Langerhans cell histiocytosis of the atlas in an adult

Abstract

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.

Primary Eosinophilic Granuloma of Adult Cervical Spine Presenting as a Radiculomyelopathy

“…If the symptoms are relatively light, as many authors stated, close observation with frequent neurological examination can be chosen as the first proper treatment after histopathological diagnosis, confirmed by open biopsy or needle biopsy2,3,10,23). Jiang et al10) strongly recommended using CT guided-needle biopsy for the confirmative diagnostic method.…”

“…Jiang et al10) strongly recommended using CT guided-needle biopsy for the confirmative diagnostic method. For patients with mild neurological symptoms, most of the authors stated that cervical immobilization is sufficient2,7,10,22,23).…”

Percutaneous vertebroplasty for Langerhans cell histiocytosis of the lumbar spine in an adult: Case report and review of the literature

“…The incidence rate of LCH is approximately 1:1,500,000 (3). Although LCH mostly occurs during childhood, it may affect patients of any age from infants to elderly individuals.…”

“…The most frequent sites of the bony lesions of LCH are the skull, femur, mandible, pelvis and spine (3,6). LCH in the spine is reported to occur in between 6.5 and 25% of cases (7), with the most frequent site being the thoracic vertebrae (54%), followed by the lumbar (35%) and cervical (11%) vertebrae (5).…”

“…A variety of treatment modalities have been reported for the management of LCH of the spine, including conservative treatments, systemic chemotherapy, curettage (with or without bone grafting), internal fixation and fusion, percutaneous vertebroplasty (PVP), corticosteroid injection into the lesion and radiotherapy (3). Although the clinical results are largely satisfactory, there is not a defined therapeutic algorithm.…”