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Published on: September 18, 2011
Langerhans cell histiocytosis with multiple spinal involvement.
Liang Jiang1, Xiao Guang Liu, Wo Quan Zhong
1Orthopaedic Department, Peking University Third Hospital, No 49 North Garden Street, HaiDian District, Beijing 100191, China.
Summary
Multiple spinal Langerhans cell histiocytosis (LCH) presents with varied symptoms and outcomes. Observation or chemotherapy may be used, with surgery reserved for severe neurological compromise.
Area of Science:
- Oncology
- Pediatric Oncology
- Radiology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease.
- Spinal involvement in LCH can lead to significant morbidity.
Purpose of the Study:
- To detail the clinical and radiologic presentation of spinal LCH.
- To analyze treatment outcomes for spinal LCH, particularly multifocal presentations.
Main Methods:
- Retrospective review of 42 spinal LCH cases from a single institution.
- Meta-analysis of 50 additional cases from literature, focusing on multifocal spinal LCH.
- Analysis of age, sex, clinical presentation, radiologic findings, therapy, and outcomes.
Main Results:
- Multifocal spinal LCH occurred in 5 of 42 reviewed cases and 27.2% of compiled literature cases.
- Common sites included thoracic (47.8%) and cervical (28.0%) spine.
- Extraspinal involvement (54.2%) and vertebra plana (50%) were frequent; neurological symptoms occurred in 3 of 5 local cases.
- Treatment varied; one death and two recurrences were noted over a mean 7.2-year follow-up.
Conclusions:
- Asymptomatic spinal LCH may be managed with observation or bracing.
- Chemotherapy is indicated for multifocal spinal LCH.
- Surgical decompression is reserved for refractory or rapidly progressing neurological compromise.
