Open AccessSystemic juvenile xanthogranuloma with multiple central nervous system lesions
Author(s) -
Ali Meshkini,
Sohrab Shahzadi,
Alireza Zali,
Aram Tajeddini,
Javad Mirzayan,
Amir Hamdi
Publication year - 2012
Publication title -
journal of cancer research and therapeutics/journal of cancer research and therapeutics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.475
H-Index - 39
eISSN - 0973-1482
pISSN - 1998-4138
DOI - 10.4103/0973-1482.99001
Subject(s) - juvenile xanthogranuloma , medicine , prednisolone , central nervous system , skin biopsy , methotrexate , pathology , biopsy , histiocyte , stereotactic biopsy , dermatology , vinblastine , brain biopsy , chemotherapy , surgery
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.