Journal of Pediatric Neurology
DOI: 10.1055/s-0041-1723755
Case Report

Purely Intraventricular Desmoplastic Infantile Astrocytoma

Giovanni Federico Nicoletti
1  Neurosurgery Unit, Highly Specialized Hospital of National Importance “Garibaldi,” Catania, Italy
,
Giuseppe Emmanuele Umana
2  Department of Neurosurgery, Cannizzaro Hospital, Trauma Center, Gamma Knife Center, Catania, Italy
,
Carmelo Riolo
1  Neurosurgery Unit, Highly Specialized Hospital of National Importance “Garibaldi,” Catania, Italy
,
Gaetano Magro
3  Anatomic Pathology Section, Department of Medical, Surgical Sciences and Advanced Technologies and Biotechnological Sciences, G.F. Ingrassia, University of Catania, Catania, Italy
,
Giovanni Bartoloni
4  Anatomic Pathology Unit, Highly Specialized Hospital of National Importance “Garibaldi,” Catania, Italy
,
Gianluca Scalia
1  Neurosurgery Unit, Highly Specialized Hospital of National Importance “Garibaldi,” Catania, Italy
› Author Affiliations
Funding None.

Abstract

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.

Patient Consent

An informed written consent was obtained from the patient's next of kin.




Publication History

Received: 31 October 2020

Accepted: 22 December 2020

Publication Date:
11 February 2021 (online)

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany