Langerhans' cell histiocytosis of the spine - Analysis of twenty-three cases

Study Design. Retrospective review of clinical and radiologic data in four major tertiary referral centers. Objectives. To report clinical and roentgenographic findings, to evaluate t he results of various treatment methods, and to propose a protocol for mana gement. Summary of Background Data. Langerhans' cell histiocytosis of the spine is a rare condition, and therefore, appropriate management is still controvers ial. Methods. Clinical and roentgenographic findings of 38 vertebral lesions of 23 children, with average follow-up of 5.4 years, were investigated. This i s the most extensive report apparent in the literature to dale. The results of treatment were assessed clinically and radiologically. Anterior vertebr al body height was measured sequentially to evaluate reconstitution of the vertebral body. Results. The last follow-up examination demonstrated no clinical evidence o f disease in all patients, regardless of treatment method. Neurologic defic its developed in four patients, but they completely disappeared. Satisfacto ry restoration of height was demonstrated in all except five vertebrae: one that had collapsed maximally when the patient was more than 15 years of ag e and four that had been fused anteriorly or posteriorly. Unsatisfactory re sults were also seen in a patient with progressive scoliosis and in one wit h an irregular endplate with disc space narrowing. Both of these complicati ons developed after curettage. Conclusions. For treatment of single or dual spinal lesions, observation wi th or without bracing seems to be sufficient. In patients with multifocal l esions, chemotherapy produces good results. For treatment of neurologic def icit, low-dose radiotherapy is favored. Patients who underwent surgery-espe cially curettage and anterior fusion- had the worst outcome.