Abstract
Abstract Atypical teratoid/rhabdoid tumor (AT/RT) is a biologically aggressive tumor of the central nervous system, usually occurring in the posterior fossa in infants and young children. Supratentorial location is relatively rare, especially tumors arising primarily within the lateral ventricles. Prognosis remains poor, despite multiple treatment modalities consisting of surgical excision, radiotherapy, and chemotherapy. We present the case of a 3-year-old girl with an AT/RT of the lateral ventricle with diffuse leptomeningeal and spinal metastasis on magnetic resonance imaging. She underwent a left frontoparietal craniotomy and near-total excision of the tumor. Immunohistochemistry confirmed the diagnosis and the child died within 3 months of surgery. There are isolated case reports in the literature regarding primary lateral ventricular AT/RTs, although secondary involvement of the ventricle from an adjacent tumor is commoner. Ours is the first case that demonstrates AT/RT arising within the body of the lateral ventricle causing extensive metastasis, both within the brain and spine. Given the dismal prognosis, the choice between further adjuvant treatment and palliative care is a matter of debate that necessitates a truthful and meaningful discussion with the parents.
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