Treatment of Severe Disseminated Juvenile Systemic Xanthogranuloma With Multiple Lesions in the Central Nervous System

Abstract

Juvenile xanthogranuloma (JXG), one of the most common forms of Langerhans-dendritic cell proliferation in young children, usually presents as spontaneously regressing cutaneous lesions. JXG with systemic (extracutaneous) involvement is a rare histiocytic disorder in which significant morbidity and death may occur. The systemic type, especially combined with multiple central nervous system lesions in young children, has a very poor prognosis. The patient described here presented with disseminated disease including lungs, liver, kidneys, ribs, scalp, and central nervous system. The patient was treated with multiagent chemotherapy based on the Langerhans cell histiocytosis II treatment protocol. The regimen used included an additional intrathecal therapy with methotrexate and prednisolone to control central nervous system lesions. The patient was treated for 28 months and has been in remission for almost 5 years.

A child with coexistent juvenile xanthogranuloma and Langerhans cell histiocytosis

“…5 As to the systemic type of JXG, chemotherapy based on LCH treatment protocol has been successful. 7,8 In the last decade, there have been several reports 9-11 of JXG developing from LCH. Six children with LCH developed JXG within several months or years after having received systemic or topical therapy.…”

Long-Term Follow-Up of Solitary Intracerebral Juvenile Xanthogranuloma

“…The recurrence rate of skin lesions after excision is very low, even with positive resection margins [37, 11, 12]. In contrast, patients with systemic disease have a much more severe clinical course [38, 36]. Some individuals with systemic or multifocal disease have been treated with adjuvant therapies, including chemotherapy, immunosuppressive therapy and radiation [19,26,27,28, 30, 31, 36, 38].…”

Use of Radiation in Treatment of Central Nervous System Juvenile Xanthogranulomatosis

“…Adjuvant therapies for florid JXG include assorted chemotherapy regimens and conventional radiation. Chemotherapeutic options for JXG are based mainly on therapies currently utilized to treat LCH [3,4,10]. These include, but are not limited to, prednisolone, vinblastine, methotrexate, 6-mercaptopurine, cladribine, and cytarabine.…”

“…A 13-year- [2]) with plans to escalate the dose to 2-CDA plus cytarabine if there was no response. In general, treatment of JXG is based on multiagent LCH therapy [3,4]. The decision to initiate second line therapy was based on the location of disease, concern for possible progression of disease in a short period of time, disease symptoms and expert opinion.…”