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The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report

INTRODUCTION: The neurofibromatoses are inherited tumor predisposition syndromes involving two major clinical phenotypes: neurofibromatosis type 1 (von Recklinghausen's disease) is linked to chromosome 17q, and tends to occur seven times more frequently than neurofibromatosis type 2. Neurofibro...

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Autores principales: Stanger, Katrin, De Kerviler, Sora, Vajtai, Istvan, Constantinescu, Mihai
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3751089/
https://www.ncbi.nlm.nih.gov/pubmed/23890233
http://dx.doi.org/10.1186/1752-1947-7-189
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author Stanger, Katrin
De Kerviler, Sora
Vajtai, Istvan
Constantinescu, Mihai
author_facet Stanger, Katrin
De Kerviler, Sora
Vajtai, Istvan
Constantinescu, Mihai
author_sort Stanger, Katrin
collection PubMed
description INTRODUCTION: The neurofibromatoses are inherited tumor predisposition syndromes involving two major clinical phenotypes: neurofibromatosis type 1 (von Recklinghausen's disease) is linked to chromosome 17q, and tends to occur seven times more frequently than neurofibromatosis type 2. Neurofibromatosis type 1 entails a distinctive cutaneous manifestation prevailed upon by benign neurofibromas, which may vary in size, number and distribution. On the histological level, neurofibromas are composed of an admixture of neurilemmal cells, including Schwann cells, fibroblasts, and – to a lesser extent – perineurial cells. CASE PRESENTATION: The case of a 39-year-old Caucasian man with a voluminous recurrent neurofibroma of 27×15cm extending from the left gluteal region to thoraco-lumbar levels Th6 through L4 is reported. Within the soft tissue tumor a pseudocyst of 7.3×9.3cm was found preoperatively. CONCLUSION: Histopathological study of the excised mass was conspicuous for revealing a large number of multinucleated floret-like giant cells within an otherwise classical soft tissue neurofibroma. Previous reports on neurofibromas with multinucleated floret-like giant cells are distinctly scant. Available evidence from the literature does not suggest any consistent correlation of multinucleated floret-like giant cells in neurofibromas with gender, age, traumatic antecedents, size of the lesion, recurrence, or malignant transformation. Furthermore, the presence of such cells may not be specific for neurofibromatosis type 1, as they occasionally are encountered in some unrelated mesenchymal neoplasms as well.
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spelling pubmed-37510892013-08-24 The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report Stanger, Katrin De Kerviler, Sora Vajtai, Istvan Constantinescu, Mihai J Med Case Rep Case Report INTRODUCTION: The neurofibromatoses are inherited tumor predisposition syndromes involving two major clinical phenotypes: neurofibromatosis type 1 (von Recklinghausen's disease) is linked to chromosome 17q, and tends to occur seven times more frequently than neurofibromatosis type 2. Neurofibromatosis type 1 entails a distinctive cutaneous manifestation prevailed upon by benign neurofibromas, which may vary in size, number and distribution. On the histological level, neurofibromas are composed of an admixture of neurilemmal cells, including Schwann cells, fibroblasts, and – to a lesser extent – perineurial cells. CASE PRESENTATION: The case of a 39-year-old Caucasian man with a voluminous recurrent neurofibroma of 27×15cm extending from the left gluteal region to thoraco-lumbar levels Th6 through L4 is reported. Within the soft tissue tumor a pseudocyst of 7.3×9.3cm was found preoperatively. CONCLUSION: Histopathological study of the excised mass was conspicuous for revealing a large number of multinucleated floret-like giant cells within an otherwise classical soft tissue neurofibroma. Previous reports on neurofibromas with multinucleated floret-like giant cells are distinctly scant. Available evidence from the literature does not suggest any consistent correlation of multinucleated floret-like giant cells in neurofibromas with gender, age, traumatic antecedents, size of the lesion, recurrence, or malignant transformation. Furthermore, the presence of such cells may not be specific for neurofibromatosis type 1, as they occasionally are encountered in some unrelated mesenchymal neoplasms as well. BioMed Central 2013-07-26 /pmc/articles/PMC3751089/ /pubmed/23890233 http://dx.doi.org/10.1186/1752-1947-7-189 Text en Copyright © 2013 Stanger et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Stanger, Katrin
De Kerviler, Sora
Vajtai, Istvan
Constantinescu, Mihai
The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
title The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
title_full The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
title_fullStr The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
title_full_unstemmed The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
title_short The riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
title_sort riddle of multinucleated “floret-like” giant cells and their detection in an extensive gluteal neurofibroma: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3751089/
https://www.ncbi.nlm.nih.gov/pubmed/23890233
http://dx.doi.org/10.1186/1752-1947-7-189
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