Pediatrics supratentorial intraventricular atypical teratoid/rhabdoid tumors: a case report and a systematic review of the literature.
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UNLABELLED: Atypical teratoid/rhabdoid tumors (AT/RTs) are rare, highly aggressive World Health Organization grade 4 brain tumors associated with early cerebrospinal fluid dissemination (CSF). AT/RTs typically occur in children under 4 years of age however, can also occur in adults and often carry a poor prognosis. These tumors are exceptionally uncommon, with evidence limited to isolated case reports. We illustrate a case of hemorrhagic left lateral ventricular AT/RT in a 5-year-old boy and systematically synthesize global literature evaluating clinical presentations, therapeutic interventions, and survival outcomes of primary supratentorial intraventricular AT/RT. Systematic searches were executed across PubMed, Embase, and Scopus databases up to 31 May 2026. Studies tracking histologically or immunohistochemically confirmed pediatric intraventricular AT/RT were included. Study quality was evaluated qualitatively via Joanna Briggs Institute tools; qualitative descriptive analysis synthesized outcomes, supplemented by post hoc stratified subgroup analysis. Twenty-four descriptive, uncontrolled studies comprising 28 pediatric patients (mean age: 3.9 years) were synthesized. The lateral ventricle was the primary epicenter (53.5%). Surgical resection margins were divided between gross total resection (GTR; 64.2%), subtotal resection (STR 17.8%), and biopsy (7.1%). The cohort demonstrated a 43.0% disease progression rate and a 69.6% (16/23) overall mortality rate among patients with follow up. Descriptive subgroup analysis demonstrated a comparable high mortality across age groups and tumor locations, whereas mortality was highest among patients with metastatic disease and appeared lower after GTR than STR 33.3% (6/18) vs. 80.0% (4/5). CONCLUSION: High risk of publication bias and inconsistent longitudinal tracking limit the evidence. Supratentorial intraventricular AT/RT exhibits high recurrence risks, but GTR combined with modern risk-adapted adjuvant protocols optimizes survival. Establishing international multi-institutional registries remains critical. WHAT IS KNOWN: • Atypical teratoid/rhabdoid tumors (AT/RTs) are rare, highly aggressive pediatric central nervous system tumors characterized by early cerebrospinal fluid dissemination (CSF) which carry a poor survival rate. While AT/RTs typically arise in the posterior fossa, primary supratentorial intraventricular tumors are rare, and current evidence is limited to isolated case reports and case series. WHAT IS NEW: • This study provides the largest qualitative synthesis of pediatric supratentorial intraventricular AT/RTs reported to date. The accompanying case is notable for its hemorrhagic lateral ventricular presentation causing obstructive hydrocephalus, diffuse leptomeningeal dissemination at diagnosis (M3 disease) with rapid clinical progression highlighting the aggressive behavior and surgical challenges associated with this rare tumor subtype.