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Subependymal
giant cell astrocytoma in children with tuberous sclerosis
Cuccia V, Zuccaro G, Sosa F, Monges J, Lubienieky F, Taratuto AL
Department of Pediatric
Neurosurgery, Hospital Nacional de Pediatria Prof. Juan P. Garrahan, Combate de
los Pozos 1881, 1245 Buenos Aires, Argentina. vcuccia@uolmail.com.ar
Methods. Out of 105 patients with tuberous sclerosis (TS) admitted to the
Hospital Nacional de Pediatria "Juan P. Garrahan" (Buenos Aires,
Argentina), we surgically treated 17 children between January 1988 and December
2000.
Two patients were operated on because of epilepsy and 15 patients because
of an intraventricular tumor (subependymal giant cell astrocytoma [SGCA]).
In
this report we focus on tumors.
Twelve of the 15 patients presented with
hydrocephalus but none of them had a preoperative shunt.
All tumors were
surgically resected using frontal transventricular or transcallosal routes.
Total removal was achieved in 12 out of 15 and subtotal removal in 3 out of 15
patients (resection of 70-95%).
Results. Exeresis of the tumor was not
accompanied by significant morbidity and there was no perioperative mortality.
Seizures and mental retardation did not improve after tumor resection.
It was
necessary to insert a postoperative shunt a long time after surgery in only one
patient.
There were no recurrences of SGCA that were totally removed after a
mean follow-up of 51.7 months.
Conclusions. We encourage surgery as soon as a
lesion is diagnosed as a tumor.
The "transformation" of subependymal
nodules (SEN) into tumors (SGCA) may be considered controversial.
PMID: 12715190 [PubMed - indexed for MEDLINE]
Source:
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12715190&dopt=Abstract
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