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Child's Nervous System
Published in: Child's Nervous System 5/2020

01-05-2020 | Astrocytoma | Original Article

Neurosurgical treatment of subependymal giant cell astrocytomas in tuberous sclerosis complex: a series of 44 surgical procedures in 31 patients

Authors: Flavio Giordano, Carla Moscheo, Matteo Lenge, Roberto Biagiotti, Francesco Mari, Iacopo Sardi, Anna Maria Buccoliero, Lorenzo Mongardi, Eleonora Aronica, Renzo Guerrini, Lorenzo Genitori

Published in: Child's Nervous System | Issue 5/2020

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Abstract

Background

Subependymal giant cell astrocytomas (SEGA) are benign tumors characteristic of tuberous sclerosis complex (TSC) that may cause hydrocephalus. Various treatments are nowadays available as mTOR inhibitors or surgery. Surgery is still a valid option especially for symptomatic and larger tumors.

Methods

From January 1994 to December 2015, 31 TSC patients harboring SEGA underwent surgery at the Department of Neurosurgery of the Meyer Pediatric Hospital, Florence. Indications for surgery were tumor size and location, growth and cystization/hemorrhage, and hydrocephalus. Clinical data, preoperative and postoperative MRI, recurrence rate, further surgical procedures, and related complications were analyzed.

Results

A total of 44 surgeries were performed in 31 TSC patients affected by SEGA, achieving gross total removal (GTR) and subtotal removal (STR), respectively, in 36 and 8 patients. Recurrences occurred in 11 patients; 9 of them underwent further surgical procedures and 2 were treated with mTOR pathway inhibitors. Surgical morbidity and mortality were, respectively, 22.7% and 2.3%. After a mean follow-up of 4.9 years, 90% of patients were tumor-free with good neurological status in 93.3%; twelve (40%) had a ventriculo-peritoneal shunt (VPS) for hydrocephalus.

Conclusions

The present series confirms that the surgical approach, combined with mTOR inhibitors, is still a valid option for the treatment of SEGAs.
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Metadata
Title
Neurosurgical treatment of subependymal giant cell astrocytomas in tuberous sclerosis complex: a series of 44 surgical procedures in 31 patients
Authors
Flavio Giordano
Carla Moscheo
Matteo Lenge
Roberto Biagiotti
Francesco Mari
Iacopo Sardi
Anna Maria Buccoliero
Lorenzo Mongardi
Eleonora Aronica
Renzo Guerrini
Lorenzo Genitori
Publication date
01-05-2020
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 5/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04449-w

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