were noted (2-year CNS-LR 14% (95% CI 5-28%)), which occurred either concurrently or after a systemic relapse. Only systemic relapse after CSI was associated with higher risk of CNS-LR on univariate analysis. No grade 3 or higher acute toxicity was seen during CSI. CONCLUSION: CSI is a well-tolerated and effective treatment option for patients with CNS leukemia. Control of systemic disease after CSI may be important for CNS local control. CNS recurrence may reflect reseeding from the systemic space.

Radiotherapy for Primary Pediatric Central Nervous System Malignancies: Current Treatment Paradigms and Future Directions.

BACKGROUND: Central nervous system tumors are the most common solid tumors in childhood. Treatment paradigms for pediatric central nervous system malignancies depend on elements including tumor histology, age of patient, and stage of disease. Radiotherapy is an important modality of treatment for many pediatric central nervous system malignancies. SUMMARY: While radiation contributes to excellent overall survival rates for many patients, radiation also carries significant risks of long-term side effects including neurocognitive decline, hearing loss, growth impairment, neuroendocrine dysfunction, strokes, and secondary malignancies. In recent decades, clinical trials have demonstrated that with better imaging and staging along with more sophisticated radiation planning and treatment set-up verification, smaller treatment volumes can be utilized without decrement in survival. Furthermore, the development of intensity-modulated radiotherapy and proton-beam radiotherapy has greatly improved conformality of radiation. KEY MESSAGES: Recent changes in radiation treatment paradigms have decreased risks of short- and long-term toxicity for common histologies and in different age groups. Future studies will continue to develop novel radiation regimens to improve outcomes in aggressive central nervous system tumors, integrate molecular subtypes to tailor radiation treatment, and decrease radiation-associated toxicity for long-term survivors.

Treatment outcomes for response-based radiotherapy in children and adolescents with central nervous system germinoma: a prospective study.

PURPOSE: The optimal dose and range of radiotherapy for central nervous system (CNS) germinoma have not yet been established. This study aimed to investigate the effects of individualized radiotherapy on the prognosis of patients with germinoma. METHODS: Based on imaging examination, tumor markers, and pathologic results, patients with germinoma received different radiotherapy strategies, including R1 (24 Gy whole ventricular irradiation + tumor-bed boost to 40 Gy), R2 (24-30 Gy craniospinal irradiation + tumor-bed boost to 54 Gy), R3 (24 Gy craniospinal irradiation + tumor-bed boost to 40 Gy), and R4 (30 Gy craniospinal irradiation + tumor-bed boost to 54 Gy with 45 Gy to spinal metastasis). RESULTS: A total of 77 patients were enrolled in this study between January 2015 and March 2021. The 3-year event-free survival (EFS) and overall survival (OS) of the whole cohort were 94.7% ± 2.6% and 96.0% ± 2.3%, respectively. The 3-year EFS for patients with localized and metastatic disease were 96.6% ± 2.4% and 89.2% ± 7.2%, respectively. The 3-year EFS of patients receiving R1, R2, R3, and R4 radiotherapy were 100%, 94.1% ± 5.7%, 100%, and 86.2% ± 9.1%, respectively. CONCLUSION: Good prognosis was still achieved after reducing dose and extent of radiation for the patients who achieved complete response (CR) after induction chemotherapy or pathological CR after second-look surgery.

Mitigating Radiotoxicity in the Central Nervous System: Role of Proton Therapy.

OPINION STATEMENT: Central nervous system (CNS) radiotoxicity remains a challenge in neuro-oncology. Dose distribution advantages of protons over photons have prompted increased use of brain-directed proton therapy. While well-recognized among pediatric populations, the benefit of proton therapy among adults with CNS malignancies remains controversial. We herein discuss the role of protons in mitigating late CNS radiotoxicities in adult patients. Despite limited clinical trials, evidence suggests toxicity profile advantages of protons over conventional radiotherapy, including retention of neurocognitive function and brain volume. Modelling studies predict superior dose conformality of protons versus state-of-the-art photon techniques reduces late radiogenic vasculopathies, endocrinopathies, and malignancies. Conversely, potentially higher brain tissue necrosis rates following proton therapy highlight a need to resolve uncertainties surrounding the impact of variable biological effectiveness of protons on dose distribution. Clinical trials comparing best photon and particle-based therapy are underway to establish whether protons substantially improve long-term treatment-related outcomes in adults with CNS malignancies.

Social cognition and adjustment in adult survivors of pediatric central nervous system tumors.

BACKGROUND: Survivors of pediatric central nervous system (CNS) tumors are at risk for neurocognitive and social difficulties throughout childhood. This study characterized social cognition (perception and reasoning from social cues) and adjustment in adulthood. METHODS: A total of 81 adult survivors of pediatric CNS tumors (51% female; mean [SD] age, 28.0 [5.8] years), were recruited across four groups: (1) no radiation therapy (RT) [n = 21], (2) infratentorial (IT) tumors + focal RT [n = 20], (3) IT tumors + craniospinal irradiation [n = 20], and (4) supratentorial tumors + focal RT [n = 20]. Prevalence of social cognitive and adjustment impairments was compared to test norms. Multivariable models examined clinical and neurocognitive predictors of social cognition and its impact on functional outcomes. RESULTS: Survivors demonstrated elevated risk of severe social cognitive impairments (social perception Morbidity Ratio [95% CI] 5.70 [3.46-9.20]), but self-reported few social adjustment problems. Survivors of IT tumors treated with craniospinal irradiation performed nearly 1 SD worse than survivors treated without RT on multiple measures of social cognition (e.g., social perception: ß = -0.89, p = .004). Impaired executive functioning and nonverbal reasoning were associated with worse social cognitive performance (e.g., social perception: ß = -0.75, p < .001; ß = -0.84, p < .001, respectively). Better social perception was associated with higher odds of attaining full-time employment (odds ratio, 1.52 [1.17-1.97]) and at least some college education (odds ratio, 1.39 [1.11-1.74]). CONCLUSIONS: Adult survivors of CNS tumors are at elevated risk of severely impaired social cognition, but do not perceive social adjustment difficulties. Better understanding of potential mechanisms underlying social cognitive deficits may inform intervention targets to promote better functional outcomes for at-risk survivors.

Brain radiotherapy in patients treated for a newly diagnosed primary central nervous system lymphoma: professional practice evaluation in 19 French centers.

INTRODUCTION: The objective of this study was a multicentric evaluation of professional practices, analyzing the irradiation technique itself and its impact on survival and recurrence sites, in primary central nervous system lymphomas (PCNSLs). METHODS: We retrospectively analyzed the technical and clinical records of 79 PCNSL patients included in the database of the national expert network for oculocerebral lymphoma ('LOC') who were treated with brain radiotherapy as first-line treatment for newly diagnosed primary central nervous system lymphoma between 2011 and 2018. RESULTS: The number of patients treated with brain radiotherapy gradually decreased over time. The heterogeneity of radiotherapy prescriptions was significant, and 55% of them did not comply with published recommendations in terms of irradiation dose and/or volume. The proportion of complete responders to induction chemotherapy treated with reduced-dose radiotherapy increased over time. Partial brain radiotherapy was associated with significantly lower overall survival in univariate analysis. In partial responders to induction chemotherapy, increasing the total dose to the brain >30 Gy and adding a boost to the WBRT induced a trend toward improved progression-free and overall survival. Five recurrences (13%) occurred exclusively in the eyes, all in patients whose eyes had been excluded from the irradiation target volume and including 2 patients without ocular involvement at diagnosis. CONCLUSION: The visibility of recommendations for prescribing brain radiotherapy for the treatment of newly diagnosed primary central nervous system lymphoma needs to be improved to harmonize practices and improve their quality. We propose an update of the recommendations.

Critical Appraisal of Proton Therapy for Patients with Central Nervous System (CNS) Malignancies.

OPINION STATEMENT: As more hospital-based proton treatment centres become operational, the indications for proton beam therapy (PBT) are being evaluated. Recent advances in PBT technology are expanding the indications for the use of protons in the treatment of central nervous system (CNS) tumours. Prospective trials that assess the late toxicity of different radiation therapy (RT) techniques are needed to confirm any expected reduction in long-term side effects with PBT. The ASTRO Model Policy on proton beam therapy currently supports the reasonable use of protons in the treatment of specific CNS tumour types. Specifically, PBT plays a key role in the management of CNS tumours where anatomy, extent of disease or previous treatment cannot be satisfactorily addressed with conventional RT. As the availability of PBT rises around the world, the number of patients with CNS disease treated with PBT will continue to grow.

[A single-center, retrospective analysis of relapse and progression patterns of primary central nervous system lymphoma: can whole brain radiotherapy be replaced?]

OBJECTIVE: To analyze recurrence and progression patterns of primary central nervous system lymphoma (PCNSL) in patients without whole brain radiotherapy (WBRT) and assess the value of WBRT in PCNSL treatment. METHODS: This retrospective single-center study included 27 patients with PCNSL, who experienced recurrence/progression after achieving complete remission (CR), partial remission, or stable disease following initial treatments with chemotherapy but without WBRT. The patients were followed up regularly after the treatment for treatment efficacy assessment. By comparing the anatomical location of the lesions on magnetic resonance images (MRI) at the initial diagnosis and at recurrence/progression, we analyzed the patterns of relapse/progression in patients with different treatment responses and different initial status of the lesions. RESULTS: MRI data showed that in 16 (59.26%) of the 27 patients, recurrence/progression occurred in out-field area (outside the simulated clinical target volume [CTV]) but within the simulated WBRT target area in 16 (59.26%) patients, and within the CTV (in-field) in 11 (40.74%) patients. None of the patients had extracranial recurrence of the tumor. Of the 11 patients who achieved CR after the initial treatments, 9 (81.82%) had PCNSL recurrences in the out-field area but within WBRT target area; of the 13 patients with a single lesion at the initial treatment, 11 (84.62%) experienced PCNSL recurrence in the out-field area but within WBRT target area. CONCLUSIONS: Systemic therapy combined with WBRT still remains the standard treatment for PCNSL patients, especially those who achieve CR after treatment or have a single initial lesion. Future prospective studies with larger sample sizes are needed to further explore the role of low-dose WBRT in PCNSL treatment.

Genetic susceptibility to cognitive decline following craniospinal irradiation for pediatric central nervous system tumors.

BACKGROUND: Survivors of pediatric central nervous system (CNS) tumors treated with craniospinal irradiation (CSI) exhibit long-term cognitive difficulties. Goals of this study were to evaluate longitudinal effects of candidate and novel genetic variants on cognitive decline following CSI. METHODS: Intelligence quotient (IQ), working memory (WM), and processing speed (PS) were longitudinally collected from patients treated with CSI (n = 241). Genotype-by-time interactions were evaluated using mixed-effects linear regression to identify common variants (minor allele frequency > 1%) associated with cognitive performance change. Novel variants associated with cognitive decline (P < 5 × 10-5) in individuals of European ancestry (n = 163) were considered replicated if they demonstrated consistent genotype-by-time interactions (P < .05) in individuals of non-European ancestries (n = 78) and achieved genome-wide statistical significance (P < 5 × 10-8) in a meta-analysis across ancestry groups. RESULTS: Participants were mostly males (65%) diagnosed with embryonal tumors (98%) at a median age of 8.3 years. Overall, 1150 neurocognitive evaluations were obtained (median = 5, range: 2-10 per participant). One of the five loci previously associated with cognitive outcomes in pediatric CNS tumors survivors demonstrated significant time-dependent IQ declines (PPARA rs6008197, P = .004). Two variants associated with IQ in the general population were associated with declines in IQ after Bonferroni correction (rs9348721, P = 1.7 × 10-5; rs31771, P = 7.8 × 10-4). In genome-wide analyses, we identified novel loci associated with accelerated declines in IQ (rs116595313, meta-P = 9.4 × 10-9), WM (rs17774009, meta-P = 4.2 × 10-9), and PS (rs77467524, meta-P = 1.5 × 10-8; rs17630683, meta-P = 2.0 × 10-8; rs73249323, meta-P = 3.1 × 10-8). CONCLUSIONS: Inherited genetic variants involved in baseline cognitive functioning and novel susceptibility loci jointly influence the degree of treatment-associated cognitive decline in pediatric CNS tumor survivors.

Radiotherapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) on the Pediatric Proton/Photon Consortium Registry (PPCR).

PURPOSE: Atypical teratoid/rhabdoid tumors (ATRT) of the central nervous system (CNS) are rare tumors with a poor prognosis and variable use of either focal or craniospinal (CSI) radiotherapy (RT). Outcomes on the prospective Pediatric Proton/Photon Consortium Registry (PPCR) were evaluated according to RT delivered. METHODS: Pediatric patients receiving RT were prospectively enrolled on PPCR to collect initial patient, disease, and treatment factors as well as provide follow-up for patient outcomes. All ATRT patients with evaluable data were included. Kaplan-Meier analyses with log-rank p-values and cox proportional hazards regression were performed. RESULTS: The PPCR ATRT cohort includes 68 evaluable ATRT patients (median age 2.6 years, range 0.71-15.40) from 2012 to 2021. Median follow-up was 40.8 months (range 3.4-107.7). Treatment included surgery (65% initial gross total resection or GTR), chemotherapy (60% with myeloablative therapy including stem cell rescue) and RT. For patients with M0 stage (n = 60), 50 (83%) had focal RT and 10 (17%) had CSI. Among patients with M + stage (n = 8), 3 had focal RT and 5 had CSI. Four-year overall survival (OS, n = 68) was 56% with no differences observed between M0 and M + stage patients (p = 0.848). Local Control (LC) at 4 years did not show a difference for lower primary dose (50-53.9 Gy) compared to ≥ 54 Gy (73.3% vs 74.7%, p = 0.83). For patients with M0 disease, four-year OS for focal RT was 54.6% and for CSI was 60% (Hazard Ratio 1.04, p = 0.95. Four-year event free survival (EFS) among M0 patients for focal RT was 45.6% and for CSI was 60% (Hazard Ratio 0.71, p = 0.519). For all patients, the 4-year OS comparing focal RT with CSI was 54.4% vs 60% respectively (p = 0.944), and the 4-year EFS for focal RT or CSI was 42.8% vs 51.4% respectively (p = 0.610). CONCLUSION: The PPCR ATRT cohort found no differences in outcomes according to receipt of either higher primary dose or larger RT field (CSI). However, most patients were M0 and received focal RT. A lower primary dose (50.4 Gy), regardless of patient age, is appealing for further study as part of multi-modality therapy.

Outcomes of Patients Treated in the UK Proton Overseas Programme: Central Nervous System Group.

AIMS: In 2008, the UK National Health Service started the Proton Overseas Programme (POP), to provide access for proton beam therapy (PBT) abroad for selected tumour diagnoses while two national centres were being planned. The clinical outcomes for the patient group treated for central nervous system (CNS), base of skull, spinal and paraspinal malignancies are reported here. MATERIALS AND METHODS: Since the start of the POP, an agreement between the National Health Service and UK referring centres ensured outcomes data collection, including overall survival, local tumour control and late toxicity data. Clinical and treatment-related data were extracted from this national patient database. Grade ≥3 late toxicities were reported following Common Terminology Criteria for Adverse Events (CTCAE) v 4.0 definition, occurring later than 90 days since the completion of treatment. RESULTS: Between 2008 and September 2020, 830 patients were treated within the POP for the above listed malignancies. Overall survival data were available for 815 patients and local control data for 726 patients. Toxicity analysis was carried out on 702 patients, with patients excluded due to short follow-up (<90 days) and/or inadequate toxicity data available. After a median follow-up of 3.34 years (0.06-11.58), the overall survival was 91.2%. The local control rate was 85.9% after a median follow-up of 2.81 years (range 0.04-11.58). The overall grade ≥3 late toxicity incidence was 11.97%, after a median follow-up of 1.72 years (0.04-8.45). The median radiotherapy prescription dose was 54 GyRBE (34.8-79.2). CONCLUSIONS: The results of this study indicate the safety of PBT for CNS tumours. Preliminary clinical outcomes following PBT for paediatric/teen and young adult and adult CNS tumours treated within the POP are encouraging, which reflects accurate patient selection and treatment quality. The rate of late effects compares favourably with published cohorts. Clinical outcomes from this patient cohort will be compared with those of UK-treated patients since the start of the national PBT service in 2018.

Critical Appraisal of Paediatric Embryonal Cancers Treated with Image-guided Intensity-modulated Proton Therapy.

AIM: To carry out a comprehensive critical appraisal of image-guided intensity-modulated proton therapy practice for craniospinal irradiation (CSI). MATERIALS AND METHODS: An image-guided intensity-modulated proton therapy database of 45 consecutive paediatric patients with central nervous system embryonal malignancies treated between January 2019 and April 2022 were critically appraised for demography, diagnosis, treatment planning strategy and treatment delivery accuracy. RESULTS: Most patients (median age: 7.5 years; male:female ratio: 34:11) had medulloblastoma (56%), followed by recurrent ependymoma (19%), pinealoblastoma (5%), germ cell (5%) and others (15%). The dose to the planning target volume-craniospinal (PTV-CS; length 39.06-79.59 cm) varied from 21 to 35 GyRBE, whereas the combined median dose to craniospinal and boost was 54 GyRBE. In all patients, the 95% isodose line covered the cribriform plate completely and optic nerves mostly, with a median V95% of 100% and 82.96%, keeping Dmax to the lens <3.9 GyRBE. In skeletally immature patients (88.38%), the anterior vertebral body was completely covered in 18.18% and underdosed in 70.15% of the cases, resulting in a median Dmean of 10.11 GyRBE to the oesophagus. Lateral spine coverage was maintained on the edges of the vertebral body in 52.2%, whereas it extended beyond in 48.8%. The median V98% for clinical target volumes and V95% for PTVs of the brain, spine and craniospinal were >97%, with excellent conformity (0.89) and homogeneity (0.07) indices for PTV-CS. All neurological organs at risk received a median Dmax ranging from 36 to 44 GyRBE from the combined CSI and boost regimens. Analysis of patient-specific quality assurance results revealed that 545 (97.67%) planar dosage verification had gamma (3% at 3 mm) values >95%. The online patient set-up verification showed translational and rotational deviation within 2 mm and 0.5° in 88-94% and 97% of the cases. Systematic and random error were within 0.90 mm and 1.71 mm in translation and 0.1° and 0.2° in rotation. CONCLUSION: A change in practice pattern was observed. The findings from our comprehensive critical appraisal add to the growing library of CSI practice and may serve as a reference for inter-institutional comparison.

Análise Epidemiológica, Clínica e Patológica de Crianças com Neoplasias do Sistema Nervoso Central Tratadas com Radioterapia no Instituto Nacional de Câncer / Epidemiological, Clinical, and Pathological Analysis of Children with Central Nervous System Neoplasms Treated with Radiotherapy at the National Cancer Institute / Análisis Epidemiológico, Clínico y Patológico de Niños con Neoplasias del Sistema Nervioso Central Tratados con Radioterapia en el Instituto Nacional de Cáncer

Introdução: As neoplasias de sistema nervoso central representam 21% do câncer infantojuvenil, sendo a principal causa de morte nessa população em países desenvolvidos. Avanços na neuroimagem têm melhorado seu diagnóstico, e a combinação de terapias tem permitido a cura, com maior preservação neurológica. A cirurgia é o principal tratamento, frequentemente combinada com radioterapia e quimioterapia. Objetivo: Descrever achados socioeconômicos, sinais e sintomas, e aspectos histológicos dos tumores cerebrais pediátricos, tratados com radioterapia. Método: Estudo transversal e descritivo, utilizando prontuários de 257 crianças e adolescentes portadores de neoplasias de sistema nervoso central, tratados com radioterapia no Instituto Nacional de Câncer, no Rio de Janeiro, de 2012 a 2020. As variáveis foram divididas em sociodemográficas, clínicas e patológicas. Resultados: Crianças brancas do sexo masculino foram as mais prevalentes, com mediana de idade de 7,5 anos no diagnóstico e de 8,0 anos na radioterapia. A maioria possuía casa própria com infraestrutura básica e renda familiar de até um salário-mínimo. Os sintomas mais comuns foram cefaleia, vômitos e alterações na marcha, variando de acordo com a localização do tumor e a idade da criança. A radioterapia foi utilizada em 64% dos pacientes, principalmente para meduloblastoma, tumores de tronco cerebral e ependimoma anaplásico. Conclusão: Estudos específicos sobre crianças e adolescentes com neoplasias cerebrais tratados com radioterapia são raros na literatura. Quando comparados com a população em geral, apresentam perfis sociodemográficos e clínicos semelhantes, com diferenças notáveis apenas na frequência dos tipos tumorais

Introduction: Central nervous system neoplasms account for 21% of pediatric cancer cases and are the leading cause of death in this population in developed countries. Advances in neuroimaging have improved diagnosis, and combination therapies have ensured the cure with greater neurological preservation. Surgery is the primary treatment, often combined with radiation therapy and chemotherapy. Objective: To describe socioeconomic findings, signs and symptoms, and histological aspects of pediatric brain tumors treated with radiation therapy. Method: A cross-sectional and descriptive study was conducted with 257 children and adolescents utilizing medical records with central nervous system neoplasms treated with radiotherapy at the National Cancer Institute, Rio de Janeiro, from 2012 to 2020. The variables were divided into sociodemographic, clinical, and pathological categories. Results: White male children were the most prevalent, with a median age at diagnosis of 7.5 years and at radiotherapy, of 8.0 years. The majority had their own home with basic infrastructure and family income of up to one minimum wage. The most common symptoms were headache, vomiting, and gait disorders, varying according to tumor location and child's age. Radiation therapy was used in 64% of patients, mainly for medulloblastoma, brainstem tumors, and anaplastic ependymoma. Conclusion: Specific studies on children and adolescents with brain neoplasms treated with radiotherapy are rare in the literature. When compared with the general population, they present similar sociodemographic and clinical profiles, with remarkable differences only in the frequency of tumor types

Introducción: Las neoplasias del sistema nervioso central representan el 21% del cáncer infantil y juvenil, siendo la principal causa de muerte en esta población en países desarrollados. Los avances en neuroimagen han mejorado su diagnóstico y la combinación de terapias viene permitiendo la cura, con una mayor preservación neurológica. La cirugía es el tratamiento principal, a menudo combinada con radioterapia y quimioterapia. Objetivo: Describir los hallazgos socioeconómicos, los signos y síntomas, y los aspectos histológicos de los tumores cerebrales pediátricos tratados con radioterapia. Método: Estudio transversal y descriptivo utilizando las historias clínicas de 257 niños y adolescentes con neoplasias del sistema nervioso central, que fueron tratados con radioterapia en el Instituto Nacional del Cáncer en Río de Janeiro entre 2012 y 2020. Las variables se dividieron en sociodemográficas, clínicas y patológicas. Resultado: Los niños blancos y de sexo masculino fueron los más prevalentes, con una mediana de edad de 7,5 años en el momento del diagnóstico y de 8,0 años en el momento de la radioterapia. La mayoría poseía una vivienda propia con infraestructura básica y un ingreso familiar de hasta un salario mínimo. Los síntomas más comunes fueron dolor de cabeza, vómitos y alteraciones en la marcha, que variaban según la ubicación del tumor y la edad del niño. Se utilizó radioterapia en el 64% de los pacientes, principalmente para meduloblastoma, tumores del tronco cerebral y ependimoma anaplásico. Conclusión: Los estudios específicos sobre niños y adolescentes con neoplasias cerebrales tratados con radioterapia son raros en la literatura. Cuando se comparan con la población en general, presentan perfiles sociodemográficos y clínicos similares, con diferencias notables solo en la frecuencia de los tipos tumorales

Cranial Radiation Therapy as Salvage in the Treatment of Relapsed Primary CNS Lymphoma.

Primary central nervous system lymphoma (PCNSL) is a rare malignancy. Standard of care is upfront high-dose methotrexate (HD-MTX) chemotherapy, while cranial radiation is more commonly used in the salvage setting. In this retrospective study, we aimed to investigate the safety and efficacy of salvage cranial radiation in PCNSL. PCNSL patients who received upfront HD-MTX chemotherapy and salvage cranial radiation after treatment failure between 1995 and 2018 were selected. Radiological response to cranial radiation was assessed as per Response Assessment in Neuro-Oncology Criteria. Twenty one patients were selected (median age 59.9 years), with median follow-up of 19.9 months. Fourteen patients (66.7%) received a boost to the gross tumour volume (GTV). Four patients (19.0%) sustained grade ≥2 treatment-related neurotoxicity post-completion of cranial radiation. Of the 19 patients who had requisite MRI with gadolinium imaging available for Response Assessment in Neuro-Oncology (RANO) criteria assessment, 47.4% achieved complete response, 47.4% achieved partial response, and 5.3% of patients exhibited stable disease. Higher dose to the whole brain (>30 Gy) was associated with higher rate of complete response (63.6%) than lower dose (≤30 Gy, 37.5%), while boost dose to the gross disease was also associated with higher rate of complete response (61.5%) compared with no boost dose (33.3%). Median overall survival was 20.0 months. PCNSL patients who relapsed following upfront chemotherapy showed a high rate of response to salvage cranial radiation, especially in those receiving greater than 30 Gy to the whole brain and boost to gross disease.