Atypical teratoid/rhabdoid tumor(AT/RT)is a rare malignancy located primarily in infrat-entorial or subcortical areas with a poor prognosis,and rarely in the lateral ventricle with a very poor prognosis.So far,only 6 cases of AT/RT in lateral ventricle have been reported in China.This article reports the diagnosis and treatment of four children with AT/RT in the lateral ventricle,and discusses the clinical manifestations,differentiation and diagnosis,treatment and prognosis of the disease through literature review,in order to im-prove clinicians'understanding of the disease and reduce missed diagnosis and misdiagnosis.
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