Langerhans Cell Histiocytosis of the Atlas: A Report of Three Cases

Abstract

L angerhans cell histiocytosis sometimes involves the cervical spine and can be associated with collapse of the vertebral body1, but involvement of the atlas is rare. To the best of our knowledge, the cases of only eight patients with Langerhans cell histiocytosis of the atlas have been reported2-8. We report the cases of three patients with Langerhans cell histiocytosis of the atlas, two of whom appeared to have been successfully treated with low-dose chemotherapy. The patients and/or their parents were informed that information concerning the case would be submitted for publication. C ase 1. An eleven-year-old boy had neck pain for two weeks followed by the development of persistent torticollis. The neck pain gradually increased, and the symptoms did not improve after the administration of antipyretic drugs. Two weeks after he began treatment, magnetic resonance imaging showed a destructive process within the lateral mass of C1, and the patient was referred to our hospital. The medical history was otherwise unremarkable. Four weeks after the onset of symptoms, the boy required walking aids because of severe neck pain and persistent torticollis; the neck was bent to the left side and slightly rotated to the right side. Physical examination revealed stiffness, pain upon motion of the cervical spine, and tenderness of the left upper cervical spine. There were no neurological deficits. Blood tests showed a slightly increased C-reactive protein level of 0.5 mg/dL (5 mg/L) (normal, 0.0 to 0.4 mg/dL [0 to 4 mg/L]) and alkaline phosphatase of 414 U/L (normal range for an adult, 90 to 340 U/L), while the white blood-cell counts were within the normal range. Cervical radiographs showed torticollis of the cervical spine, and computed tomography demonstrated an osteolytic lesion in the left lateral mass of the atlas (Fig. 1). Magnetic resonance …