Document Detail


Optic nerve tumor in tuberous sclerosis complex is not responsive to sirolimus.
MedLine Citation:
PMID:  20472200     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 12-year-old girl with clinically established tuberous sclerosis complex, and without signs of neurofibromatosis type 1, developed a right retro-ocular optic nerve tumor. After rapid growth for 1 year after its discovery, the optic nerve tumor demonstrated modest progression. The patient received the mammalian target of rapamycin inhibitor, sirolimus, for recurrent subependymal giant cell brain tumors. Although her left ventricular subependymal giant cell tumor demonstrated a 49% reduction in volume, the optic nerve tumor did not respond, and even underwent slight (6%) growth during the 16-month treatment. The quality of this child's vision has remained normal in both eyes, and she is otherwise asymptomatic with regard to the optic nerve tumor.
Authors:
Steven P Sparagana; David C Wilkes; Catherine E Thompson; Daniel C Bowers
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric neurology     Volume:  42     ISSN:  1873-5150     ISO Abbreviation:  Pediatr. Neurol.     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-05-17     Completed Date:  2010-08-13     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8508183     Medline TA:  Pediatr Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  443-6     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier Inc. All rights reserved.
Affiliation:
Division of Neurology, Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, TX 75219, USA. steven.sparagana@tsrh.org
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MeSH Terms
Descriptor/Qualifier:
Antibiotics, Antineoplastic / therapeutic use
Astrocytoma / drug therapy*,  pathology
Brain Neoplasms / drug therapy*,  pathology
Child
Female
Humans
Neoplasm Recurrence, Local / drug therapy*,  pathology
Optic Nerve Neoplasms / drug therapy*,  etiology,  pathology
Sirolimus / therapeutic use*
Treatment Outcome
Tuberous Sclerosis / complications*,  pathology
Chemical
Reg. No./Substance:
0/Antibiotics, Antineoplastic; 53123-88-9/Sirolimus

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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