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Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report

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Abstract

Case presentation

An 11-year-old boy presented with a complaint of a painful temporal mass. Brain magnetic resonance imaging (MRI) showed a 3-cm-sized, homogeneously enhancing mass in the greater wing of the left sphenoid bone, which was diagnosed as Langerhans cell histiocytosis (LCH). Chemotherapy with vincristine and prednisolone was performed for 1 year. After 1 year and 11 months off treatment, he developed symptoms such as polydipsia and polyuria. Brain MRI showed thickening of the pituitary stalk with enhancement, suggestive of LCH involvement, and no recurrence in the sphenoid bone. After 4 years and 4 months off treatment, he developed multiple, subcutaneous, asymptomatic, and yellowish variable-sized papules on his face, posterior neck, and back, which were pathologically diagnosed as juvenile xanthogranuloma (JXG). Brain MRI revealed multifocal enhancing skull lesions in the left parietal, right frontal, and left occipital bones, which were also diagnosed as JXG. After 5 years and 8 months off treatment, the number of variable-sized skin lesions was increased without changes in the lesions in the skull and pituitary stalk.

Conclusion

We report a case of disseminated JXG occurring after treatment of LCH. These clinical co-presentations suggested a close relationship between their pathogenesis.

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Acknowledgments

This study was supported by the Basic Science Research Program through the National Research Foundation of Korea (NRF), funded by the Ministry of Science, ICT, & Future Planning (2017R1A1A1A05001020).

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Authors and Affiliations

  1. Department of Neurosurgery, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, 160, Ilsim-ri, Hwasun-eup, Hwasun-gun, Jeollanam-do, 519-809, Republic of Korea

    Tae-Kyu Lee & Tae-Young Jung

  2. Department of Pediatrics, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, 160, Ilsim-ri, Hwasun-eup, Hwasun-gun, Jeollanam-do, 519-809, Republic of Korea

    Hee-Jo Baek

  3. Department of Radiology, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju, Republic of Korea

    Seul-Kee Kim

  4. Department of Pathology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Jeollanam-do, Republic of Korea

    Kyung-Hwa Lee

  5. Department of Dermatology, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, Jeollanam-do, Republic of Korea

    Sook Jung Yun

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  1. Tae-Kyu Lee
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Correspondence to Tae-Young Jung or Hee-Jo Baek.

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Lee, TK., Jung, TY., Baek, HJ. et al. Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report. Childs Nerv Syst 34, 765–770 (2018). https://doi.org/10.1007/s00381-017-3675-8

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  • DOI: https://doi.org/10.1007/s00381-017-3675-8

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