Histiocytosis and the nervous system: From diagnosis to targeted therapies Review
| Authors: | Cohen Aubart, F.; Idbaih, A.; Emile, J. F.; Amoura, Z.; Abdel-Wahab, O.; Durham, B. H.; Haroche, J.; Diamond, E. L. |
| Review Title: | Histiocytosis and the nervous system: From diagnosis to targeted therapies |
| Abstract: | Histiocytoses are heterogeneous hematopoietic diseases characterized by the accumulation of CD68(+) cells with various admixed inflammatory infiltrates. The identification of the pivotal role of the mitogen-activated protein kinase (MAPK) pathway has opened new avenues of research and therapeutic approaches. We review the neurologic manifestations of 3 histiocytic disorders with frequent involvement of the brain and spine: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), and Rosai-Dorfman-Destombes disease (RDD). Central nervous system (CNS) manifestations occur in 10%-25% of LCH cases, with both tumorous or neurodegenerative forms. These subtypes differ by clinical and radiological presentation, pathogenesis, and prognosis. Tumorous or degenerative neurologic involvement occurs in 30%-40% of ECD patients and affects the hypothalamic-pituitary axis, meninges, and brain parenchyma. RDD lesions are typically tumorous with meningeal or parenchymal masses with strong contrast enhancement. Unlike LCH and ECD, neurodegenerative lesions or syndromes have not been described with RDD. Familiarity with principles of evaluation and treatment both shared among and distinct to each of these 3 diseases is critical for effective management. Refractory or disabling neurohistiocytic involvement should prompt the consideration for use of targeted kinase inhibitor therapies. © 2021 The Author(s) 2021. |
| Keywords: | clinical feature; pathogenesis; review; risk factor; central nervous system; contrast enhancement; histiocytosis; neurologic disease; nerve degeneration; langerhans cell histiocytosis; mapk pathway; mortality rate; nervous system tumor; prognosis; human; sinus histiocytosis; erdheim chester disease; erdheim-chester disease; rosai-dorfman-destombes disease |
| Journal Title: | Neuro-Oncology |
| Volume: | 23 |
| Issue: | 9 |
| ISSN: | 1522-8517 |
| Publisher: | Oxford University Press |
| Date Published: | 2021-09-01 |
| Start Page: | 1433 |
| End Page: | 1446 |
| Language: | English |
| DOI: | 10.1093/neuonc/noab107 |
| PUBMED: | 33993305 |
| PROVIDER: | scopus |
| PMCID: | PMC8408883 |
| DOI/URL: | |
| Notes: | Review -- Source: Scopus |
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