Intraventricular Superatentorial AT/RT in pediatrics : a case report and literature review

Abstract Introduction: Atypical teratoid/rhabdoid tumors (AT/RTs) are rare highly malignant and rapidly growing tumors usually seen in the posterior fossa in children and less commonly supratentorial compartment. Intraventricular location is considered even more scarce. The authors report a case of ATRT originating from the lateral ventricle in a child and did a literature review Methodology: To investigate for previous reports of an atypical tertatoid and rhaboid tumors located in the lateral ventricle in children, we performed a literature search that yielded 19 results. These articles were screened for cases of primary supratentorial intraventriuclar AT/RTs.Result: The literature search led to the identification of 11 articles including 11 patients. Herein, the authors present the 12th case of AT/RT in a 5 yr old who presented with vomiting and generalized tonic clonic seizures. Computed tomography scan and magnetic resonance imaging showed heterogeneous huge mass within the left lateral ventricle with central area of hemorrhage and necrosis . The patient underwent Emergency left frontal craniotomy with complete removal of the tumor. Histological examination confirmed the diagnosis of AT/RTConclusion: The results of a literature search suggest that a ATRT arising in the lateral ventricle is quit rare. These cases emphasize on the necessity of considering AT/RT in the list of differential diagnosis of supratentorial intraventricular tumors in pediatrics. The prognosis of this particular subtype is still poor.