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Pediatric histiocytoses in the United States: incidence and outcomes
Samuel Golpanian1, Jun Tashiro1, David J Gerth1
1Division of Plastic, Aesthetic, and Reconstructive Surgery, Department of Surgery, University of Miami, Leonard M. Miller School of Medicine, Miami, Florida.
Insights
Pediatric histiocytoses, including Langerhans cell histiocytosis (class I) and malignant histiocytosis (class III), show varying incidence and survival. Surgical intervention improves outcomes for class I disease, while class III has lower survival.
Area of Science:
- Pediatric oncology
- Hematology
- Epidemiology
Background:
- Histiocytoses are rare pediatric disorders.
- This study analyzes incidence and survival of pediatric histiocytoses.
Purpose of the Study:
- To investigate the epidemiology and outcomes of pediatric histiocytoses.
- To compare class I (Langerhans cell histiocytosis) and class III (malignant histiocytosis).
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results database (1973-2010).
- Analyzed demographics, clinical characteristics, and survival for 828 pediatric cases (<20 years).
Main Results:
- Overall incidence was 0.142/100,000; highest in younger children and certain ethnicities.
- Class III disease showed higher incidence, disseminated presentation, and lower median survival (33 months) compared to class I.
- Younger children (<1 year) and adolescents (15-19 years) had the worst outcomes; surgical excision improved survival, radiation had no effect.
- Survival improved over the study period.
Conclusions:
- Class I histiocytosis presents with localized disease and benefits from surgery.
- Class III histiocytosis has higher incidence, disseminated disease, and poorer survival.
- Radiation therapy did not impact survival; overall survival has improved over four decades.
Background:
Histiocytoses are rare disorders affecting the pediatric population.
Materials And Methods:
Surveillance, Epidemiology, and End Results database was searched for pediatric cases (<20 y old) of histiocytosis diagnosed between 1973 and 2010. Demographics, clinical characteristics, and survival outcomes were analyzed using standard statistical methods. Class I disease (Langerhans cell histiocytosis) and class III (malignant histiocytosis) were included in the data set.
Results:
A total of 828 cases were identified. Overall incidence was 0.142/100,000 persons per annum. Incidence was highest in younger children and those of Asian or Native American descent. Class III disease had a higher incidence versus class I. Adolescents tended to present with class III, whereas young children presented with class I. Disseminated disease was present in most cases of class III, whereas class I had more localized cases. Surgical excision was more likely to be performed in class I. Overall median survival was 349 mo. Patients 15-19 y old and children<1 y old had the worst outcomes. Class I had higher survival compared with class III, which had a median survival of 33 mo. Cases with hematologic spread carried the worst prognosis. Surgical excision conferred a survival advantage while radiation had no effect. Survival improved over the study period. Gender and race had no association with survival.
Conclusions:
Class I disease had localized cases and showed benefit from surgical intervention. Class III disease had a higher incidence and was associated with disseminated disease and lower survival. Radiation therapy did not affect survival. Overall survival increased over the previous 40 y.
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