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Liver involvement of Langerhans' cell histiocytosis in children
Xiaoping Yi1, Tong Han2, Hongyan Zai2
1Department of Radiology, Xiangya Hospital, Central South University Changsha 410008, China.
Insights
Liver involvement in children with Langerhans cell histiocytosis (LCH) is common, affecting over half of patients. Early diagnosis and systemic chemotherapy are crucial for better outcomes in pediatric LCH liver disease.
Area of Science:
- Pediatric Oncology
- Hepatology
- Radiology
Background:
- Liver involvement is a frequent complication in pediatric Langerhans cell histiocytosis (LCH).
- The specific clinical, pathological, and radiological features of hepatic LCH in children are not well-defined.
- Understanding these features is critical for timely diagnosis and management.
Purpose of the Study:
- To investigate the clinicopathological and radiological characteristics of hepatic Langerhans cell histiocytosis in children.
- To determine the incidence and impact of liver involvement on survival in pediatric LCH patients.
- To evaluate the role of imaging modalities in assessing hepatic LCH.
Main Methods:
- Retrospective analysis of 14 pediatric patients with hepatic LCH.
- Review of clinical, pathological, and radiological data.
- Correlation of imaging findings (CT, MRI) with disease staging and extent.
Main Results:
- The study identified a 51.9% rate of liver involvement in pediatric LCH patients, predominantly in disseminated cases.
- Hepatomegaly (78.6%) and liver function abnormalities (64.3%) were common clinical findings.
- Multi-modal imaging, including CT and MRI, provided significant diagnostic information for staging and assessing lesion extent, impacting survival outcomes.
Conclusions:
- The actual rate of liver involvement in pediatric LCH may be underestimated.
- Regular clinical, biological, and abdominal imaging screening is recommended for all pediatric LCH patients at diagnosis.
- Early initiation of systemic chemotherapy is associated with improved outcomes in children with hepatic LCH.
Objective:
Liver involvement is relatively frequent in children with Langerhans cell histiocytosis (LCH). Its features remain poorly defined.
Methods:
A retrospective study was carried out on 14 hepatic LCH children in our hospital. The Clinicopathological and radiological features of this disease was discussed.
Results:
The rate of liver involvement in children LCH patients is 51.9%. Majority of the patients were disseminated cases. Hepatomegaly was clinically confirmed in 11 cases (78.6%). Liver function dysfunction was seen in nine (64.3%) children. The association of multi-modal imaging significantly yielded more diagnostic information. There are some imaging characteristics of this disease, CT and MRI could help to assess the staging, extent of the hepatic lesions. We found that liver involvement had a significant impact on survival. Patients treated with systemic chemotherapy earlier from time of diagnosis had a relatively better outcome.
Conclusions:
The rate of liver involvement in children LCH patients maybe much higher than that of expected. We suggest that clinical and biological liver evaluation and abdominal imaging must be performed regularly onwards to screen every LCH children patient from the time of the initial diagnosis. Patient should be treated with systemic chemotherapy earlier.
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