Skip to main content

Advertisement

SpringerLink
Log in

Histiocytosis in the pediatric spine: a clinical and radiographic analysis of 50 patients

  • Case Series
  • Published:
Spine Deformity Aims and scope Submit manuscript

Abstract

Study design

Descriptive, retrospective. Scientific level of evidence IV.

Objectives

The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine.

Summary of background data

Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine.

Material and methods

A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2 years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed.

Results

Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5 years and 2 months (6 months to 13 years and 3 months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3 years and 5 months (range 2–12 years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment.

Conclusions

Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Dubousset R, Miladi L (1987) Histiocitosis X in the juvenile spine. Spine 12:167–172

    Article  Google Scholar 

  2. Badalian Very G, Vergilio JA, Degar BA et al (2010) Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 116(11):1919–1923

    Article  CAS  Google Scholar 

  3. Jang KS, Jung YY, KIM SW, (2010) Langerhans cell histiocytosis causing cervical myelopathy in a child. J Korean Neurosurg Soc 47(6):458–460

    Article  Google Scholar 

  4. Lichtenstein L, Histiocytosis X (1953) Integration of eosinophilic granuloma of bone, “Letterer-Sewe disease”, and “Hand Schuller –Christian disease” as related manifestations of a single nosologic entity. Arch Pathol 56:84–102

    CAS  Google Scholar 

  5. Lichtenstein L (1964) Histiocytosis X (eosinophilic granuloma of bone, Letterer Siwe disease, and Schuller- Christian disease). Further observations of pathological and clinical importance. JBJS A46:76–90

    Article  Google Scholar 

  6. Ng-Cheng-Hin B, O’Hanlon-Brown C, Alifrangis C et al (2011) Langerhans cell histiocytosis: old disease new treatment. QJM 104(2):89–96

    Article  CAS  Google Scholar 

  7. Dundon C, Williams H, Laipply T (1946) Eosinophilic Granuloma of bone. Radiology 47:433–444

    Article  CAS  Google Scholar 

  8. Peng X, Pan T, Chen L et al (2009) Langerhans’ cell histiocytosis of the spine in children with soft tissue extension and chemotherapy. Int Orthop 33(3):731–736

    Article  Google Scholar 

  9. Mammano S, Candiotto S, Balsano M (1997) Cast and brace treatment of eosinophilic granuloma of the spine: Long term follow up. JPO 17(6):817–827

    Google Scholar 

  10. Schajowicz F, Slullitel J (1973) Eosinophilic granuloma of bone and its relationship to Hand Schuller- Christian and Letterer – Siwe syndromes. JBJS (BR) 55:545–565

    Article  CAS  Google Scholar 

  11. Nesbit M, Kieffer S, D´Angio G (1969) Reconstitution of vertebral height in histiocytosis X: A long term follow up. JBJS 51A:1360–1368

    Article  Google Scholar 

  12. Poulsen J, Jensen J, Thommesen P (1975) Ewing´s sarcoma simulating vertebra plana. Acta Orthop Scand 46:211–215

    Article  CAS  Google Scholar 

  13. Garg S, Metha S, Dormans J (2003) An atypical presentation of Langerhans cell histiocytosis of the cervical spine in a child. Spine 28:E445-448

    Article  Google Scholar 

  14. Bertram C, Madert J, Eggers C (2002) Eosinophilic granuloma of the cervical spine. Spine 27(13):1408–1413

    Article  Google Scholar 

  15. Yeom JS, Lee CK, Shin SH et al (1999) Langerhans cell histiocytosis of the spine: Analysis of twenty- three cases. Spine 24(16):1740

    Article  CAS  Google Scholar 

  16. Garg S, Metha S, Dormans J (2004) Langerhans cell histiocytosis of the spine in children: long term follow up. JBJS A86:1740–1750

    Article  Google Scholar 

  17. Seimon L (1981) Eosinophilic granuloma of the spine. JPO 1:371–376

    CAS  Google Scholar 

  18. Huang WD, Yang XH, Wu ZP et al (2013) Langerhans cell histiocytosis of spine: a comparative study of clinical, imaging features, and diagnosis in children, adolescents, and adults. Spine J 13(9):1108–1117

    Article  Google Scholar 

  19. Bunch W (1981) Orthopedic and rehabilitation aspects of eosinophilic granuloma. Am J Ped Hem Oncol 3:151–156

    CAS  Google Scholar 

  20. Ochsner S (1966) Eosinophilic granuloma of bone: Experience with 20 cases. Am J Roentgenol 97:719–726

    Article  CAS  Google Scholar 

  21. Floman Y, Bar-On E, Mosheiff R et al (1997) Eosinophilic granuloma of the spine. JPO 6B:260–265

    Google Scholar 

  22. Sessa S, Sommelet D, Lascombes P et al (1994) Treatment of Langerhans-cell histiocytosis in children. JBJS 76A:1513–1525

    Article  Google Scholar 

  23. Lauffenburger M, Dull S, Toselli R et al (1995) Eosiniphilic granuloma of the adult spine: A case report and review of the literature. J Spine Disord 8:243–248

    Article  CAS  Google Scholar 

  24. Levine S, Dormans J, Meyer J et al (1996) Langerhans cell histiocytosis of the spine in children. Clin Orthop 323:288–293

    Article  Google Scholar 

  25. Ippolito E, Farsetti P, Tudisco C (1984) Vertebra plana. Long term follow up in five patients. JBJS 66A:1363–1368

    Google Scholar 

Download references

Funding

Not applicable.

Author information

Authors and Affiliations

  1. Servicio de Patología Espinal, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Combate de los Pozos 1881, C1245AAM, Buenos Aires, CABA, Argentina

    Carlos Alberto Moyano, Rodrigo German Remondino, Carlos Alberto Tello, Lucas Piantoni, Eduardo Galaretto, Ida Alejandra Francheri Wilson, Ernesto Bersusky & Mariano Augusto Noël

Authors
  1. Carlos Alberto Moyano
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rodrigo German Remondino
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Carlos Alberto Tello
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Lucas Piantoni
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Eduardo Galaretto
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ida Alejandra Francheri Wilson
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ernesto Bersusky
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mariano Augusto Noël
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Design of study: CAM, RGR, CAT, MAN. Consulting references: RGR, LP, IAFW, EG, EB. Participation in draft manuscript: CAM, RGR, CAT, LP. Revise manuscript: IAFW, CAT, RGR, EG, EB. Approved the final version of the paper: CAM, LP, RGR, CAT, IAFW, EG, EB, MAN.

Corresponding author

Correspondence to Carlos Alberto Moyano.

Ethics declarations

Conflicts of interest

The authors declare that they have no competing interests. No conflicts of interest or funding received during the conduct of this study.

IRB

The study was approved by the hospital Institutional Review Board (IRB), because of the retrospective observational nature of the study IRB waived the informed consent.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Moyano, C.A., Remondino, R.G., Tello, C.A. et al. Histiocytosis in the pediatric spine: a clinical and radiographic analysis of 50 patients. Spine Deform 9, 823–831 (2021). https://doi.org/10.1007/s43390-020-00261-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s43390-020-00261-8

Keywords

Search

Navigation