Purely Intraventricular Desmoplastic Infantile Astrocytoma

Desmoplastic infantile astrocytomas (DIAs) are rare pediatric tumors characterized by superficial brain cortex involvement, along with the meninges of the supratentorial compartment, and are classified as grade I neoplasms according to the 2016 World Health Organization. A 5-year-old female patient was admitted to our unit with abnormal decorticate posturing, bilateral mydriasis with weak pupillary light reflex, and brisk lower limbs reflexes. Her medical history was unremarkable. Magnetic resonance imaging of the brain revealed a massive lesion with bilateral intraventricular growth mainly prevailing on the left and involving the ipsilateral foramen of Monro. After external ventricular drainage positioning, Dandy's transfrontal transcortical approach to the left lateral ventricle, a meticulous ependymal microsurgical dissection of the lesion was performed, resulting in an excision of the cystic component of the left lateral ventricle. A gross total removal of the lesion was performed with an en bloc resection of the deeper cystic part. Thirty days after surgery, the patient presented with fluent speech, conserved axial, and extremity sensorimotor functions, except a mild central facial paresis which progressively improved. To the best of our knowledge, this is the first case of DIA characterized by purely intraventricular growth. Tumor recurrence, although considered rare, represents an unpredictable event. Therefore, an adequate follow-up must be reserved for each patient.