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Published in: Child's Nervous System 8/2023

20-03-2023 | Langerhans Cell Histiocytosis | Case Report

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

Authors: Alberto Benato, Giulio Riva, Fabio Raneri

Published in: Child's Nervous System | Issue 8/2023

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Abstract

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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Metadata
Title
Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature
Authors
Alberto Benato
Giulio Riva
Fabio Raneri
Publication date
20-03-2023
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 8/2023
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-023-05926-z

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