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All ranked pediatric cancer papers
This scoping review of 18 studies comprising 365 patients reports heterogeneous definitions of idiopathic syringomyelia, generally favorable conservative outcomes in clinically stable children, and evidence that occult arachnoid or cerebrospinal-fluid-flow abnormalities may underlie many adult cases.
The reviewed evidence supports clinical observation for many stable pediatric cases and substrate-directed surgery in selected symptomatic patients with identified arachnoid pathology; any relevance to pediatric oncology is indirect because tumor-associated syringomyelia was explicitly excluded, so application to children with cancer would require separate study.
In a retrospective Dutch nationwide cohort, 14 of 76 eligible children undergoing extremity soft tissue sarcoma resection developed 90-day wound complications, with higher odds associated with neoadjuvant chemotherapy and preoperative radiotherapy.
The study provides observational evidence that neoadjuvant chemotherapy and preoperative radiotherapy are associated with postoperative wound complications; it is plausible—but not tested here—that risk-adapted treatment sequencing, multidisciplinary surgical planning, or selective reconstructive coverage could reduce this morbidity.
In 14 young Jordanian patients with clinical or immunohistochemical evidence of mismatch repair deficiency, the study identified pathogenic or likely pathogenic mismatch-repair variants in nine families, characterized tumor mutational features, and found one ultrahypermutated tumor with a somatic POLE proofreading-domain mutation.
The reported germline and tumor molecular findings support improved identification of CMMRD/Lynch-associated cancers and molecular stratification in this population; it is plausible—but not tested here—that validated MMRD, hypermutation, or combined MMRD–POLE status could inform treatment selection or surveillance.
In 143 surgically treated patients with skull-base juvenile nasopharyngeal angiofibroma, the internally validated GVV score predicted several intraoperative bleeding outcomes better than the Fisch-Andrews and UPMC systems and showed comparable recurrence discrimination.
The evidence supports the GVV score as a prognostic risk-stratification tool within the analyzed cohort; it may, by inference, help tailor perioperative preparation, bleeding-mitigation strategies, and recurrence surveillance, but the record does not show that score-guided management improves clinical outcomes.
This paper presents an updated European expert-consensus effort to harmonize diagnosis and management recommendations for exceptionally rare olfactory neuroblastoma in children and adolescents.
The record supports the value of pediatric-specific, internationally harmonized clinical guidance; it is reasonable—but not demonstrated here—to hypothesize that standardizing diagnosis, staging, and multimodal management could improve treatment selection and outcomes.
In a multicenter retrospective cohort of 53 children with Down syndrome and newly diagnosed myeloid leukemia in Türkiye, DS-related myeloid leukemia was associated with significantly better 5-year overall and event-free survival than standard AML occurring in children with Down syndrome.
The observed outcome differences support distinguishing DS-ML from standard AML-DS for treatment planning; it is reasonable but not proven by this retrospective study that subtype-adapted treatment intensity could reduce early mortality or relapse and improve survival.
In a 180-patient retrospective tertiary-center cohort, juvenile dermatomyositis was associated with more calcinosis and arthritis, a different autoantibody profile, and higher remission rates than adult-onset disease, while malignancy-associated dermatomyositis occurred only among adults and increasing age was associated with malignancy and mortality.
The evidence supports age-stratified risk assessment in dermatomyositis; as an inference requiring prospective validation, age and serologic phenotype might help tailor malignancy evaluation and management intensity, but the record does not test a therapy or establish a pediatric-oncology intervention.
In 31 young patients with medulloblastoma, paired silent verb-generation fMRI scans were distinguished before versus shortly after radiotherapy with 71% accuracy, with descriptive mapping identifying altered activation across cerebellar-cerebral language and control regions.
The study provides evidence that task-based fMRI can detect early post-radiotherapy network changes; it is reasonable but unproven to hypothesize that validated longitudinal imaging signatures could help identify neurocognitive toxicity and inform monitoring or future treatment-adaptation strategies.
In a single-center retrospective cohort of 420 children with medulloblastoma and preoperative hydrocephalus, management pathways differed in recorded infection and recurrent hydrocephalus rates, but matched ETV comparisons were non-significant and an internally evaluated VP-shunt infection model showed limited discrimination.
The evidence shows exploratory associations among hydrocephalus-management pathways; it supports the hypothesis—but does not establish—that selecting ETV for anatomically appropriate patients might reduce infection relative to VP shunting without substantially increasing recurrent hydrocephalus, a possibility requiring prospective multi-institutional validation.
In a single-center retrospective cohort of 145 RUNX1-mutated AML patients, single-site and multi-site RUNX1 mutations were associated with similar remission and survival outcomes, while allo-HSCT was associated with better OS and RFS in both groups.
The observed associations support the hypothesis that allo-HSCT may improve outcomes in RUNX1-mutated AML regardless of RUNX1 mutation multiplicity; however, this is an inference from nonrandomized retrospective data and does not establish transplant efficacy or pediatric applicability.
This report describes two young women with aggressive, treatment-refractory SMARCB1/INI1-deficient PTCL-NOS, documenting characteristic pathology and distinct genetic routes to SMARCB1 inactivation while reviewing the limited literature on this emerging entity.
The cases support SMARCB1/INI1 loss as a diagnostic and biologic feature of this lymphoma; the suggestion that this loss could confer sensitivity to histone deacetylase inhibitors is an inference from emerging prior evidence and was not prospectively tested or shown to produce benefit in the two reported patients.
In a cross-sectional comparison of 30 children within five years after ALL therapy and 64 healthy controls, HR-pQCT identified reduced cortical bone parameters, while all survivors had normal lumbar-spine DXA z-scores and 17% had vertebral fractures.
Evidence: HR-pQCT detected cortical abnormalities not apparent on lumbar-spine DXA in pediatric ALL survivors. Inference requiring prospective validation: adding HR-pQCT to survivorship assessment could identify children with occult skeletal fragility who may benefit from closer monitoring or future bone-protective interventions, but this study did not test screening outcomes or treatment efficacy.