Central Nervous System Histiocytic and Dendritic Cell Neoplasm

MedGen UID:: 457583
•Concept ID:: C2985357
•: Neoplastic Process

Definition

A neoplasm that originates from histiocytes and accessory cells and affects the central nervous system. It is usually associated with the presence of identical tumors outside the central nervous system. Representative examples include Langerhans cell histiocytosis, histiocytic sarcoma, and follicular dendritic cell sarcoma. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCentral Nervous System Histiocytic and Dendritic Cell Neoplasm

Disease
- Disorder by Site
  - Hematopoietic and Lymphoid System Disorder
    - Hematologic disorder
      - Hematopoietic and lymphoid cell neoplasm
        Central nervous system hematopoietic neoplasm
        Central Nervous System Histiocytic and Dendritic Cell Neoplasm
        Central Nervous System Erdheim-Chester Disease
        Central Nervous System Histiocytic Sarcoma
        Central Nervous System Langerhans Cell Histiocytosis

Recent clinical studies

Etiology

An Analysis of the Pathologic Features of Blastic Plasmacytoid Dendritic Cell Neoplasm Based on a Comprehensive Literature Database of Cases.

Ohgami RS, Aung PP, Gru AA, Hussaini M, Singh K, Querfeld C, Yao K, Small C, Gollapudi S, Jaye D, Wang SA, Pullarkat S, George TI
Arch Pathol Lab Med 2023 Jul 1;147(7):837-846. doi: 10.5858/arpa.2021-0612-RA. PMID: 36170615

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Diagnosis

An Analysis of the Pathologic Features of Blastic Plasmacytoid Dendritic Cell Neoplasm Based on a Comprehensive Literature Database of Cases.

Primary histiocytic sarcoma of the clivus with focal extension into central nervous system and neurologic manifestations: First description at an unusual site with an overwhelming and rapid progression.

Perez I, Gokden M, Day JD, Yaziji H, Pina-Oviedo S
Clin Neuropathol 2022 Mar-Apr;41(2):74-82. doi: 10.5414/NP301400. PMID: 34958302

Uncommon histiocytic disorders: Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease.

Haroche J, Abla O
Hematology Am Soc Hematol Educ Program 2015;2015:571-8. doi: 10.1182/asheducation-2015.1.571. PMID: 26637774

Imaging of Nervous System Involvement in Hematologic Malignancies: What Radiologists Need to Know.

Keraliya AR, Krajewski KM, Giardino AA, Tirumani SH, Shinagare AB, Ramaiya NH, Jagannathan JP
AJR Am J Roentgenol 2015 Sep;205(3):604-17. doi: 10.2214/AJR.14.14092. PMID: 26295649

Histiocytic sarcoma involving the central nervous system: clinical, immunohistochemical, and molecular genetic studies of a case with review of the literature.

Sun W, Nordberg ML, Fowler MR
Am J Surg Pathol 2003 Feb;27(2):258-65. doi: 10.1097/00000478-200302000-00017. PMID: 12548175

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Therapy

Primary histiocytic sarcoma of the brain mimicking cerebral abscess.

Almefty RO, Tyree TL, Fusco DJ, Coons SW, Nakaji P
J Neurosurg Pediatr 2013 Sep;12(3):251-7. Epub 2013 Jul 26 doi: 10.3171/2013.6.PEDS12533. PMID: 23889356

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