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.
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.
Many of the medical and scientific terms used in this summary are found in the NCI Dictionary of Genetics Terms. When a linked term is clicked, the definition will appear in a separate window.
Creating evidence-based summaries on cancer genetics is challenging because the rapid evolution of new information often results in evidence that is incomplete or of limited quality. In addition, established methods for evaluating the quality of the evidence are available for some, but not all, aspects of...
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 Late Disease and Treatment Effects of Childhood LCH section of this summary for more information about the incidence, type, and monitoring of late effects of childhood cancer and its therapy.)
The annual incidence of LCH has been estimated to be two to ten cases per 1 million children aged 15 years or younger.[1,2] The male to female ratio (M/F) is close to one and the median age of presentation is 30 months. A report from Stockholm County, Sweden, described an annual incidence of 8.9 cases of LCH per 1 million children with a total of 29 cases in 10 years. Most of these cases were diagnosed between September and February (M/F = 1.2). A 4-year survey of 251 new LCH cases in France found an annual incidence of 4.6 cases per 1 million children younger than 15 years (M/F = 1.2). A survey of LCH in northwest England (Manchester) revealed an overall incidence of 2.6 cases per 1 million child-years.
Identical twins with LCH, and non-twin siblings or multiple cases in one family, have been reported. Over 90% of adult pulmonary LCH occurs in young adults who smoke, often more than 20 cigarettes per day.[8,9]