Faculty Collaboration Database

A management strategy for intraventricular subependymal giant cell astrocytomas in tuberous sclerosis complex. J Neurosurg Pediatr 2014 Jan;13(1):21-8

Date

11/05/2013

Pubmed ID

24180681

DOI

10.3171/2013.9.PEDS13193

Scopus ID

2-s2.0-84892427439 (requires institutional sign-in at Scopus site)   27 Citations

Abstract

OBJECT: Subependymal giant cell astrocytomas (SEGAs) are benign tumors, most commonly associated with tuberous sclerosis complex (TSC). The vast majority of these tumors arise from the lateral ependymal surface adjacent to the foramen of Monro, therefore potentially encroaching on one or both foramina, and resulting in obstructive hydrocephalus that necessitates surgical decompression. The indications for surgery, intraoperative considerations, and evolution of the authors' management paradigm are presented.

METHODS: Patients with TSC who underwent craniotomy for SEGA resection at New York University Langone Medical Center between January 1997 and March 2011 were identified. Preoperative imaging, clinical characteristics, management decisions, operative procedures, and outcomes were reviewed.

RESULTS: Eighteen patients with TSC underwent 22 primary tumor resections for SEGAs. The indication for surgery was meaningful radiographic tumor progression in 16 of 21 cases. The average age at the time of operation was 10.3 years. Average follow-up duration was 52 months (range 12-124 months). The operative approach was intrahemispheric-transcallosal in 16 cases, transcortical-transventricular in 5, and neuroendoscopic in 1. Nine tumors were on the right, 9 on the left, and 3 were bilateral. Gross-total resection was documented in 16 of 22 cases in our series, with radical subtotal resection achieved in 4 cases, and subtotal resection (STR) in 2 cases. Two patients had undergone ventriculoperitoneal shunt placement preoperatively and 7 patients required shunt placement after surgery for moderate to severe ventriculomegaly. Two patients experienced tumor progression requiring reoperation; both of these patients had initially undergone STR.

CONCLUSIONS: The authors present their management strategy for TSC patients with SEGAs. Select patients underwent microsurgical resection of SEGAs with acceptable morbidity. Gross-total resection or radical STR was achieved in 90.9% of our series (20 of 22 primary tumor resections), with no recurrences in this group. Approximately half of our patient series required CSF diversionary procedures. There were no instances of permanent neurological morbidity associated with surgery.

Author List

Harter DH, Bassani L, Rodgers SD, Roth J, Devinsky O, Carlson C, Wisoff JH, Weiner HL

Author

Chad Carlson MD Professor in the Neurology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Astrocytoma
Brain Neoplasms
Child
Child, Preschool
Disease Progression
Female
Follow-Up Studies
Hospitals, University
Humans
Infant
Male
Medical Records
Microsurgery
Neoplasm Recurrence, Local
Neuroendoscopy
Neurosurgical Procedures
New York
Reoperation
Retrospective Studies
Tuberous Sclerosis