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The clinical diagnosis and management options for intracranial juvenile xanthogranuloma in children: based on four cases and another 39 patients in the literature

  • Case Report - Brain Tumors
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Abstract

Background

Juvenile xanthogranulomas (JXGs) are uncommon non-Langerhans cell histiocytic proliferations which occur most often in children. Rare cases of intracranial JXGs in children have been reported. The precise treatment strategy for intracranial JXG with high fatality is still unclear.

Method

We present four cases of intracranial JXG with 2–6 years of follow-up. Review of the previous literature since 1980 revealed another 39 pediatric intracranial JXGs.

Results

Their clinical characteristics varied significantly. Most intracranial JXGs presented in young children (88 %). Males (72 %) were affected more often than females. The differential diagnosis included two important components: the magnetic resonance imaging (MRI) characteristics and the pathohistiocytic markers. Statistical analysis suggested that there were no significant association between resection of intracranial lesions, multiple intracranial lesions, systematic lesions and clinic outcome (p = 0.12, p = 0.13, p = 0.60 respectively). Also, the manifestation with multiple intracranial lesions did not have a significant association with systematic JXG (p = 0.26).

Conclusions

We found no significant associations between clinic characteristics, surgical resection and outcome. When feasible, total surgical resection of intracranial lesion may be curative.

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Acknowledgments

This work was supported by grants from the National Natural Science Foundation of China (81271382)

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Authors and Affiliations

  1. Department of pediatric Neurosurgery, Xinhua hospital, Shanghai Jiao Tong University, Shanghai, 200092, China

    Baocheng Wang, Huiming Jin, Yang Zhao & Jie Ma

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  1. Baocheng Wang
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  2. Huiming Jin
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Correspondence to Jie Ma.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study. The patient/next of kin/guardian has consented to submission of this case report to the journal.

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No funding was received for this research.

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Wang, B., Jin, H., Zhao, Y. et al. The clinical diagnosis and management options for intracranial juvenile xanthogranuloma in children: based on four cases and another 39 patients in the literature. Acta Neurochir 158, 1289–1297 (2016). https://doi.org/10.1007/s00701-016-2811-7

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  • DOI: https://doi.org/10.1007/s00701-016-2811-7

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