Incidence
Risk Factors
Prognosis

Children and adolescents with Langerhans cell histiocytosis (LCH) should be treated by a multidisciplinary team of health professionals who are experienced with this disease and its treatment. This multidisciplinary team approach incorporates the skills of the primary care physician, pediatric surgical subspecialists, radiation oncologists, pediatric medical oncologists/hematologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life. (Refer to the PDQ Supportive and Palliative Care summaries for specific information about supportive care for children and adolescents with cancer.)

Clinical trials organized by the Histiocyte Society have been accruing patients on childhood treatment studies since the 1980s. Information on centers enrolling patients on these trials can be found on the NCI Web site.

Because of treatment advances, the outcome for children with LCH involving high-risk organs (spleen, liver, bone marrow and lung) has improved.[1] The outcome for children with LCH involving low-risk organs (skin, bones, lymph nodes and pituitary gland) has always been excellent, but the major challenge is to reduce the relatively high incidence (20%–30%) of recurrent lesions. Children with high-risk or low-risk disease should be followed annually to document and attempt to correct adverse side effects of therapy or the disease. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information about the incidence, type, and monitoring of late effects of childhood cancer and its therapy.

The incidence of LCH has been estimated to be two to 10 cases per million children aged 15 years or younger.[2] The male/female ratio is close to one and the median age of presentation is 30 months.[3] Identical twins with LCH, as well as non twin siblings or multiple cases in one family, have been reported.[4] A survey of LCH in northwest England (Manchester) revealed an overall incidence of 2.6 cases per million child years.[5]

Solvent exposure in parents and perinatal infections have a weak association with LCH, but there is no increase in cases after viral epidemics.[6] An increased frequency of family members with thyroid disease has been reported.[7]

The nomenclature used for LCH indicates the disease extent (i.e., single organ, single site [single system], multisystem [multisite or multiple sites] or multiple organs [diffuse disease]). Prognosis and treatment are closely linked to the extent of disease at presentation and whether high-risk organs (liver, spleen, lung, bone marrow) are involved. If patients with involvement of high-risk organs do not respond adequately by the 12th week of therapy, their chance of survival is 25% to 40%.[1] 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.[1] Involvement of orbital, mastoid, and temporal bones is associated with an increased risk of diabetes insipidus in addition to increased frequency of anterior pituitary hormone deficiencies and neurologic problems. (See discussion below on “CNS Risk” areas.)

References

1. 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.  [PUBMED Abstract]
   
   
2. 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. 
   
   
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.  [PUBMED Abstract]
   
   
4. Aricò M, Nichols K, Whitlock JA, et al.: Familial clustering of Langerhans cell histiocytosis. Br J Haematol 107 (4): 883-8, 1999.  [PUBMED Abstract]
   
   
5. 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.  [PUBMED Abstract]
   
   
6. Nicholson HS, Egeler RM, Nesbit ME: The epidemiology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 12 (2): 379-84, 1998.  [PUBMED Abstract]
   
   
7. Bhatia S, Nesbit ME Jr, Egeler RM, et al.: Epidemiologic study of Langerhans cell histiocytosis in children. J Pediatr 130 (5): 774-84, 1997.  [PUBMED Abstract]
   
   

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