Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report

Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the ava...

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Detalles Bibliográficos
Autores principales: Tosun, Hacı Bayram, Serbest, Sancar, Turk, Bilge Aydın, Gumustas, Seyit Ali, Uludag, Abuzer
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2015
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4630200/
https://www.ncbi.nlm.nih.gov/pubmed/26604833
http://dx.doi.org/10.2147/IMCRJ.S92015
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author Tosun, Hacı Bayram
Serbest, Sancar
Turk, Bilge Aydın
Gumustas, Seyit Ali
Uludag, Abuzer
author_facet Tosun, Hacı Bayram
Serbest, Sancar
Turk, Bilge Aydın
Gumustas, Seyit Ali
Uludag, Abuzer
author_sort Tosun, Hacı Bayram
collection PubMed
description Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision.
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spelling pubmed-46302002015-11-24 Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report Tosun, Hacı Bayram Serbest, Sancar Turk, Bilge Aydın Gumustas, Seyit Ali Uludag, Abuzer Int Med Case Rep J Case Report Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision. Dove Medical Press 2015-10-28 /pmc/articles/PMC4630200/ /pubmed/26604833 http://dx.doi.org/10.2147/IMCRJ.S92015 Text en © 2015 Tosun et al. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.
spellingShingle Case Report
Tosun, Hacı Bayram
Serbest, Sancar
Turk, Bilge Aydın
Gumustas, Seyit Ali
Uludag, Abuzer
Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
title Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
title_full Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
title_fullStr Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
title_full_unstemmed Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
title_short Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
title_sort giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4630200/
https://www.ncbi.nlm.nih.gov/pubmed/26604833
http://dx.doi.org/10.2147/IMCRJ.S92015
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