Langerhans' Cell Histiocytosis (Histiocytosis X) of the Temporal Bone
NONG Dong-xiao
NONG Hui-tu
TANG An-zhou
CHEN Ruo-ze
摘要：Objectives Langerhans' Cell histiocytosis (LCH) is a rare disease, which remains poorly understood and whose cellular origin remains unknown. To increase understanding of temporal bone LCH, it is necessary to study recent advances in the diagnosis and treatment of this disease. Methods The long term(5 to 30 years) results of 21 temporal bone LCH cases treated between 1973 and 2003 were reviewed. Surgery, radiotherapy,pharmacologic therapy or a combination of these treatments were employed in these cases. Results Eighteen patients were cured (18/21, 85%). Six patients developed residual diabetes insipidus (DI) and dwarfism (28%).Three patients died (14%). Conclusions The Alessi classification system for LCH based on the extent of disease accurately predicts prognosis and is a useful guide in selecting treatment methodologies. X-ray, computed tomography and magnetic resonance imaging have proved useful in defining the extent of osseous and soft tissue diseases. Diagnosis of LCH is based on clinical presentations, radiographic findings and histopathological results.Surgery and radiotherapy are the main treatment modalities. Pharmacologic therapy should be used in patients with aggressive, disseminate, and refractory lesions. LCH has a predilection for children and prognosis depends on age and extent of vital organ involvement.
机标关键词：temporal bonemagnetic resonance imagingdiagnosis and treatmentclassification systemdiabetes insipidussoft tissueused in
分类号：R76(耳鼻咽喉科学)
论文发表日期：2007-01-01
在线出版日期：2025-08-15（本平台首次上网日期，不代表文献的发表时间）
页数：6( 36-41 )
英文信息