Clinical features and treatment outcomes of Langerhans cell histiocytosis of the spine.

Background Context: Langerhans cell histiocytosis (LCH) of the spine is a relatively rare condition with unknown etiology. The diagnosis and treatment protocols for spine LCH remain controversial.

Purpose: In this study, we evaluated the efficacy and safety of our proposed diagnosis and treatment protocol introduced in 2009.

Study Design: This is a retrospective study.

Patient Sample: A total of 110 patients with spine LCH who had been diagnosed and treated in our hospital from October 1997 to November 2015 were included in this study.

Outcome Measures: The age, gender, symptoms, neurologic function, lesion distribution, radiological features, pathology, treatment, outcome, and treatment complications of the patients were collected. Visual analog scale (VAS) for pain and Frankel scale for neurologic status were also documented.

Methods: We retrospectively reviewed 110 patients with spine LCH who had been diagnosed and treated in our hospital from October 1997 to November 2015. The indications for computed tomography (CT)-guided biopsy and surgery for spine LCH have become more stringent since 2009. In cases of a solitary spinal lesion, immobilization and observation were usually first suggested. Chemotherapy was suggested for cases with multifocal LCH lesions, and low-dosage radiotherapy was restricted to recurrent solitary lesion. This project was supported by our hospital (No. Y71508-01) (¥ 400,000).

Result: This series included 69 male and 41 female patients (age range, 1-52 years). Pain was the most common symptom (93.6%, 103/110). Pathologic diagnosis was achieved in 72 cases (65.5%). CT-guided biopsies were performed in 91.3% (42/46) and 73.2% (41/56) of cases before and after 2009, respectively (p=.02). Ninety-eight cases (89.1%) were followed up for a mean 66.3 (range, 24-159) months. Immobilization and observation were performed in 25.9% (14/54) and 75.0% (42/56) of cases before and after 2009, respectively (p<.001). Approximately 35.2% (19/54) and 10.7% (6/56) of cases had surgery (p=.002) before and after 2009, respectively. During the follow-up, no significant difference was found in the outcomes between the two groups treated before and after 2009 (p=.64).

Conclusion: Biopsy is not mandatory for typical spine lesions of LCH. Given the self-healing tendency of spine LCH, immobilization and observation remain the first-choice treatments for LCH lesions. Conservative biopsy and treatment protocols might be more appropriate for spinal LCH.