Shanghai, China

Author Affiliations: Department of Pediatric Neurosurgery (Sun LP, Jin HM, Yang B); Department of Pathology (Wu XR), Xin Hua Hospital Affiliated to Shanghai Jiaotong University, Shanghai 200092, China

Corresponding Author: Lian-Ping Sun, MD, Department of Pediatric Neurosurgery, Xin Hua Hospital Affiliated to Shanghai Jiaotong University, Shanghai 200092, China (Tel: 86-21-65790000 ext 8285; Email: slp1128@126.com)

Background: Juvenile xanthogranuloma (JXG) is a disorder of histiocyte proliferation. Most cases present with a solitary cutaneous lesion. JXG with systemic involvement is rare with significant morbidity. Intracranial solitary JXG may be misdiagnosed before operation.

Methods: A 5-month-old boy showed an elevated anterior fontanel but no other abnormalities on admission. Brain MRI showed a large mass in the right parietal region.

Results: The tumor was removed with the encroached meninges. A JXG in the right parietal region was diagnosed pathologically.

Conclusion: Total excision of the tumor may be curative with a prerequisite of ensuring normal vital signs and nervous function.

Key words: infant; intracranial; juvenile xanthogranuloma; surgery

                 World J Pediatr 2009;5(1):71-73