Management of Spinal Langerhans Cell Histiocytosis in Children: A Systematic Review

Spinal Langerhans cell histiocytosis can manifest as solitary site unifocal form or as systemic form in children. The management options for solitary spinal site unifocal form are many. They include spontaneous resolution of the lesion and supervised treatment, steroid injection of the lesion, systemic chemotherapy, radiation therapy and surgery. Multiple options create a decision-making dilemma for the treating specialist. The authors sought to formulate a management algorithm of spinal Langerhans cell histiocytosis based on Garg's grading of radiographic vertebral body collapse.

The Preferred Reporting Items for Systematic Reviews and Meta-Analyses-2020 guidelines were followed in conducting the review and studies were filtered from established medical databases. Articles published between 2003 and 2022 were included after applying strict inclusion and exclusion criteria. The first and second authors reviewed the abstracts of filtered studies before including them. The study was registered with Prospero. The bias assessment of included studies was assessed using the MINOR's criteria.

Eight retrospective case series were analyzed. Within these studies, a total of 116 children (mean age 7.4 years) had undergone treatment. The mean follow-up period was 52.1 months. Among these patients, there were 37 tumors in the cervical spine, 40 in the thoracic spine, 25 in the lumbar spine, and a single tumor in the sacrum. Systemic chemotherapy has been found to reduce the risk of radiographic vertebral body collapse (p < 0.05). Surgery provides optimal outcomes in patients with Garg's grade IB , II spinal tumors and restores vertebral body height (p < 0.05). No case series were found pertaining to grade III. Reconstitution of vertebral body height, an important radiological parameter indicating the endpoint or healing of the lesion, was early achieved with surgery followed by systemic chemotherapy, bracing, and supervised management.

Observation can be preferred in Garg's grade IA. Grade IB and II tumors respond well to surgery. Treatment for grade III tumors needs to be tailored on an individual basis.

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