Langerhans cell histiocytosis of the atlas in an adult.
Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a rare disorder (approximately 1:1,500,000 inhabitants) characterized by clonal proliferation and excess accumulation of pathologic Langerhans cells causing local or systemic effects. The exact etiology of LCH is still unknown. LCH could affect patients of any age, although most present when they are children. The most frequent sites of the bony lesions are the skull, femur, mandible, pelvis and spine. A variety of treatment modalities has been reported, but there was no evidence suggesting that any one treatment was more advantageous than another. We present an adult with LCH of the atlas. A 26-year-old young man presented with a 2-month history of neck pain and stiffness. CT revealed osteolytic lesion in the left lateral mass of atlas with compression fracture. Histopathological diagnosis was Langerhans cell histiocytosis by percutaneous needle biopsy under CT guidance. The patient underwent conservative treatment, including Halo-vest immobilization and radiotherapy. At 7-year follow-up, the patient was asymptomatic except for mild motion restriction of the neck. CT revealed a significant reconstruction of the C1 lateral mass.
Zhong
Wo Quan
WQ
Orthopaedic Department, Peking University Third Hospital, No 49, North Garden Street, HaiDian District, 100191, Beijing, China. zhong811@yeah.net
Jiang
Liang
L
Ma
Qing Jun
QJ
Liu
Zhong Jun
ZJ
Liu
Xiao Guang
XG
Wei
Feng
F
Yuan
Hui Shu
HS
Dang
Geng Ting
GT
D000328:Adult; D001270:Cervical Atlas / Q000000981:diagnostic imaging* / Q000473:pathology*; D016267:External Fixators; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007103:Immobilization / Q000295:instrumentation / Q000379:methods; D008297:Male; D019547:Neck Pain / Q000209:etiology; D011878:Radiotherapy; D013122:Spinal Diseases / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D016103:Spinal Fractures / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome