Langerhans cell histiocytosis of the spine in children. Long-term follow-up.

BACKGROUND: Langerhans cell histiocytosis causes destructive lesions in a child's spine. Few large, long-term studies have evaluated the clinical and radiographic presentation, natural history, outcomes of modern treatment approaches, and maintenance of normal spinal growth and stability after the diagnosis of this disease in children. METHODS: Twenty-six children with biopsy-proven Langerhans cell histiocytosis involving the spine were treated at our institution between 1970 and 2003. They had a total of forty-four involved vertebrae (twenty cervical, fourteen thoracic, and ten lumbar). Vertebral body collapse was measured on radiographs and classified as grade I (0% to 50% collapse) or grade II (51% to 100% collapse) and subclassified as A (symmetric collapse) or B (asymmetric collapse). Lesions of the posterior elements of the spine were classified as grade III. Twenty-three children were followed for two years or more (mean, 9.4 years), and the analyses of treatment and long-term outcomes were performed in that group of patients. RESULTS: There was a predominance of lesions in the cervical spine (p