How is juvenile xanthogranuloma (JXG) treated?

Updated: Feb 08, 2019
  • Author: Bhupendra C K Patel, MD, FRCS; Chief Editor: Michael Taravella, MD  more...
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Pharmacotherapy: Topical, subconjunctival, intralesional, and systemic corticosteroids are used for intraocular and orbital juvenile xanthogranuloma (JXG). Orbital lesions may respond to intralesional steroid injections. Iris lesions are treated with topical prednisolone or subconjunctival steroids. These are followed by sub-Tenon steroids if no improvement occurs in several weeks.

The first-line treatment of iris JXG is high-dose corticosteroids applied topically (every two hours during waking hours and ointment at night) with a slow taper. If there is difficulty administering topical medication, periocular steroids can be considered, although the effects of the steroids may last for 3-4 months.

Systemic steroids are considered only if the topical steroids do not yield an adequate response.

Radiotherapy: Low-dose radiation may be the treatment of choice for diffuse uveal lesions, especially if glaucoma is present, or if there is poor response to steroid treatment. Typically, 100-200 cGy is administered per dose over a 2- to 3-week period. The total is usually kept under 500 cGy, but higher doses are used for poorly responsive tumors.

Antimetabolites are sometimes used as adjuvants to radiotherapy.

Appropriate glaucoma medications should be used in the setting of hyphema and increased intraocular pressure.

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