CNS tumor

Lymphoma & histiocytic tumors

Histiocytic tumors

Topic Completed: 1 January 2006

Minor changes: 7 May 2021

Copyright: 2002-2021,, Inc.

PubMed Search: CNS histiocytic tumors

Nat Pernick, M.D.
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Cite this page: Pernick N. Histiocytic tumors. website. Accessed December 8th, 2021.
Erdheim-Chester disease
    Definition / general:
  • See also Lymph node topic
  • Very rare ( < 100 cases reported), nonfamilial, neoplastic, xanthogranulomatous, non-Langerhans cell systemic histiocytosis first identified by William Chester in 1930
  • Usually associated with disease of long bones
  • Etiology not well understood
  • Mean age 57 years, range 25 - 76 years, no gender preference
  • Three year survival is 50 - 65%
  • Prognosis usually depends on extent of extraosseous disease
  • Rarely involves CNS
  • Usually hypothalamus and posterior pituitary (causing diabetes insipidus), retroorbital space
  • May affect dura
Histiocytic sarcoma
Juvenile xanthogranuloma
Langerhans cell histiocytosis
    Definition / general:
  • See also Lymph node topic
  • Rare lytic skull lesion or parasellar mass
  • Children and young adults
  • May arise from primary histiocytic proliferation with secondary atrophy or from demyelination and gliosis of unknown origin (J Child Neurol 2000;15:150)
Rosai-Dorfman disease
    Definition / general:
  • See also Lymph node topic
  • Also called sinus histiocytosis with massive lymphadenopathy
  • Often (25 - 43%) has extranodal involvement
  • Rare (100 cases/year in US) and isolated CNS findings are extremely rare
  • Benign but may be associated with systemic disease
  • Children and young adults (mean age 41 years, range 22 - 63 years)
  • CNS tumors usually associated with dura
  • References: Mod Pathol 2001;14:172, Clin Neuropathol 2005;24:112 (isolated CNS disease)
Microscopic (histologic) images

Case #488

Rosai-Dorfman disease of CNS

Board review style question #1
    In distinguishing CNS Rosai-Dorfman disease (RDD) from other histiocytoses, what is the correct immunophenotype for RDD?

  1. S100-, CD1a-
  2. S100+, CD1a+
  3. S100+, CD1a-
  4. S100-, CD1a+
Board review style answer #1
C. S100+, CD1a-

Explanation: CNS Rosai-Dorfman disease forms lymphohistiocytic masses with prominent pale S100+, CD1a- histiocytes. Answer A (S100-, CD1a-) is the staining pattern of histiocytes in Erdheim-Chester disease. Answer B (S100+, CD1a+) is the staining pattern of histiocytes in Langerhans cell histiocytosis.

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