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

PURPOE: To report long-term outcome of patient with necrobiotic xanthogranuloma, to invetigate the propriety of therapeutic urgical exciion or debulking, and to tudy tiue pecimen by immunoperoxidae taining and in itu hybridization. METHOD: Medical record of all patient at the Mayo Clinic, Rocheter, Minneota, with necrobiotic xanthogranuloma between 1980 and 1997 were reviewed. A follow-up letter wa ent to each patient inquiring about the current tatu of the leion, the treatment regimen, and aociated ytemic dieae. REULT: The average age (± tandard deviation) of the 15 men and 11 women wa 56.8 ± 14.8 year. Of the 26 patient, 21 (81%) had leion of the ocular adnexa. Ulceration of the leion occurred in 11 patient (42%). The leion recurred after urgical removal in 11 patient (42%) and on prior inciion ite from unrelated operation in three patient (12%). The average duration of follow-up from the appearance of characteritic kin leion wa 10 ± 6.1 year. Four patient had multiple myeloma, five had a plama cell dycraia, and one had a lymphoproliferative diorder during thi period. Time to development of aociated malignancy ranged from 8 year before the kin leion to 11 year after the kin leion. Overall urvival wa 100% at 10 year and 90% at 15 year (95% confidence limit, 0.73 to 1.00). Immunoperoxidae tain demontrated that mot hitiocyte are not of Langerhan cell lineage. Monoclonal immune globulin were not identified in tiue pecimen. CONCLUION: Care of patient with necrobiotic xanthogranuloma hould include avoidance of urgical removal, if poible, and lifelong follow-up to detect the development of aociated malignancy