Skip to main content

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.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2


  1. Aizawa T, Sato T, Tanaka Y, Kokubun S (2005) Signal intensity changes on MRI during the healing process of spinal Langerhans cell granulomatosis: report of two cases. J Spinal Disord Tech 18:98–101

    Article  PubMed  Google Scholar 

  2. Azouz EM, Saigal G, Rodriguez MM, Podda A (2005) Langerhans’ cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 35:103–115

    Article  PubMed  Google Scholar 

  3. Bertram C, Madert J, Eggers C (2002) Eosinophilic granuloma of the cervical spine. Spine 27:1408–1413

    Article  PubMed  Google Scholar 

  4. Floman Y, Bar-On E, Mosheiff R, Mirovsky Y, Robin GC, Ramu N (1997) Eosinophilic granuloma of the spine. Pediatr Orthop (Br) 6(4):260–265

    CAS  Google Scholar 

  5. Garg S, Mehta S, Dormans JP (2003) An atypical presentation of Langerhans cell histiocytosis of the cervical spine in a child. Spine 28:E445–E448

    Article  PubMed  Google Scholar 

  6. Garg S, Mehta S, Dormans JP (2004) Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Jt Surg Am 86:1740–1750

    Google Scholar 

  7. Goldberg SA, O’Connor SC, Sprinz PG (2008) Prostaglandin inhibitors in the treatment of single-system Langerhans cell histiocytosis: pharmacologic rationale and report of two cases. Pediatr Hematol Oncol 30:778–780

    Article  Google Scholar 

  8. Greenberger JS, Crocker AC, Vawter G, Jaffe N, Cassady JR (1981) Results of treatment of 127 patients with systemic histiocytosis. Medicine (Baltimore) 60:311–338

    Article  CAS  Google Scholar 

  9. Islinger RB, Kuklo TR, Owens BD, Horan PJ, Choma TJ, Murphey MD, Temple HT (2000) Langerhans’ cell histiocytosis in patients older than 21 years. Clin Orthop Relat Res 379:231–235

    Article  PubMed  Google Scholar 

  10. Levine SE, Dormans JP, Meyer JS, Corcoran TA (1996) Langerhans’ cell histiocytosis of the spine in children. Clin Orthop Relat Res 323:288–293

    Article  PubMed  Google Scholar 

  11. Liu XG, Zhong WQ, Liu ZJ, Yuan HS, Jiang L, Ma QJ, Wei F, Dang GT (2009) Diagnosis and treatment of Langerhans cell histiocytosis of the cervical spine. Chin J Spine Spinal Cord 19(6):431–436

    Google Scholar 

  12. Mammano S, Candiotto S, Balsano M (1997) Cast and brace treatment of eosinophilic granuloma of the spine: long-term follow-up. J Pediatr Orthop 17:821–827

    Article  CAS  PubMed  Google Scholar 

  13. Ngu BB, Khanna AJ, Pak SS, McCarthy EF, Sponseller PD (2004) Eosinophilic granuloma of the atlas presenting as torticollis in a child. Spine 29(5):E98–E100

    Article  PubMed  Google Scholar 

  14. Peng XS, Pan T, Chen LY, Huang G, Wang J (2008) Langerhans’ cell histiocytosis of the spine in children with soft tissue extension and chemotherapy. International Orthopaedics (SICOT) online

  15. Puigdevall M, Bosio S, Hokama J, Maenza R (2008) Langerhans cell histiocytosis of the atlas in the pediatric spine: total reconstitution of the bone lesion after nonoperative treatment. A report of two cases. J Bone Jt Surg Am 90:1994–1997

    Article  Google Scholar 

  16. Robert H, Dubousset J, Miladi L (1987) Histiocytosis X in the juvenile spine. Spine 12(2):167–172

    Article  CAS  PubMed  Google Scholar 

  17. Sessa S, Sommelet D, Lascombes P, Prevot J (1994) Treatment of Langerhans-cell histiocytosis in children. J Bone Jt Surg Am 76:1513–1525

    CAS  Google Scholar 

  18. Tan HQ, Li MH, Wu CG, Gu YF, Zhang H, Fang C (2007) Percutaneous vertebroplasty for eosinophilic granuloma of the cervical spine in a child. Pediatr Radiol 37:1053–1057

    Article  PubMed  Google Scholar 

  19. Tanaka N, Fujimoto Y, Okuda T, Nakanishi K, Sumida T, Manabe H, Ochi M (2005) Langerhans cell histiocytosis of the atlas. A report of three cases. J Bone Jt Surg Am 87:2313–2317

    Article  Google Scholar 

  20. Von Stebut E, Schadmand-Fischer S, Bräuninger W, Kreft A, Doberauer C, Steinbrink K (2008) Successful treatment of adult multisystemic Langerhans cell histiocytosis with psoralen-UV-A, prednisolone, mercaptopurine, and vinblastine. Arch Dermatol 144:649–653

    Article  Google Scholar 

  21. Yeom JS, Lee CK, Shin HY, Lee CS, Han CS, Chang H (1999) Langerhans’ cell histiocytosis of the spine analysis of twenty-three cases. Spine 24:1740–1749

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Liang Jiang.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Zhong, W.Q., Jiang, L., Ma, Q.J. et al. Langerhans cell histiocytosis of the atlas in an adult. Eur Spine J 19, 19–22 (2010).

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI:


  • Langerhans cell histiocytosis
  • Eosinophilic granuloma
  • Spine