Systemic juvenile xanthogranuloma with multiple central nervous system lesions

J Cancer Res Ther. 2012 Apr-Jun;8(2):311-3. doi: 10.4103/0973-1482.99001.

Abstract

Juvenile xanthogranulomatosis (JXG) is an uncommon histiocytic disorder that is usually benign and limited to the skin. The systemic form of JXG is rare and may be associated with severe morbidity and mortality especially in central nervous system (CNS) involvement. Here, we describe a six-year-old boy with disseminated skin lesions and neurological signs and symptoms. Diagnostic work up revealed multiple brain lesions. A skin biopsy and a stereotactic brain biopsy considered suggestive of systemic JXG. Treatment with prednisolone, vinblastine and methotrexate was successful with regression of skin and CNS lesions. The patient has been in remission for almost three years.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Brain Diseases / diagnostic imaging*
  • Brain Diseases / drug therapy
  • Brain Diseases / pathology
  • Child
  • Drug Therapy, Combination
  • Humans
  • Male
  • Methotrexate / therapeutic use
  • Prednisolone / therapeutic use
  • Radiography
  • Treatment Outcome
  • Vinblastine / therapeutic use
  • Xanthogranuloma, Juvenile / diagnostic imaging*
  • Xanthogranuloma, Juvenile / drug therapy
  • Xanthogranuloma, Juvenile / pathology

Substances

  • Antineoplastic Agents
  • Vinblastine
  • Prednisolone
  • Methotrexate