Intracranial solitary juvenile xanthogranuloma successfully treated with stereotactic radiosurgery

Abstract

Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis, which primarily affects the skin in young children. Although intracranial involvement is rare, it differs from other systemic JXG because its responsiveness to non-surgical treatment is poor. We present the case of a 2-year-old boy with left abducens nerve palsy. Imaging studies revealed a mass in the left Meckel's cave. Systemic examination showed no other abnormalities including his skin. After partial removal, JXG was diagnosed based on immunohistochemical evaluation. He underwent a stereotactic radiosurgery. Then, the mass decreased, and his abducens nerve palsy improved. This is the seventh case with isolated intracranial JXG, and the first case to be treated with radiosurgery. We consider that stereotactic radiosurgery is an excellent choice for treatment of intracranial JXG.

Spontaneous regression of infantile dural-based non-Langerhans cell histiocytosis after surgery: case report

“…10,19 The immunohistochemical examination is typically positive for CD68, but negative for CD1a and S100 protein. 8,10,11,19 Of the 29 cases, 17 were intracranial, 2,3,5,11,[13][14][15]18,29,31,33,34,36,38,39,42 9 were intraspinal, 6,7,11,17,24,25,32, 35,43 and 3 showed both intracranial and intraspinal lesions. 8,12,41 Only one case was in an adult.…”

“…17 Among the 29 cases, 22 followed a benign course, whereas 7 showed progression of the disease. 8,12,29,31,33,39,41 These 7 cases occurred in Meckel's cave or presented with multiple lesions. Orsey et al 31 reported a JXG diffusely involving the CNS that subsequently underwent malignant transformation and dissemination to the peritoneum and bone marrow.…”

Intracranial solitary juvenile xanthogranuloma in an infant

“…It is rare to see a brain tumor with no cutaneous lesion, and there are 16 cases reported so far. [3][4][5][6][7][8] JXG is a specialized proliferation of nonLangerhans' cells. [9] It is not a real tumor but a course of reactive proliferation of histiocytes caused by some stimulants such as viral infection and physical factors and often be confused with Langerhan's cells hyperplasia (LCH) clinically.…”

Intra-abdominal Xanthogranulomatoses Secondary to Postoperative Bile Leak

“…Xanthogranuloma is one of the varaints of non-Langerhan cell histocytoses [3]. It is a benign tumor histologically characterized by foamy histiocytes, numerous Touton giant cells and rare eosinophils [4].…”