ATRT of lateral ventricle in a child: A Rare Tumor at a Very Rare Location

Atypical teratoid/rhabdoid tumors (AT/RTs) of infancy are highly malignant central nervous system neoplasms that are most commonly seen during the first 2 years of life with limited therapeutic options. To date, only two cases have been described in the lateral ventricle. A 4-year-old boy presented with a 4-month history of increased intracranial pressure. Cerebral magnetic resonance imaging (MRI) revealed a huge intraventricular tumor, occupying the entire temporal horn and the body of the left lateral ventricle. The boy was operated through a left temporal transventricular approach with gross total removal of the lesion. The histopathological diagnosis was an AT/RT. The infant underwent adjuvant chemotherapy and radiation therapy. The 1-year MRI of control showed a local recurrence of the tumor. Then after, Gamma Knife radiosurgery was performed because of the small volume and the deep location of the lesion. At the 3-month follow-up, the MRI showed a significant growth of the tumor volume, and the child was given additional adjuvant chemotherapy. Unfortunately, he died 9 months later. AT/RT of the lateral ventricle is a very rare tumor in children, associated with a poor prognosis in spite of multimodal treatment. Gamma knife surgery (GKS) was rarely reported as a treatment modality of AT/RT. The aim of this work is to discuss about the rarity of this tumor and the best treatment strategy to improve prognosis.