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Is “watchful waiting” superior to surgery in children with neurofibromatosis type 1 presenting with extracranial and extramedullary tumor mass at diagnosis?

Identifieur interne : 000248 ( Istex/Corpus ); précédent : 000247; suivant : 000249

Is “watchful waiting” superior to surgery in children with neurofibromatosis type 1 presenting with extracranial and extramedullary tumor mass at diagnosis?

Auteurs : Wojciech Wozniak ; Marek W. Karwacki

Source :

RBID : ISTEX:B2ABE1D8A8C22852CB09EF2408D00FE1973B8862

English descriptors

Abstract

Abstract: Aim: The aim of this study was to compare aggressive surgery to “watchful waiting” in neurofibromatosis type 1 (NF-1) patients with benign peripheral nerve sheath tumors exceeding 150 cm3. Materials and methods: Observational study based on imaging studies and results of surgery was utilized. Results: Twenty-seven out of 114 NF-1 patients (mean age of 12.8 years) registered throughout the last 10 years were studied. The mean tumor volume was 237 cm3. Among them, 18 (66%) underwent surgery. In 15 (84%), regrowth of a tumor was observed. Reoperation was necessary in five (27%). Two out of these five children (2/18, 11%) required a third operation. Stable process has been shown in the remaining nine children (34%) since diagnosis and in most operated children (10/18; 56%), besides the regrowth of the tumor. Conclusions: “Watchful waiting” could be superior to aggressive surgery in selected patients without “oncological awareness” confirmed by imaging techniques (mostly magnetic resonance imaging). NF-1 children with gross tumor mass require lifetime imaging monitoring and complex medical evaluation.

Url:
DOI: 10.1007/s00381-008-0668-7

Links to Exploration step

ISTEX:B2ABE1D8A8C22852CB09EF2408D00FE1973B8862

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<affiliation>E-mail: marekwk@astercity.net</affiliation>
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<dateCreated encoding="w3cdtf">2007-12-23</dateCreated>
<dateIssued encoding="w3cdtf">2008-12-01</dateIssued>
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<abstract lang="en">Abstract: Aim: The aim of this study was to compare aggressive surgery to “watchful waiting” in neurofibromatosis type 1 (NF-1) patients with benign peripheral nerve sheath tumors exceeding 150 cm3. Materials and methods: Observational study based on imaging studies and results of surgery was utilized. Results: Twenty-seven out of 114 NF-1 patients (mean age of 12.8 years) registered throughout the last 10 years were studied. The mean tumor volume was 237 cm3. Among them, 18 (66%) underwent surgery. In 15 (84%), regrowth of a tumor was observed. Reoperation was necessary in five (27%). Two out of these five children (2/18, 11%) required a third operation. Stable process has been shown in the remaining nine children (34%) since diagnosis and in most operated children (10/18; 56%), besides the regrowth of the tumor. Conclusions: “Watchful waiting” could be superior to aggressive surgery in selected patients without “oncological awareness” confirmed by imaging techniques (mostly magnetic resonance imaging). NF-1 children with gross tumor mass require lifetime imaging monitoring and complex medical evaluation.</abstract>
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<subject lang="en">
<genre>Keywords</genre>
<topic>Neurofibromatosis type 1</topic>
<topic>Peripheral nerve sheath tumors</topic>
<topic>Gross tumor mass</topic>
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<title>Child's Nervous System</title>
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<title>Childs Nerv Syst</title>
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<dateIssued encoding="w3cdtf">2008-11-11</dateIssued>
<copyrightDate encoding="w3cdtf">2008</copyrightDate>
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<genre>Medicine & Public Health</genre>
<topic>Neurosciences</topic>
<topic>Neurosurgery</topic>
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<identifier type="ISSN">0256-7040</identifier>
<identifier type="eISSN">1433-0350</identifier>
<identifier type="JournalID">381</identifier>
<identifier type="IssueArticleCount">22</identifier>
<identifier type="VolumeIssueCount">12</identifier>
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<date>2008</date>
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<number>24</number>
<caption>vol.</caption>
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<number>12</number>
<caption>no.</caption>
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<start>1431</start>
<end>1436</end>
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<identifier type="DOI">10.1007/s00381-008-0668-7</identifier>
<identifier type="ArticleID">668</identifier>
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<accessCondition type="use and reproduction" contentType="copyright">Springer-Verlag, 2008</accessCondition>
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