Langerhans cell histiocytosis of the spine in adults: A rare and controversial disorder: A systematic review

Introduction

Langerhans cell histiocytosis (LCH) involving the spine in adults is rare. If left untreated, LCH lesions can results in progressive neurological deficits and structural instability. While much has been reported on LCH in children, the pathology, treatment and outcomes in adults is less well understood. We present a comprehensive literature review of all cases of adult-onset spinal LCH reported to date.

Objective

To present the outcomes and clinical experience in the management of spinal LCH in the adult population.

Methods

A systematic review was performed by searching online databases to analyze all reported cases of adult-onset spinal LCH.

Results

Forty-seven patients were identified and analyzed. The male ratio was 63.82 %. The mean age at presentation was 34.70 (range; 20–69) years. Local pain, radiating pain, and restricted movement were the most frequent symptoms. The lumbar vertebrae were the most frequently involved. The vertebral body was involved in 53.19 % of the patients, and only three had vertebral plana morphology. Spinal lesions involved a single vertebra in 42 patients (89.36 %), whereas 5 patients (10.63 %) had multiple vertebral involvement. Lytic destruction of the vertebra is the most notable radiologic feature. Twenty-nine patients underwent surgery, including anterior, posterior, combined decompression, and other approahces. The mean follow-up duration was 22 months (range; 1–120 months). The outcome was good to excellent in all except seven, which reported recurrence or progression.

Conclusion

Surgical intervention seemed to help adult patients showing neurological problems or spinal instability. Non-operative treatment, though common in children, was linked to disease progression/recurrence, underscoring the need for customized treatment plans.