Maxillofacial manifestations of Langerhans cell histiocytosis: a clinical and therapeutic analysis of 10 patients.

The definition of Langerhans cell histiocytosis (formerly known as histiocytosis X) includes the clinical syndromes Hand-Schueller-Christian syndrome, Abt-Letterer-Siwe syndrome, and eosinophilic granuloma. The paper gives an overview of current diagnostic and treatment strategies of LCH. Furthermore, records and clinical data of 10 patients with LCH were evaluated retrospectively. Patients' age ranged from 13 years to 42 years. The mandible was more frequently involved than the maxilla. Three patients (30%) had systemic manifestations of LCH in addition to their oral lesions. The longest follow-up period was 12 years. During follow-up six patients (60%) developed recurrent LCH and received adjuvant chemo- or radiation therapy. For solitary bone lesions, surgical curettage is the recommended treatment. Those patients with multi-organ involvement or recurrent LCH should be included into clinical trials initiated by the Histiocyte Society.