Langerhans Cell Histiocytosis of the Spine in Children

Purpose: Vertebral destruction in Langerhans cell histiocytosis (LCH) of the spine may lead to the development of spinal deformity, especially in the growing pediatric patient. This study examines the presentation, natural history, and long-term outcomes in children with LCH of the spine in terms of both spinal alignment and disease-free survival.

Methods: 26 children with biopsy-proven LCH involving the spine have been treated at our institution since 1972. Clinical charts and radiographs were retrospectively reviewed for disease presentation, extent and morphology of vertebral collapse, treatment, and condition at follow-up. Vertebral body collapse was measured on radiographs and classified as grade I (0-50% collapse) or grade II (50-100% collapse) and sub-classified as A (symmetric collapse) or B (asymmetric collapse). Three patients were diagnosed within the past two years and were excluded from the follow-up analysis.

Results: 14 boys and 12 girls ranging in age from 0.2-16.4 years (mean 8.2) had 44 involved vertebrae (20 cervical, 14 thoracic, 10 lumbar). Long-term follow-up was available for 23 patients (41 vertebrae) ranging from 2- 22.7 years (mean 9.3 years). Lesions showed a predilection for the cervical spine ( <0 .02) and a relative paucity of lesions involved the thoracic spine ( < 0.006). Eleven children were found to have multi-focal skeletal disease after routine use survey or bone scan following initial diagnosis. Cervical lumbar showed significant association with multi-level spinal 0.03 0.04 respectively). Only two patients had systemic disease. Local pain was presenting symptom in all children. three presented neurologic symptoms (radicular pain), none who deficits. Initial radiographic collapse grade IA 20 children, IB 3, IIA 10, IIB 9. Two located atypically posterior elements not classified. As opposed II lesions, I much more likely create symmetric 0.03). Ten received chemotherapy, four radiation therapy, both. Three developed deformity, (both IIA) later fusion. The other (grade IIB) stable mild decrease lordosis. (one IIA, one posterior) (<5mm) C3 on C4 anterior subluxation that spontaneously resolved vertebral reconstitution. Despite heterogeneous treatment, are alive well no evidence including resolution at follow-up.

Conclusions: This is the largest reported case series of LCH of the spine to date. Although severe (grade II) lesions are more likely to be asymmetric, they do not appear to predispose children to later spinal deformity. The natural history of LCH of the spine in the absence of systemic disease or spinal deformity is such that aggressive surgical management is not indicated and only follow-up to monitor recovery and spinal balance is necessary. Chemotherapy may be considered in patients with multiple sites of disease; radiation therapy, however, is no longer used due to increased risk of secondary sarcoma.