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MRI features of intra-axial histiocytic brain mass lesions
1Russell H. Morgan Department of Radiology and Radiological Science, Division of Neuroradiology, The Johns Hopkins University School of Medicine, Baltimore, USA.
Aim:
To describe MRI features, including diffusion-weighted imaging (DWI), magnetic resonance spectroscopy (MRS), and perfusion-weighted imaging (PWI), of intra-axial tumour-like presentations of four different subtypes of histiocytosis.
Material And Methods:
The brain MRI findings of 23 patients with histologically proven histiocytosis were reviewed retrospectively (11 Langerhans cell histiocytosis [LCH], eight Erdheim-Chester disease [ECD], one overlap form LCH/ECD, two Rosai-Dorfman disease [RDD], and one haemophagocytic lymphohistiocytosis [HLH]) with single or multiple enhancing intraparenchymal brain lesions.
Results:
Histiocytic brain mass lesions show some similar MRI features including Supra and/or infratentorial and/or paraventricular subcortical well-delineated masses, linear ependymal enhancement along the ventricles and brain stem lesions. Masses always present with mixed hyper- and hypointense signal on T2-weighted imaging (WI). Their enhancement is often homogeneous. Apparent diffusion coefficient (ADC) values are often normal or elevated.
Conclusion:
The presence of multiple periventricular and subcortical enhancing lesions with mixed signal intensity on T2WI and normal or high ADC values should lead radiologists to consider the diagnosis of histiocytic lesions and search for associated systemic lesions.
Insights
MRI reveals specific features of histiocytosis brain lesions, including enhancing periventricular masses with mixed T2 signal and normal or high apparent diffusion coefficient (ADC) values, aiding diagnosis.
Area of Science:
- Neuroradiology
- Oncology
- Pathology
Background:
- Histiocytosis encompasses rare disorders characterized by abnormal proliferation of histiocytes.
- Intra-axial brain lesions in histiocytosis can mimic primary or metastatic tumors.
- Accurate diagnosis relies on characteristic imaging findings and histopathological confirmation.
Purpose of the Study:
- To delineate the Magnetic Resonance Imaging (MRI) features of intra-axial histiocytic lesions.
- To evaluate the utility of diffusion-weighted imaging (DWI), magnetic resonance spectroscopy (MRS), and perfusion-weighted imaging (PWI) in characterizing these lesions.
- To differentiate histiocytosis from other intra-axial brain tumors.
Main Methods:
- Retrospective review of brain MRI scans from 23 histologically confirmed histiocytosis patients.
- Inclusion of subtypes: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), Rosai-Dorfman disease (RDD), and haemophagocytic lymphohistiocytosis (HLH).
- Analysis of lesion characteristics on T2-weighted imaging, enhancement patterns, and apparent diffusion coefficient (ADC) values.
Main Results:
- Common MRI findings include supra/infratentorial, paraventricular, subcortical masses with well-defined borders.
- Linear ependymal enhancement and brainstem lesions were observed.
- Lesions typically show mixed hyper- and hypointense signals on T2WI with homogeneous enhancement and normal or elevated ADC values.
Conclusions:
- Multiple periventricular and subcortical enhancing lesions with mixed T2 signal and normal/high ADC values suggest histiocytic lesions.
- Radiologists should consider histiocytosis in the differential diagnosis for such presentations.
- Correlation with systemic findings is crucial for definitive diagnosis and management.

