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Meningeal Rosai-Dorfman disease mimicking meningioma

Fabien Forest1, Anh Tuan N'guyen, Jacques Fesselet

  • 1Department of Pathology and Neuropathology, Timone Hospital, 264 rue Saint Pierre, 18285 CEDEX 5, Marseille, France.

Annals of Hematology
|December 25, 2013
PubMed

Insights

Rosai-Dorfman disease affecting the central nervous system is rare and challenging to diagnose. This study highlights diagnostic difficulties and emphasizes awareness for accurate identification of this uncommon meningeal tumor.

Area of Science:

  • Neuropathology
  • Oncology

Background:

  • Rosai-Dorfman disease (RDD) is a rare histiocytic disorder.
  • Central nervous system (CNS) involvement, particularly meningeal RDD, is exceptionally uncommon.
  • Diagnosis is often complicated by its rarity and mimicry of other meningeal pathologies.

Purpose of the Study:

  • To describe unusual cases of meningeal Rosai-Dorfman disease.
  • To illustrate the diagnostic challenges in preoperative and pathological settings.
  • To raise awareness among pathologists and physicians regarding this rare differential diagnosis.

Main Methods:

  • Retrospective analysis of three patients surgically treated for suspected meningioma.
  • Review of initial and final pathological diagnoses.
  • Histopathological examination and immunohistochemistry (S100 antibody).

Main Results:

  • All three cases were initially misdiagnosed as schwannoma, lymphoplasmacyte-rich meningioma, or inflammatory tumor.
  • Final diagnosis in all cases was confirmed as Rosai-Dorfman disease of the CNS.
  • The study underscores significant preoperative and pathological diagnostic hurdles.

Conclusions:

  • Meningeal Rosai-Dorfman disease presents diagnostic difficulties, mimicking meningiomas clinically and radiologically.
  • Pathologists and clinicians must consider RDD in the differential diagnosis of lymphocyte-rich meningeal tumors.
  • Thorough medical history and S100 antibody use can improve diagnostic accuracy.

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