Purpose: To describe the systemic treatment of recurrent eosinophilic granuloma.
Methods: Case report and review of Langerhans cell histiocytosis.
Results: A 9-year-old boy had painful, right upper eyelid swelling. A computed tomography scan showed a right superolateral orbital mass with evidence of bony erosion. Frozen section evaluation was consistent with eosinophilic granuloma. Biopsy and curettage were performed. Systemic work-up was negative for extraorbital disease. Recurrence of the orbital mass 6 weeks after surgery was confirmed by magnetic resonance imaging (MRI). Four weeks after treatment with prednisone and vinblastine, MRI showed a marked decrease in the size of the orbital lesion. Additional treatment with 6-mercaptopurine and methotrexate was initiated, and MRI 6 months later showed complete resolution of the lesion.
Conclusions: The treatment of recurrent eosinophilic granuloma is challenging. The goal is to effectively cure the lesion with minimal short-term and long-term side effects. Systemic steroids, vinca alkaloids, antimetabolites, and antifols are preferred because they have less toxicity and mutagenicity. Combination chemotherapy used in conjunction with treatment by a pediatric hematologist/oncologist appears to be an effective method of treating recurrent disease.