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Langerhans Cell Histiocytosis in the central nervous system, cranial and spinal bones: imaging features
Lara Hemerly De Mori1, Gabriela Amaral Ribas1, Luciane Dos Santos Oliveira1
1Department of Diagnostic Imaging, Rio de Janeiro State University, Rio de Janeiro, Brazil.
Insights
Langerhans Cell Histiocytosis (LCH) is a rare disorder involving pathological cell growth. This review details CNS, cranial, and spinal bone involvement, focusing on imaging, clinical, and pathological findings.
Area of Science:
- Oncology
- Neurology
- Radiology
Background:
- Langerhans Cell Histiocytosis (LCH) is a rare neoplastic disorder of pathological dendritic cell proliferation.
- It is often linked to MAPK pathway activation and BRAF V600E mutations.
- LCH can affect various organs, including bones, skin, and the central nervous system (CNS).
Purpose of the Study:
- To review the imaging, clinical, and pathological features of CNS, cranial, and spinal bone involvement in LCH.
- To discuss the differential diagnosis of LCH in these areas.
- To highlight the role of imaging in diagnosis and management.
Main Methods:
- Literature search of PubMed from 1989 to 2024.
- Inclusion of original research, reviews, and case reports.
- Exclusion of editorials and letters to the editor.
Main Results:
- Imaging, including CT and MRI, is crucial for diagnosing LCH bone and CNS involvement.
- LCH can present as tumoral or neurodegenerative forms with varied symptoms.
- Accurate diagnosis requires integrating clinical, radiological, and histopathological data.
Conclusions:
- Comprehensive evaluation is necessary for LCH diagnosis and management.
- Understanding imaging findings aids in differentiating LCH from other conditions.
- Further research may refine diagnostic and therapeutic strategies for LCH.
Introduction:
Langerhans Cell Histiocytosis (LCH) is a rare neoplastic disorder characterized by monoclonal proliferation and tissue infiltration by pathological dendritic cells, often associated with abnormal activation of the MAPK signaling pathway and BRAF V600E mutation. The diagnosis is established through clinical, radiological, and histopathological evaluation. It can manifest in various organs, notably bones, skin, and the hypothalamic-pituitary axis. The assessment of central nervous system (CNS) involvement in LCH includes both parenchymal and non-parenchymal structures, including craniofacial bones, hypothalamic-pituitary axis, and meninges.
Areas Covered:
We used the PubMed database and reviewed relevant English-language literature published from 1989 to 2024. The sources included original researches, reviews and case reports, excluding editorials and letters to the editor. This review focuses on the existing evidence regarding the imaging, clinical, and pathological aspects of CNS, cranial and spinal bones involvement in LCH and its differential diagnosis.
Expert Opinion:
Imaging plays an essential role in properly diagnosing, guiding biopsy and monitoring treatment. Computed tomography (CT) is particularly helpful for evaluating bone involvement, while magnetic resonance imaging (MRI) is preferred for assessing parenchymal brain and meningeal lesions. The disease can present in either tumoral or neurodegenerative forms, manifesting with varied symptoms depending on the affected region.
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