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Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

INTRODUCTION: Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growin...

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Autores principales: Benato, Alberto, Riva, Giulio, Raneri, Fabio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10025804/
https://www.ncbi.nlm.nih.gov/pubmed/36939904
http://dx.doi.org/10.1007/s00381-023-05926-z
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author Benato, Alberto
Riva, Giulio
Raneri, Fabio
author_facet Benato, Alberto
Riva, Giulio
Raneri, Fabio
author_sort Benato, Alberto
collection PubMed
description INTRODUCTION: Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited. METHODS: Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG. RESULTS: In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination. CONCLUSION: Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.
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spelling pubmed-100258042023-03-21 Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature Benato, Alberto Riva, Giulio Raneri, Fabio Childs Nerv Syst Case Report INTRODUCTION: Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited. METHODS: Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG. RESULTS: In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination. CONCLUSION: Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG. Springer Berlin Heidelberg 2023-03-20 /pmc/articles/PMC10025804/ /pubmed/36939904 http://dx.doi.org/10.1007/s00381-023-05926-z Text en © The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023, Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Case Report
Benato, Alberto
Riva, Giulio
Raneri, Fabio
Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
title Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
title_full Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
title_fullStr Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
title_full_unstemmed Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
title_short Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
title_sort eosinophilic granuloma of the calvarium: is conservative management a valid option? illustrative case and systematic review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10025804/
https://www.ncbi.nlm.nih.gov/pubmed/36939904
http://dx.doi.org/10.1007/s00381-023-05926-z
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