AI Article Synopsis
- * Sirolimus, a medication that inhibits the mTOR pathway, has shown promise as an effective oral treatment for various cancers and immune-related disorders.
- * This report highlights two pediatric cases where oral sirolimus effectively treated JXG, suggesting it could provide quick disease resolution and long-term remission, prompting further research into its potential benefits for this condition.
Article Abstract
Juvenile xanthogranuloma (JXG) with extensive cutaneous or visceral organ involvement is often associated with high morbidity and treatment commonly involves surgical excision, radiotherapy, systemic steroids, or chemotherapy. Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, is an oral antitumor and immunosuppressive therapy used to treat various neoplastic disorders, including histiocytic disorders. We report two pediatric cases of JXG successfully treated with oral sirolimus monotherapy, and postulate that sirolimus may induce rapid disease resolution and long-term remission for patients with both skin-limited and multisystemic JXG. Our findings warrant further investigation of the relationship between the mTOR pathway and JXG.
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| http://dx.doi.org/10.1111/pde.15579 | DOI Listing |
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