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Published on: August 27, 2017
Neuroimaging features of CNS histiocytosis syndromes
Yuting Wang1, Sandra Camelo-Piragua2, Asif Abdullah3
1Department of Radiology, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Chengdu, China; Department of Radiology, University of Michigan Health System, Ann Arbor, MI, United States of America.
Insights
Histiocytosis syndromes are rare disorders of histiocyte accumulation affecting multiple organs, including the central nervous system. This review details imaging features of CNS histiocytosis to aid diagnosis.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Histiocytosis syndromes (HS) are rare, heterogeneous disorders involving abnormal histiocyte accumulation.
- These syndromes can affect multiple organ systems, notably the central nervous system (CNS).
- Diagnosis is challenging, often requiring pathological confirmation despite imaging clues.
Purpose of the Study:
- To review and describe the imaging features of histiocytosis syndromes affecting the CNS.
- To enhance understanding of CNS manifestations of these rare disorders.
Main Methods:
- Literature review focusing on imaging findings in CNS histiocytosis.
- Discussion of specific HS entities: Langerhans cell histiocytosis (LCH), Rosai-Dorfman Disease (RDD), Erdheim Chester Disease (ECD), hemophagocytic lymphohistiocytosis (HLH), and crystal-storing histiocytosis (CSH).
Main Results:
- Imaging findings in CNS histiocytosis are diverse and entity-specific.
- Radiological features can suggest HS but often require correlation with clinical and pathological data.
- Each discussed HS entity presents unique patterns of CNS involvement on imaging.
Conclusions:
- Recognizing characteristic imaging features is crucial for early suspicion of CNS histiocytosis.
- Integrated diagnostic approach combining imaging, clinical, and pathological findings improves diagnostic accuracy.
- Further research into imaging biomarkers may aid in differentiating HS subtypes affecting the CNS.
Abstract:
Histiocytosis syndromes (HS) are group of heterogeneous disorders characterized by abnormal accumulation and infiltration of histiocytes, cells derived from hematopoietic cells of monocyte/macrophage lineage. Overall these disorders are rare. When they do occur they involve many organ systems including the central nervous system (CNS). While imaging findings can provide important clues, diagnosis of this disorder is challenging and definitive diagnosis often necessitates pathologic examination. In this review, we describe imaging features of HS involving the CNS, with the aim to increase our understanding of these disorders. The entities discussed in this review will include: Langerhans cell histiocytosis (LCH), Rosai-Dorfman Disease (RDD), Erdheim Chester Disease (ECD), hemophagocytic lymphohistiocytosis (HLH), and crystal-storing histiocytosis (CSH).
