Atypical teratoid/rhabdoid tumor of the third ventricle in a pediatric patient: a case report

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and highly malignant embryonal tumor of the central nervous system. It typically occurs in children younger than 3 years and is most commonly located in the posterior fossa. Intraventricular locations, such as the third ventricle, are extremely rare and pose significant diagnostic and therapeutic challenges. Here, we report the case of a 4-year-old girl presenting with symptoms of elevated intracranial pressure, including headache, nausea, and vomiting. She was neurologically intact at presentation. Neuroimaging revealed a heterogeneous mass within the third ventricle, obstructing the foramen of Monro. An endoscopic biopsy was performed to obtain diagnostic tissue, and a ventriculoperitoneal shunt was placed to manage hydrocephalus. Histopathological examination confirmed AT/RT. This rare case underscores the need to consider AT/RT in the differential diagnosis of intraventricular cystic–solid tumors in early childhood and offers insight into the diagnostic workup and surgical management of similar cases.