Christopher S Hong1, Robert M Starke2, Michael A Hays3, James W Mandell3, David Schiff4, Ashok R Asthagiri5. 1. College of Medicine, The Ohio State University, Columbus, Ohio, USA. 2. Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia, USA. Electronic address: bobby.starke@gmail.com. 3. Department of Pathology, University of Virginia, Charlottesville, Virginia, USA. 4. Department of Neurology, University of Virginia, Charlottesville, Virginia, USA. 5. Department of Neurological Surgery, University of Virginia, Charlottesville, Virginia, USA.
Abstract
BACKGROUND: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare condition, classically characterized by painless, massive cervical lymphadenopathy. Histologically, the pathognomonic findings include a dense, mixed inflammatory infiltrate with areas of emperipolesis. Albeit infrequent, when Rosai-Dorfman disease affects the central nervous system, it typically manifests as an isolated dural lesion, often mimicking a meningioma. A purely intraparenchymal manifestation of Rosai-Dorfman disease of the brain and spine with absent dural involvement is exceedingly rare. CASE DESCRIPTION: In this report, we describe a 59-year-old woman who underwent surgical excision of an intraparenchymal cerebellar lesion. Histologic analysis of the resected specimen diagnosed isolated Rosai-Dorfman disease with absent systemic involvement. We also provide an updated review of the literature of nondural-based Rosai-Dorfman disease in the central nervous system. CONCLUSIONS: With the recent increase of such reported cases, it becomes imperative that Rosai-Dorfman be considered more than as a dural lesion that may mimic meningioma. Diagnostic and therapeutic challenges surrounding this disease entity are also discussed.
BACKGROUND: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare condition, classically characterized by painless, massive cervical lymphadenopathy. Histologically, the pathognomonic findings include a dense, mixed inflammatory infiltrate with areas of emperipolesis. Albeit infrequent, when Rosai-Dorfman disease affects the central nervous system, it typically manifests as an isolated dural lesion, often mimicking a meningioma. A purely intraparenchymal manifestation of Rosai-Dorfman disease of the brain and spine with absent dural involvement is exceedingly rare. CASE DESCRIPTION: In this report, we describe a 59-year-old woman who underwent surgical excision of an intraparenchymal cerebellar lesion. Histologic analysis of the resected specimen diagnosed isolated Rosai-Dorfman disease with absent systemic involvement. We also provide an updated review of the literature of nondural-based Rosai-Dorfman disease in the central nervous system. CONCLUSIONS: With the recent increase of such reported cases, it becomes imperative that Rosai-Dorfman be considered more than as a dural lesion that may mimic meningioma. Diagnostic and therapeutic challenges surrounding this disease entity are also discussed.
Authors: Ruham Alshiekh Nasany; Anne S Reiner; Jasmine H Francis; Oussama Abla; Katherine S Panageas; Eli L Diamond Journal: Orphanet J Rare Dis Date: 2022-03-02 Impact factor: 4.123
Authors: Rafael Trindade Tatit; Paulo Eduardo Albuquerque Zito Raffa; Giovana Cassia de Almeida Motta; André Alexandre Bocchi; Júlia Loripe Guimaraes; Paulo Roberto Franceschini; Paulo Henrique Pires de Aguiar Journal: Surg Neurol Int Date: 2021-06-21