Oral sirolimus for the treatment of juvenile xanthogranuloma: Report of two pediatric cases.
histiocytic disorders
juvenile xanthogranuloma (JXG)
mammalian target of rapamycin (mTOR)
pediatric dermatology
sirolimus
Journal
Pediatric dermatology
ISSN: 1525-1470
Titre abrégé: Pediatr Dermatol
Pays: United States
ID NLM: 8406799
Informations de publication
Date de publication:
05 Mar 2024
05 Mar 2024
Historique:
received:
14
11
2023
accepted:
16
02
2024
medline:
6
3
2024
pubmed:
6
3
2024
entrez:
6
3
2024
Statut:
aheadofprint
Résumé
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.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2024 Wiley Periodicals LLC.
Références
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