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.
Key-words:
Atypical teratoid/rhabdoid tumor - children - lateral ventricle - radiosurgery - surgery