A case of isolated intracranial juvenile xanthogranuloma in a 14‒year‒ old female

Hanae Ito, Yoji Tanaka, Keisuke Ohnaka, Kazuhide Shimizu, Daisuke Kobayashi, Motoki Inaji, Tadashi Nariai, Taketoshi Maehara

Research output: Contribution to journalArticlepeer-review


Juvenile xanthogranuloma(JXG)is a benign cutaneous tumor with a predilection for the head and neck region, that usually appears in children, and central nervous system involvement is rare. Here we present a case of an isolated intracranial JXG. A 14‒year‒old female suffered from transient headache and right visual disorder after intense exercise, and magnetic resonance imaging demonstrated a solid mass with edema at the base of the left occipital lobe. She underwent total removal of the tumor with left occipital craniotomy. The pathological findings were non‒Langerhans cell histiocytic proliferation, and finally diagnosed as JXG based on clinical features. Patients diagnosed with intracranial JXG have a favorable outcome if it is totally removed. However, long‒term prognosis of this disease is still unknown. Moreover, there are some cases of multiple lesions that show more aggressive progression and need adjuvant thera-pies. Therefore, careful follow up is needed for patients with JXG.

Original languageEnglish
Pages (from-to)513-518
Number of pages6
JournalJapanese Journal of Neurosurgery
Issue number7
Publication statusPublished - 2020
Externally publishedYes


  • BRAF mutation
  • Juvenile xanthogranuloma
  • Non‒Langerhans cell histiocytic proliferation


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