gms | German Medical Science

60th Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Benelux countries and Bulgaria

German Society of Neurosurgery (DGNC)

24 - 27 May 2009, Münster

Isolated intracranial localization of the Rosai-Dorfman disease

Meeting Abstract

  • A. Heckel - Neurochirurgische Klinik der Universität Ulm, Bezirkskrankenhaus Günzburg
  • A. Scheuerle - Pathologische Abteilung der Universität Ulm am BKH Günzburg
  • C.R. Wirtz - Neurochirurgische Klinik der Universität Ulm, Bezirkskrankenhaus Günzburg
  • G. Antoniadis - Neurochirurgische Klinik der Universität Ulm, Bezirkskrankenhaus Günzburg

Deutsche Gesellschaft für Neurochirurgie. 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien. Münster, 24.-27.05.2009. Düsseldorf: German Medical Science GMS Publishing House; 2009. DocP16-06

doi: 10.3205/09dgnc427, urn:nbn:de:0183-09dgnc4275

Published: May 20, 2009

© 2009 Heckel et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: Sinus histiocytosis with massive lymphadenopathy is a synonym for the Rosai-Dorfman disease. It describes a benign, non Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. This case presents a rare isolated extranodal involvement of the central nervous system in the context of this disease. So far over fifty cases have been reported.

Methods: A thirty-nine year-old male had been suffering from retroorbital headache for four months, additionally from left-sided hypoacusis as well as from hypoesthesia in the region of the left tongue, palate, and lip. A cranial MRI showed a contrast enhancing hyperdense, meningioma-like petroclival tumor with a spatial extension from the foramen magnum to the optic chiasm with compression of the brain stem. Besides, two further supratentorial lesions were found. The radiological preoperative diagnosis was a petroclival meningioma.

Results: With the suspected diagnosis of meningioma the patient underwent surgery with total mass resection. According to the first histopathology the tumor was described as a lymphoplasmocytic meningioma WHO I°. Reference pathology on the basis of a recurrence after eighteen months initially stated a chronic reactive inflammation and in the end came up with the diagnosis of a non-Langerhans-cell histiocytosis of the Rosai-Dorfman type. It showed a dense infiltrate of lymphocytes, plasma cells, and histiocytes. Neither eosinophilic granulomas nor Langerhans giant cells were seen. The patient underwent a staging for other typical localizations like the lymph nodes, the skin, the upper respiratory tract or the orbit, but without any findings. A standard therapeutic concept does not exist, but after initial surgery corticosteroids, antimetabolites like vinblastin or methotrexate, and interferon alpha may be applied. The latest drug under investigation is imatinib.

Conclusions: Rosai-Dorfman histiocytosis belongs to the rheumatic disease spectrum and is altogether rarely seen, especially in this isolated intracranial variant. Its ability to radiologically mimic meningioma should put this disease into the list of differential diagnoses.