1. Neuroimaging features of CNS histiocytosis syndromes.
- Author
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Wang, Yuting, Camelo-Piragua, Sandra, Abdullah, Asif, Ibrahim, Mohannad, and Parmar, Hemant A.
- Subjects
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LANGERHANS-cell histiocytosis , *CENTRAL nervous system , *HEMATOPOIESIS , *NON-langerhans-cell histiocytosis , *SYNDROMES , *CALVARIA , *BRAIN imaging - 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). • Histiocytosis syndromes (HS) are group of disorders characterized by abnormal infiltration of histiocytes, cells derived from hematopoietic cells of monocyte/macrophage lineage. • Revised classification from 2016 incorporates clinical, radiographic, pathological, phenotypic, genetic, and/or molecular features. However, diagnosis remains challenging and definitive diagnosis often necessitates pathologic examination. • LCH typically causes enhancing suprasellar mass/ thick infundibular stalk, with classic "bone in bone' type calvarial lesions and vertebra plana. • ECD can present with infiltrative intracranial masses, lesions of the infundibular stalk and focal nodular dural thickening. Diffuse infiltrative orbital lesions are also seen. • Intracranial features of RDD often involved dural based lesions mimicking meningioma. Parenchymal lesions with RDD are rare. [ABSTRACT FROM AUTHOR]
- Published
- 2020
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