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    Langerhans Cell Histiocytosis Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Childhood LCH

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    Risk Factors

    Although the following risk factors have been identified for LCH, strong and consistent associations have not been confirmed:

    • Solvent exposure in parents.[10]
    • Family history of cancer.[11]
    • Personal or family history of thyroid disease.[10,12]
    • Perinatal infections.[10,11]
    • Parental occupational exposure to metal, granite, or wood dust.[11]

    Prognosis

    Prognosis is closely linked to the extent of disease at presentation when high-risk organs (liver, spleen, and/or bone marrow) are involved and to the response to initial treatment. The high-risk designation comes from the high mortality rate (35%) in those who did not respond well to therapy in the first 6 weeks. For many years, lung was thought to be a high-risk organ but isolated lung involvement in pediatric LCH is no longer considered to pose a significant risk of death.[13] Because of treatment advances, the outcome for children with LCH involving high-risk organs has improved.[14,15] Data from HISTSOC-LCH-III showed an 84% overall survival (OS) rate for patients treated for 12 months with systemic chemotherapy.[16]

    Patients with single-system disease and low-risk multisystem disease do not usually die from LCH, but recurrent disease may result in considerable morbidity and significant late effects.[17] The major treatment challenge is to reduce the 20% to 30% incidence of recurrent lesions and the significant incidence of permanent consequences in this group of patients. HISTSOC-LCH-III data showed that there was a significant difference in reactivation for risk organ subjects when comparing 6 months versus 12 months of treatment, with 12 months being better (54% vs. 37%).[16]

    Prognostic factors in LCH have been identified and can be categorized as follows:

    • Age at diagnosis: Although age younger than 2 years was once thought to portend a worse prognosis, data from the LCH-II study showed that patients aged 2 years or younger without high-risk organ involvement had the same response to therapy as older patients.[15] By contrast, the OS was poorer in neonates with risk-organ involvement compared with infants and children with the same extent of disease when patients were treated for only 6 months.[15]
    • Response to treatment: Response to therapy at 6 to 12 weeks has been shown to be a more important prognostic factor than age.[18] The overall response to therapy is influenced by the duration and intensity of treatment.[14,15]
    • Organ involvement: Involvement of craniofacial bones including orbital, mastoid, and temporal bones is associated with an increased risk of diabetes insipidus and an increased frequency of anterior pituitary hormone deficiencies and neurologic problems. (Refer to the Endocrine system subsection in the Multisystem Disease Presentation section of this summary for more information on diabetes insipidus.)

    References:

    1. Carstensen H, Ornvold K: The epidemiology of Langerhans cell histiocytosis in children in Denmark, 1975-89. [Abstract] Med Pediatr Oncol 21 (5): A-15, 387-8, 1993.
    2. Salotti JA, Nanduri V, Pearce MS, et al.: Incidence and clinical features of Langerhans cell histiocytosis in the UK and Ireland. Arch Dis Child 94 (5): 376-80, 2009.
    3. A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans' Cell Histiocytosis Study Group. Arch Dis Child 75 (1): 17-24, 1996.
    4. Stålemark H, Laurencikas E, Karis J, et al.: Incidence of Langerhans cell histiocytosis in children: a population-based study. Pediatr Blood Cancer 51 (1): 76-81, 2008.
    5. Guyot-Goubin A, Donadieu J, Barkaoui M, et al.: Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000-2004. Pediatr Blood Cancer 51 (1): 71-5, 2008.
    6. Alston RD, Tatevossian RG, McNally RJ, et al.: Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998. Pediatr Blood Cancer 48 (5): 555-60, 2007.
    7. Aricò M, Nichols K, Whitlock JA, et al.: Familial clustering of Langerhans cell histiocytosis. Br J Haematol 107 (4): 883-8, 1999.
    8. Tazi A, Soler P, Hance AJ: Adult pulmonary Langerhans' cell histiocytosis. Thorax 55 (5): 405-16, 2000.
    9. Vassallo R, Ryu JH, Colby TV, et al.: Pulmonary Langerhans'-cell histiocytosis. N Engl J Med 342 (26): 1969-78, 2000.
    10. Bhatia S, Nesbit ME Jr, Egeler RM, et al.: Epidemiologic study of Langerhans cell histiocytosis in children. J Pediatr 130 (5): 774-84, 1997.
    11. Venkatramani R, Rosenberg S, Indramohan G, et al.: An exploratory epidemiological study of Langerhans cell histiocytosis. Pediatr Blood Cancer 59 (7): 1324-6, 2012.
    12. Nicholson HS, Egeler RM, Nesbit ME: The epidemiology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 12 (2): 379-84, 1998.
    13. Ronceray L, Pötschger U, Janka G, et al.: Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome. J Pediatr 161 (1): 129-33.e1-3, 2012.
    14. Gadner H, Grois N, Arico M, et al.: A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr 138 (5): 728-34, 2001.
    15. Gadner H, Grois N, Pötschger U, et al.: Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood 111 (5): 2556-62, 2008.
    16. Gadner H, Minkov M, Grois N, et al.: Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood 121 (25): 5006-14, 2013.
    17. 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 42 (5): 438-44, 2004.
    18. Minkov M, Prosch H, Steiner M, et al.: Langerhans cell histiocytosis in neonates. Pediatr Blood Cancer 45 (6): 802-7, 2005.

    This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http:// cancer .gov or call 1-800-4-CANCER.

    WebMD Public Information from the National Cancer Institute

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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