Ann Hematol DOI 10.1007/s00277-013-1994-8
ORIGINAL ARTICLE
Meningeal Rosai–Dorfman disease mimicking meningioma Fabien Forest & Anh Tuan N’Guyen & Jacques Fesselet & Philippe Metellus & Corinne Bouvier & André Maues de Paula & Pierre-Hughes Roche & Dominique Figarella-Branger
Received: 25 February 2013 / Accepted: 6 December 2013 # Springer-Verlag Berlin Heidelberg 2013
Abstract Rosai–Dorfman disease of the central nervous system is extremely rare and difficult to diagnose also for pathologists. We describe three unusual cases of meningeal Rosai– Dorfman disease and illustrate the difficulties of preoperative and pathological diagnosis. We retrospectively analyzed three patients who underwent surgery for a suspected meningioma for whom the final diagnosis was Rosai–Dorfman disease of the central nervous system. Pathological initial diagnosis was schwannoma, lymphoplasmacyte-rich meningioma, or inflammatory tumor, but final diagnosis in all cases was Rosai–Dorfman disease. These cases underline the preoperative and pathological difficulties of such diagnosis. Pathologists and physicians should be aware of the occurrence of such rare localization of this disease and should think about this differential diagnosis in lymphocyte-rich meningeal tumors F. Forest : C. Bouvier : A. M. de Paula : D. Figarella-Branger Department of Pathology and Neuropathology, Timone Hospital, 264 rue Saint Pierre, 18285 CEDEX 5 Marseille, France A. T. N’Guyen Department of Pathology, Saint Anne Military Hospital, BP 20545, 83041 CEDEX 9 Toulon, France J. Fesselet Department of Neurosurgery, Saint Anne Military Hospital, BP 20545, 83041 CEDEX 9 Toulon, France P. Metellus Department of Neurosurgery, Timone Hospital, 264 rue Saint Pierre, 13385 CEDEX 5 Marseille, France
mimicking, clinically and radiologically, a meningioma. Communication of significant previous medical history to pathologists and careful examination of slides with appropriate medical history and the use of S100 antibody in the diagnosis of meningeal tumors mimicking Rosai–Dorfman disease could lower the rate of misdiagnosis. Keywords Destombes Rosai–Dorfman syndrome . Meningioma . Meningeal neoplasms . Orbital neoplasm . Non-Langerhans-cell histiocytosis
Introduction Since the initial description of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease, RDD) on lymph nodes and the identification of this new entity by Rosai and Dorfman, various extranodal localizations have been described [1, 2]. A rate of 43 % for extranodal manifestations has been reported, but neurological localization remains rare [3]. The most commonly implicated site in the central nervous system is the dura mater, and in this case, clinical, radiological and pathological diagnosis remains a challenge. In this study, we report three new cases of meningeal RDD with clinical and radiological presentations mimicking meningiomas. The aim of this report is to alert both physicians and pathologists of this rare presentation of this disease in order to avoid misdiagnosis of this rarely encountered meningeal lesion.
P.
ORIGINAL ARTICLE
Meningeal Rosai–Dorfman disease mimicking meningioma Fabien Forest & Anh Tuan N’Guyen & Jacques Fesselet & Philippe Metellus & Corinne Bouvier & André Maues de Paula & Pierre-Hughes Roche & Dominique Figarella-Branger
Received: 25 February 2013 / Accepted: 6 December 2013 # Springer-Verlag Berlin Heidelberg 2013
Abstract Rosai–Dorfman disease of the central nervous system is extremely rare and difficult to diagnose also for pathologists. We describe three unusual cases of meningeal Rosai– Dorfman disease and illustrate the difficulties of preoperative and pathological diagnosis. We retrospectively analyzed three patients who underwent surgery for a suspected meningioma for whom the final diagnosis was Rosai–Dorfman disease of the central nervous system. Pathological initial diagnosis was schwannoma, lymphoplasmacyte-rich meningioma, or inflammatory tumor, but final diagnosis in all cases was Rosai–Dorfman disease. These cases underline the preoperative and pathological difficulties of such diagnosis. Pathologists and physicians should be aware of the occurrence of such rare localization of this disease and should think about this differential diagnosis in lymphocyte-rich meningeal tumors F. Forest : C. Bouvier : A. M. de Paula : D. Figarella-Branger Department of Pathology and Neuropathology, Timone Hospital, 264 rue Saint Pierre, 18285 CEDEX 5 Marseille, France A. T. N’Guyen Department of Pathology, Saint Anne Military Hospital, BP 20545, 83041 CEDEX 9 Toulon, France J. Fesselet Department of Neurosurgery, Saint Anne Military Hospital, BP 20545, 83041 CEDEX 9 Toulon, France P. Metellus Department of Neurosurgery, Timone Hospital, 264 rue Saint Pierre, 13385 CEDEX 5 Marseille, France
mimicking, clinically and radiologically, a meningioma. Communication of significant previous medical history to pathologists and careful examination of slides with appropriate medical history and the use of S100 antibody in the diagnosis of meningeal tumors mimicking Rosai–Dorfman disease could lower the rate of misdiagnosis. Keywords Destombes Rosai–Dorfman syndrome . Meningioma . Meningeal neoplasms . Orbital neoplasm . Non-Langerhans-cell histiocytosis
Introduction Since the initial description of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease, RDD) on lymph nodes and the identification of this new entity by Rosai and Dorfman, various extranodal localizations have been described [1, 2]. A rate of 43 % for extranodal manifestations has been reported, but neurological localization remains rare [3]. The most commonly implicated site in the central nervous system is the dura mater, and in this case, clinical, radiological and pathological diagnosis remains a challenge. In this study, we report three new cases of meningeal RDD with clinical and radiological presentations mimicking meningiomas. The aim of this report is to alert both physicians and pathologists of this rare presentation of this disease in order to avoid misdiagnosis of this rarely encountered meningeal lesion.
P.
