Treatment and outcome of vertebral Langerhans cell histiocytosis at the Children's Hospital of Eastern Ontario.

BACKGROUND

We wished to evaluate the treatment methods for vertebral Langerhans cell histiocytosis (LCH) (a rare reticuloendothelial disorder) at a tertiary care pediatric centre and compare treatment and outcomes with those reported in the recent literature.

METHODS

A total of 55 charts were retrieved between 1980 and 2003 for children with LCH. Only those children who were under 18 years of age, had a diagnosis of LCH, histiocytosis X or eosinophilic granuloma and had documented vertebral involvement were included. The data collected were compared with data in the literature with respect to epidemiologic features, symptoms, investigations and procedures done, treatment, outcome and follow-up.

RESULTS

Of the 8 children who met the inclusion criteria for vertebral LCH, the most common presenting complaint was back or neck pain. The thoracic vertebrae were most commonly affected followed equally by cervical and lumbar spines. Most children underwent a complete diagnostic work-up. A single solitary lesion was found in only 1 child. Biopsies were attempted in all cases with 6 positive results. Treatment varied depending on the severity of the presenting complaint; however, none of the tumours was completely resected. Follow-up averaged 3.4 years, and only 1 child has had a recurrence.

CONCLUSION

A multidisciplinary investigation is recommended for children with suspected vertebral LCH. Treatment depends on the severity of the disease.