The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease

<p>Abstract</p> <p>Background</p> <p>Malignant peripheral nerve sheath tumours (MPNST) are known to develop in patients with Neurofibromatosis type I (NF1) resulting in a decreased overall survival. The association between NF1 and the development of such MPNST has been...

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Main Authors: van Diest Paul J, Wuisman Paul IJM, Bron Johannes L, Brinkman Justus-Martijn, Comans Emile FI, Molthoff Carla FM
Format: Article
Language:English
Published: BMC 2007-11-01
Series:World Journal of Surgical Oncology
Online Access:http://www.wjso.com/content/5/1/130
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author van Diest Paul J
Wuisman Paul IJM
Bron Johannes L
Brinkman Justus-Martijn
Comans Emile FI
Molthoff Carla FM
author_facet van Diest Paul J
Wuisman Paul IJM
Bron Johannes L
Brinkman Justus-Martijn
Comans Emile FI
Molthoff Carla FM
author_sort van Diest Paul J
collection DOAJ
description <p>Abstract</p> <p>Background</p> <p>Malignant peripheral nerve sheath tumours (MPNST) are known to develop in patients with Neurofibromatosis type I (NF1) resulting in a decreased overall survival. The association between NF1 and the development of such MPNST has been investigated in detail. The biological behaviour however of multiple disseminated neurofibromas in patients with NF1 and the risk factors for malignant transformation remain unknown. Clinical signs are unreliable and additional imaging techniques are therefore required. Of such, positron emission tomography using [<sup>18</sup>F]-2-fluoro-2-deoxy-D-glucose (<sup>18</sup>FDG PET) is used to detect malignant changes in neurofibromas.</p> <p>Case presentation</p> <p>A case is presented of a patient suffering from NF1 with clinical signs of malignant change and accumulation of <sup>18</sup>FDG in multiple neurofibromas. Histopathological examination of 20 lesions however, did not reveal any malignant features. There was no statistically significant relation between<sup>18</sup>FDG accumulation and malignant change, but rather with pain, size and growth.</p> <p>Conclusion</p> <p>This case adds to the knowledge of the diverse biological behaviour of neurofibromas in patients with NF1</p>
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spelling doaj.art-2e6183e81bb84c2aab486ccbee74ab422022-12-22T00:37:52ZengBMCWorld Journal of Surgical Oncology1477-78192007-11-015113010.1186/1477-7819-5-130The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's diseasevan Diest Paul JWuisman Paul IJMBron Johannes LBrinkman Justus-MartijnComans Emile FIMolthoff Carla FM<p>Abstract</p> <p>Background</p> <p>Malignant peripheral nerve sheath tumours (MPNST) are known to develop in patients with Neurofibromatosis type I (NF1) resulting in a decreased overall survival. The association between NF1 and the development of such MPNST has been investigated in detail. The biological behaviour however of multiple disseminated neurofibromas in patients with NF1 and the risk factors for malignant transformation remain unknown. Clinical signs are unreliable and additional imaging techniques are therefore required. Of such, positron emission tomography using [<sup>18</sup>F]-2-fluoro-2-deoxy-D-glucose (<sup>18</sup>FDG PET) is used to detect malignant changes in neurofibromas.</p> <p>Case presentation</p> <p>A case is presented of a patient suffering from NF1 with clinical signs of malignant change and accumulation of <sup>18</sup>FDG in multiple neurofibromas. Histopathological examination of 20 lesions however, did not reveal any malignant features. There was no statistically significant relation between<sup>18</sup>FDG accumulation and malignant change, but rather with pain, size and growth.</p> <p>Conclusion</p> <p>This case adds to the knowledge of the diverse biological behaviour of neurofibromas in patients with NF1</p>http://www.wjso.com/content/5/1/130
spellingShingle van Diest Paul J
Wuisman Paul IJM
Bron Johannes L
Brinkman Justus-Martijn
Comans Emile FI
Molthoff Carla FM
The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease
World Journal of Surgical Oncology
title The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease
title_full The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease
title_fullStr The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease
title_full_unstemmed The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease
title_short The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease
title_sort correlation between clinical nuclear and histologic findings in a patient with von recklinghausen s disease
url http://www.wjso.com/content/5/1/130
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