Langerhans' cell histiocytosis of the head and neck

Langerhans' cell histiocytosis has been accepted by many to replace the term Histiocytosis X, describing a poorly defined continuum of diseases involving neoplastic proliferation of histiocytes. Twenty-four cases of histologically confirmed Langerhans' cell histiocytosis with head and neck involvement were seen between the years 1970 to 1986, and charts were reviewed retrospectively. Control of local osseous disease was successful using radiation therapy in 100% of those cases with follow-up. Surgical curettage of bone lesions was successful in a small number of cases. Chemotherapy alone or in combination with radiation appeared to enhance survival in patients with recurrent disease or multisystem involvement. Sixteen of the 24 patients (67%) are disease free with a mean follow-up of 6 years. Prompt diagnosis and careful follow-up are important to improve survival and prevent complications.