Successful Treatment of Congenital Systemic Juvenile Xanthogranuloma With Langerhans Cell Histiocytosis???Based Chemotherapy

Abstract

Juvenile xanthogranuloma (JXG), one of the most common forms of non-Langerhans cell histiocytosis (LCH), usually presents in young children as spontaneously regressing cutaneous lesions. However, the systemic type of JXG is difficult to treat in newborn infants, and fatal cases have been reported. In the patient described here, solid masses were discovered by fetal sonography during the 38th gestational week. At birth she had multiple tumors on the back, cheek, and hip as well as marked hepatosplenomegaly accompanied by respiratory failure. Laboratory results indicated pancytopenia, obstructive liver dysfunction, and coagulopathy. Brain magnetic resonance imaging revealed a tumor at the left pontine angle, and dysmorphic histiocytes were present in her spinal fluid. She was diagnosed with systemic JXG by histopathologic findings of the hip mass. The LCH-based multiagent chemotherapy including cytarabine, vincristine, methotrexate, and prednisolone ameliorated the symptoms rapidly. She was treated for 12 months and is currently doing well as a normally developing 2-year-old.

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.…”

Imaging Features of Juvenile Xanthogranuloma of the Pediatric Head and Neck

“…undefined; however, treatment regimens similar to those used for LCH have been used successfully. [27][28][29] …”

Intracranial solitary juvenile xanthogranuloma in an infant

“…With regard to a single tumor, it is necessary to resect the tumor totally to save the patient or to release the compression to nerves. If the tumor locates at the surgically inaccessible location or there are multicentric lesions, the patient will still have a eusemia with stereotactic radiosurgery [10] or the therapeutic alliance [12] in time with cytarabine, vincristine, MTX, prednisolone (or hydrocortisone).…”