Langerhans cell histiocytosis with multiple spinal involvement

Abstract

To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four soft tissue involvement and three had posterior arch extension. Compiling data from the eight largest case series of the spinal LCH reveals that 27.2% multiple vertebrae lesions. In these 55 cases, there were 26 female and 29 male with the mean age of 7.4 years (range 0.2-37). A total of 182 vertebrae were involved including 28.0% in the cervical spine, 47.8% in thoracic and 24.2% in the lumbar spine. Extraspinal LCH lesion was documented in 54.2% cases, visceral involvement in 31.1% and vertebra plana in 50% cases. Paravertebral and epidural extension were not documented in most cases. Pathological diagnosis was achieved in 47 cases including 8 open spine biopsy. The treatment strategy varied depending on different hospitals. One patient died, two had recurrence and the others had no evidence of the disease with an average of 7.2 years (range 1-21) of follow-up. Asymptomatic spinal lesions could be simply observed with or without bracing and chemotherapy is justified for multiple lesions. Surgical decompression should be reserved for the uncommon cases in which neurologic compromise does not respond to radiotherapy or progresses too rapidly for radiotherapy.

Fig. 1

Radiography showed T11 vertebral plana and irregular T10 with slight regional kyphosis (a, b). MRI revealed that the lesion extended to the right neural foramen of T10 (c, d). Axial CT manifested that the osteolytic lesion extended from vertebral body to the lamina of T10 (e, f). At the 8 (g) and 57-month follow-up (h–k), the lytic lesion healed and the shape of collapsed vertebral bodies did not change with slight regional kyphosis

Fig. 2

CT and MRI manifested C4–6 vertebral body bony destruction with paravertebral and epidural extension (a–e). Lateral radiography and CT reconstruction showed solid fusion with slight kyphosis at the 61-month follow-up (f, g)

Fig. 3

A 24-year-old man presented with a 12-year history of thoracic LCH and laminectomy. Radiography showed thoracic kyphosis (a, b). The lesion involved T1–7 and T10 vertebrae (c–i) with paravertebral and bilateral ribs extension (thick arrow)

Fig. 4

Proposed protocol for the management of LCH with multifocal spinal lesions. Asterisk indicates typical presentation means for multifocal lesions with vertebra plana without posterior arch, epidural and paravertebral extension