Multifocal Langerhans Cell Histiocytosis Involving Bilateral Temporal Bones Lungs and Hypothalamus in an Adult

摘要

Multifocal Langerhans' cell histocytosis is a rare condition in adults. We present the case of a 31-year-old female who initially presented with a clinical appearance of acute mastoiditis. The patient ultimately had involvement of bilateral temporal bones, lungs, and hypothalamus. Treatment with methotrexate, steroids, and desmopressin acetate (DDAVP) resulted in initial clinical improvement though not resolution. The patient also underwent radiation therapy to the hypothalamic lesion. The literature is reviewed, focusing on the diagnostic challenge of this disease process in adults. The additional morbidity of surgery, specifically mastoidectomy, in the setting of Langerhan's cell histiocytosis is discussed.