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

Haci Bayram Tosun,1 Sancar Serbest,2 Bilge Aydin Turk,3 Seyit Ali Gumustas,1 Abuzer Uludag1 1Department of Orthopaedics and Traumatology, Faculty of Medicine, Adiyaman University, Adiyaman, 2Department of Orthopaedics and Traumatology, Faculty of Medicine, Kirikkale University, Kirikkale, 3Departmen...

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Main Authors: Tosun HB, Serbest S, Turk BA, Gumustas SA, Uludag A
Format: Article
Language:English
Published: Dove Medical Press 2015-10-01
Series:International Medical Case Reports Journal
Online Access:https://www.dovepress.com/giant-malignant-peripheral-nerve-sheath-tumor-of-thigh-in-an-adolescen-peer-reviewed-article-IMCRJ
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author Tosun HB
Serbest S
Turk BA
Gumustas SA
Uludag A
author_facet Tosun HB
Serbest S
Turk BA
Gumustas SA
Uludag A
author_sort Tosun HB
collection DOAJ
description Haci Bayram Tosun,1 Sancar Serbest,2 Bilge Aydin Turk,3 Seyit Ali Gumustas,1 Abuzer Uludag1 1Department of Orthopaedics and Traumatology, Faculty of Medicine, Adiyaman University, Adiyaman, 2Department of Orthopaedics and Traumatology, Faculty of Medicine, Kirikkale University, Kirikkale, 3Department of Pathology, Faculty of Medicine, Adiyaman University, Adiyaman, Turkey Abstract: 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. Keywords: nerve sheath neoplasm, sarcoma, adolescent, neurofibromatosis, lower extremity
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spelling doaj.art-1f0b179dfcb642caa643e01a5de781ba2022-12-22T02:55:21ZengDove Medical PressInternational Medical Case Reports Journal1179-142X2015-10-012015default26727124343Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case reportTosun HBSerbest STurk BAGumustas SAUludag AHaci Bayram Tosun,1 Sancar Serbest,2 Bilge Aydin Turk,3 Seyit Ali Gumustas,1 Abuzer Uludag1 1Department of Orthopaedics and Traumatology, Faculty of Medicine, Adiyaman University, Adiyaman, 2Department of Orthopaedics and Traumatology, Faculty of Medicine, Kirikkale University, Kirikkale, 3Department of Pathology, Faculty of Medicine, Adiyaman University, Adiyaman, Turkey Abstract: 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. Keywords: nerve sheath neoplasm, sarcoma, adolescent, neurofibromatosis, lower extremityhttps://www.dovepress.com/giant-malignant-peripheral-nerve-sheath-tumor-of-thigh-in-an-adolescen-peer-reviewed-article-IMCRJ
spellingShingle Tosun HB
Serbest S
Turk BA
Gumustas SA
Uludag A
Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report
International Medical Case Reports Journal
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
url https://www.dovepress.com/giant-malignant-peripheral-nerve-sheath-tumor-of-thigh-in-an-adolescen-peer-reviewed-article-IMCRJ
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