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

PURPOSE: To report long-term outcomes of patients with necrobiotic xanthogr anuloma, to investigate the propriety of therapeutic surgical excision or d ebulking, and to study tissue specimens by immunoperoxidase staining and in situ hybridization. METHODS: Medical records of all patients at the Mayo Clinic, Rochester, Min nesota, with necrobiotic xanthogranuloma between 1980 and 1997 were reviewe d. A follow up letter was sent to each patient inquiring about the current status of the lesions, the treatment regimen, and associated systemic disea ses, RESULTS: The average age (+/- standard deviation) of the 15 men and 11 wome n 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 avera ge duration of follow up from the appearance of characteristic skin lesions was 10 +/- 6.1 years. Four patients had multiple myeloma, five had a plasm a cell dyscrasia, and one had a lymphoproliferative disorder during this pe riod. Time to development of associated malignancy ranged from 8 years befo re the skin lesions to 11 years after the skin lesions. Overall survival wa s 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 Lan gerhans cell lineage. Monoclonal immune globulins were not identified in ti ssue specimens. CONCLUSION: Care of patients with necrobiotic xanthogranuloma should includ e avoidance of surgical removal, if possible, and lifelong follow up to det ect the development of associated malignancy, (C) 2000 by Elsevier Science Inc. All rights reserved.