Juvenile Xanthogranuloma


Diagnosis and Treatment

Juvenile xanthogranuloma is the disease most commonly confused with LCH. Presenting symptoms may be similar, so a biopsy is necessary to tell the difference between the two diseases.

When a biopsy is performed, a small piece of the tissue is obtained so that it can be viewed under a microscope by a pathologist. If the cells in the tissue have certain characteristics, the diagnosis of JXG can be made. JXG cells are negative for the presence of proteins called S-100 and CD1a, while LCH cells are positive. Instead JXG cells are positive for proteins called CD68 and factor XIIIa. A type of cell called a Touton giant cell is present in 85% of JXG cases. This is a histiocyte filled with fat that has a specific way of clustering that helps make the diagnosis.

An ultrasound may be performed to see if the JXG is also in the abdominal organs such as liver or spleen. In this procedure, high-energy sound waves are bounced off organs and tissue and make echoes, which form a picture of the internal body. In addition depending on the symptoms other tests such as CAT scans or MRI scans or bone X-rays may be done.

Treatment varies according to the extent and severity of disease involvement. Observation only is often a reasonable approach. Patients with a single lesion or just a few lesions usually need no therapy. Surgical removal may be undertaken for several reasons: to obtain a biopsy for diagnosis, when there is an organ-function problem because of disease, for cosmetic reasons or to remove scar tissue. Apart from these reasons, skin-only JXG in children should be observed without therapy. For the small percentage of patients who have symptomatic or rapidly growing disease, treatment with chemotherapy or low-dose radiation has been reported, although there is no standard treatment that is agreed upon. With eye involvement, steroids may be applied to the surface of a lesion, injected within the lesion, or taken in pill form. Rarely low dose radiation treatment may be given to the eye to prevent visual loss.

JXG in adults tends to be more complicated and is not known to improve spontaneously without treatment. However, most cases of childhood JXG are self-limited and resolve without treatment. Adult JXG does not tend to resolve spontaneously and usually requires some form of therapy. This may involve surgical removal alone or additional therapy depending on the clinical situation.

 

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