Intracranial Erdheim-Chester Disease Mimicking Parafalcine Meningioma: Report of Two Cases and Review of the Literature

World Neurosurg. 2018 Feb:110:365-370. doi: 10.1016/j.wneu.2017.11.074. Epub 2017 Nov 28.

Abstract

Background: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis that typically occurs in middle-aged patients. It is usually characterized by multifocal osteosclerotic lesions of the long-bones, however many cases have extraskeletal involvement. Central nervous system (CNS) involvement is common, but isolated CNS involvement at presentation has rarely been reported.

Case description: Here we report two cases of dural-based ECD mimicking meningioma on imaging with no other identified sites of disease.

Conclusion: ECD is a rare disease, with isolated CNS involvement reported only a few times in the literature. The significance of this presentation requires additional study and long-term follow up.

Keywords: Erdheim-Chester Disease; Intracranial; Parafalcine meningioma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Erdheim-Chester Disease / diagnostic imaging
  • Erdheim-Chester Disease / physiopathology*
  • Female
  • Humans
  • Intralaminar Thalamic Nuclei / diagnostic imaging
  • Magnetic Resonance Imaging
  • Male
  • Meningeal Neoplasms / physiopathology*
  • Meningioma / physiopathology*
  • Middle Aged

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD68 antigen, human