Two Cases of Langerhans Cell Histiocytosis of the Temporal Bone

The most well-known childhood histiocytosis, previously known as histiocytosis X, is Langerhans cell histiocytosis and includes the clinical entities of eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease. The hallmark of this disease is the presence of a clonal proliferation of cells of the monocyte lineage containing the characteristic electron microscopic findings of a Langerhans cell. The skeleton is involved in 80% of patients and bone lesions may be single or numerous and are seen most commonly in the skull, especially in the temporal bone. Prognosis is relatively favorable. We have experienced two cases of Langerhans cell histiocytosis with temporal bone involvement which were treated surgically in two children. (J Clinical Otolaryngol 2005;16:132-135)