Solitary juvenile xanthogranuloma mimicking intracranial tumor in children

Idit Tamir, Rina Davir, Yakov Fellig, Michael Weintraub, Shlomo Constantini, Sergey Spektor*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Juvenile xanthogranuloma (JXG) is primarily a benign cutaneous disorder of non-Langerhans hystiocytic proliferation. Systemic involvement occurs in 4% of patients; isolated central nervous system (CNS) lesions are rare. We report solitary CNS-JXG lesions in two patients. A 3.5-year-old boy with a parietal-occipital lesion underwent total resection with no surgical morbidity and no recurrence at 16-month follow-up. A 3.5-year-old girl underwent subtotal resection of a tumor extending from the left Meckel's cave and invading the cavernous sinus and left orbit with extensive cranial nerve involvement. Tumor regrowth with leptomeningeal spread at 9-month and 12-month follow-up was managed with steroids and chemotherapy (vinblastine and later cladribine). We present our experience and review the literature pertaining to rare reports of solitary CNS-JXG.

Original languageEnglish
Pages (from-to)183-188
Number of pages6
JournalJournal of Clinical Neuroscience
Volume20
Issue number1
DOIs
StatePublished - Jan 2013
Externally publishedYes

Keywords

  • CNS xanthogranuloma
  • Histiocytosis
  • Meckel's cave
  • Xanthogranuloma

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