Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement

PURPOSE: To report long-term outcomes of patients with necrobiotic xanthogranuloma, to investigate the propriety of therapeutic surgical excision or debulking, and to study tissue specimens by immunoperoxidase staining and in situ hybridization. METHODS: Medical records of all patients at the Mayo Clinic, Rochester, Minnesota, with necrobiotic xanthogranuloma between 1980 and 1997 were reviewed. A follow-up letter was sent to each patient inquiring about the current status of the lesions, the treatment regimen, and associated systemic diseases. RESULTS: The average age (± standard deviation) of the 15 men and 11 women was 56.8 ± 14.8 years. Of the 26 patients, 21 (81%) had lesions of the ocular adnexa. Ulceration of the lesions occurred in 11 patients (42%). The lesions recurred after surgical removal in 11 patients (42%) and on prior incision sites from unrelated operations in three patients (12%). The average duration of follow-up from the appearance of characteristic skin lesions was 10 ± 6.1 years. Four patients had multiple myeloma, five had a plasma cell dyscrasia, and one had a lymphoproliferative disorder during this period. Time to development of associated malignancy ranged from 8 years before the skin lesions to 11 years after the skin lesions. Overall survival was 100% at 10 years and 90% at 15 years (95% confidence limit, 0.73 to 1.00). Immunoperoxidase stains demonstrated that most histiocytes are not of Langerhans cell lineage. Monoclonal immune globulins were not identified in tissue specimens. CONCLUSION: Care of patients with necrobiotic xanthogranuloma should include avoidance of surgical removal, if possible, and lifelong follow-up to detect the development of associated malignancy.