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Original Article
A Study of Central Diabetes Insipidus in Head and Neck Langerhans Cell Histiocytosis: A Single Center Experience
Clin Pediatr Hematol Oncol 2018;25:136-41.
Published online October 31, 2018
© 2018 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Mi Jin Lee, M.D., Byeong Sub Park, M.D., Yeong Myong Yoo, M.D., Hyun Joo Jung, M.D. and Jun Eun Park, M.D., Ph.D.

Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea
Correspondence to: Jun Eun Park
Department of Pediatrics, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon 16499, Korea
Tel: +82-31-219-5167
Fax: +82-31-219-5169
Received September 17, 2018; Revised September 26, 2018; Accepted October 2, 2018.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Background: Langerhans cell histiocytosis (LCH) frequently involves the head and neck and increases the risk of central nervous system (CNS) involvement of LCH, such as central diabetes insipidus (CDI), when the craniofacial bones are involved. We analyzed risk factors and clinical features of CDI among patients with LCH involving the head and neck.
Methods: From January 1, 2000 to May 1, 2018, 63 patients with histologically confirmed LCH in the Department of Pediatrics, Ajou University Hospital were retrospectively analyzed.
Results: Forty eight cases (76.2%) of patients had head and neck involvement, and 9 cases (14.3%) in craniofacial bones at the time of initial diagnosis of LCH. CDI was found in 6 cases (9.5%) among all LCH patients, 6 cases (12.2%) among patients with head and neck involvement, and 3 cases (33.3%) among patients with craniofacial bone involvement. Three cases of CDI occurred at the time of initial LCH diagnosis, and another 3 cases occurred at the time of 2, 4, and 8 years after initial LCH diagnosis. Of the 6 CDI patients, 3 had CNS risk lesions and 3 had no CNS risk lesions, but all had multisystem involvement of LCH.
Conclusion: CDI can occur even in patients with head and neck LCH without CNS risk lesions, if there are multisystem involvement of LCH. Patients with head and neck LCH may develop CDI over time, so continuous observations should be done while considering the occurrence of CDI.
Keywords: Langerhans cell histiocytosis, Head and neck, Central diabetes insipidus
  1. Haupt R, Minkov M, Astigarraga I, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 2013;60:175-84.
    Pubmed KoreaMed CrossRef
  2. Nicollas R, Rome A, Belaïch H, et al. Head and neck manifestation and prognosis of Langerhans' cell histiocytosis in children. Int J Pediatr Otorhinolaryngol 2010;74:669-73.
    Pubmed CrossRef
  3. Buchmann L, Emami A, Wei JL. Primary head and neck Langerhans cell histiocytosis in children. Otolaryngol Head Neck Surg 2006;135:312-7.
    Pubmed CrossRef
  4. DiNardo LJ, Wetmore RF. Head and neck manifestations of histiocytosis-X in children. Laryngoscope 1989;99:721-4.
    Pubmed CrossRef
  5. Grois N, Fahrner B, Arceci RJ, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr 2010;156:873-81.
    Pubmed CrossRef
  6. Kim BE, Koh KN, Suh JK, et al. Clinical features and treatment outcomes of Langerhans cell histiocytosis: a nationwide survey from Korea histiocytosis working party. J Pediatr Hematol Oncol 2014;36:125-33.
    Pubmed CrossRef
  7. Haupt R, Nanduri V, Calevo MG, et al. Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group. Pediatr Blood Cancer 2004;42:438-44.
    Pubmed CrossRef
  8. Braier J, Chantada G, Rosso D, et al. Langerhans cell histiocytosis:retrospective evaluation of 123 patients at a single institution. Pediatr Hematol Oncol 1999;16:377-85.
    Pubmed CrossRef
  9. Donadieu J, Rolon MA, Thomas C, et al. Endocrine involvement in pediatric-onset Langerhans' cell histiocytosis: a populationbased study. J Pediatr 2004;144:344-50.
    Pubmed CrossRef
  10. Grois N, Pötschger U, Prosch H, et al. Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatr Blood Cancer 2006;46:228-33.
    Pubmed CrossRef
  11. Maghnie M, Cosi G, Genovese E, et al. Central diabetes insipidus in children and young adults. N Engl J Med 2000;343:998-1007.
    Pubmed CrossRef
  12. Donadieu J, Rolon MA, Pion I, et al. Incidence of growth hormone deficiency in pediatric-onset Langerhans cell histiocytosis:efficacy and safety of growth hormone treatment. J Clin Endocrinol Metab 2004;89:604-9.
    Pubmed CrossRef
  13. Grois N, Prayer D, Prosch H, Minkov M, Pötschger U, Gadner H. Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis. Pediatr Blood Cancer 2004;43:59-65.
    Pubmed CrossRef
  14. Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016;127:2672-81.
    Pubmed KoreaMed CrossRef
  15. Maria Postini A, del Prever AB, Pagano M, et al. Langerhans cell histiocytosis: 40 years' experience. J Pediatr Hematol Oncol 2012;34:353-8.
    Pubmed CrossRef
  16. Abla O, Egeler RM, Weitzman S. Langerhans cell histiocytosis:Current concepts and treatments. Cancer Treat Rev 2010;36:354-9.
    Pubmed CrossRef
  17. Alston RD, Tatevossian RG, McNally RJ, Kelsey A, Birch JM, Eden TO. Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998. Pediatr Blood Cancer 2007;48:555-60.
    Pubmed CrossRef
  18. Guyot-Goubin A, Donadieu J, Barkaoui M, Bellec S, Thomas C, Clavel J. Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000-2004. Pediatr Blood Cancer 2008;51:71-5.
    Pubmed CrossRef
  19. Zinn DJ, Chakraborty R, Allen CE. Langerhans cell histiocytosis:emerging insights and clinical implications. Oncology (Williston Park) 2016;30:122-32.
  20. Jezierska M, Stefanowicz J, Romanowicz G, Kosiak W, Lange M. Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy Dermatol Alergol 2018;35:6-17.
    Pubmed KoreaMed CrossRef
  21. Quraishi MS, Blayney AW, Walker D, Breatnach FB, Bradley PJ. Langerhans' cell histiocytosis: head and neck manifestations in children. Head Neck 1995;17:226-31.
    Pubmed CrossRef
  22. Koh KN, Im HJ, Seo JJ. Recent updates in Langerhans cell histiocytosis. Clin Pediatr Hematol Oncol 2015;22:15-21.
  23. Grois N, Flucher-Wolfram B, Heitger A, Mostbeck GH, Hofmann J, Gadner H. Diabetes insipidus in Langerhans cell histiocytosis: results from the DAL-HX 83 study. Med Pediatr Oncol 1995;24:248-56.
    Pubmed CrossRef
  24. Wnorowski M, Prosch H, Prayer D, Janssen G, Gadner H, Grois N. Pattern and course of neurodegeneration in Langerhans cell histiocytosis. J Pediatr 2008;153:127-32.
    Pubmed CrossRef
  25. Broadbent V, Pritchard J. Diabetes insipidus associated with Langerhans cell histiocytosis: is it reversible? Med Pediatr Oncol 1997;28:289-93.

October 2018, 25 (2)
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  • Jun Eun Park