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 unknow...

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Bibliographic Details
Published in:European spine journal 2010-01, Vol.19 (1), p.19-22
Main Authors: Zhong, Wo Quan, Jiang, Liang, Ma, Qing Jun, Liu, Zhong Jun, Liu, Xiao Guang, Wei, Feng, Yuan, Hui Shu, Dang, Geng Ting
Format: Article
Language:English
Subjects:
Adult
Cervical Atlas - diagnostic imaging
Cervical Atlas - pathology
External Fixators
Grand Rounds
Histiocytosis, Langerhans-Cell - diagnosis
Histiocytosis, Langerhans-Cell - physiopathology
Histiocytosis, Langerhans-Cell - therapy
Humans
Immobilization - instrumentation
Immobilization - methods
Male
Medicine
Medicine & Public Health
Neck Pain - etiology
Neurosurgery
Radiotherapy
Spinal Diseases - diagnosis
Spinal Diseases - physiopathology
Spinal Diseases - therapy
Spinal Fractures - diagnostic imaging
Spinal Fractures - etiology
Spinal Fractures - pathology
Surgical Orthopedics
Tomography, X-Ray Computed
Treatment Outcome
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Summary: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.
ISSN:0940-6719
1432-0932
DOI:10.1007/s00586-009-1172-9