Multifocal Langerhans' cell histiocytosis involving bilateral temporal bones, lungs, and hypothalamus in an adult

Multifocal langerhans' cell histiocytosis is a rare condition in adults. We present the case of a 31-year-old female who initially presented with a cl inical appearance of acute mastoiditis. The patient ultimately had involvem ent of bilateral temporal bones, lungs, and hypothalamus. Treatment with me thotrexate, steroids, and desmopressin acetate (DDAVP) resulted in initial clinical improvement though not resolution. The patient also underwent radi ation therapy to the hypothalamic lesion. The literature is reviewed, focus ing on the diagnostic challenge of this disease process in adults. The addi tional morbidity of surgery, specifically mastoidectomy, in the setting of Langerhan's cell histiocytosis is discussed.