Atypical teratoid rhabdoid tumor of the lateral ventricle

Atypical teratoid/rhabdoid tumors (AT/RTs) are rare and highly malignant embryonal central nervous system neoplasms, usually seen in very young children with rapid fatal outcome despite aggressive treatment. They are most commonly located in the posterior fossa. Intraventricular location is extremely rare. To the best of our knowledge, only 4 cases of lateral ventricle location were reported in the literature. We report the fifth case of lateral ventricle AT/RT in a 2-month-old male who presented with rapid increase of his head circumference. Brain computed tomography scan and magnetic resonance imaging showed heterogeneous huge mass within the left lateral ventricle extending to the parieto-occipital parenchyma and markedly enhancing by contrast. The baby underwent left transparietal approach with complete removal of the tumor. Histological examination confirmed the diagnosis of AT/RT. An aggressive chemotherapy was administrated postoperatively. The outcome is good without neurological deficit or recurrence after 3 years and half of follow-up.