2011
DOI: 10.4103/0028-3886.82770
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Isolated intracranial Rosai Dorfman disease

Abstract: Rosai Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder mainly affecting the lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. We report a case of isolated intracranial RDD occurring in a relatively elder patient, which was shown by histological examination to have a dura-based involvem… Show more

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Cited by 12 publications
(9 citation statements)
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“…Microscopically, lymph nodes show dilated sinuses containing foamy histiocytes and plasma cells. Many of these histiocytes contain intact hematopoietic cells, mostly lymphocytes within their cytoplasm, a phenomenon known as emperipolesis 2,3,9 . These histiocytes are positive for S-100 protein and CD-68 and negative for CD1a based on immunohistochemical examination 2,7,10 .…”
Section: Discussionmentioning
confidence: 99%
“…Microscopically, lymph nodes show dilated sinuses containing foamy histiocytes and plasma cells. Many of these histiocytes contain intact hematopoietic cells, mostly lymphocytes within their cytoplasm, a phenomenon known as emperipolesis 2,3,9 . These histiocytes are positive for S-100 protein and CD-68 and negative for CD1a based on immunohistochemical examination 2,7,10 .…”
Section: Discussionmentioning
confidence: 99%
“…It usually presents as bilateral painless cervical lymphadenopathy. Intracranial RDD occurs in less than 5% of the cases and the dura is the most common site of involvement (5,9). Systemic symptoms may be absent in isolated intracranial disease.…”
Section: Discussionmentioning
confidence: 99%
“…Extranodal involvement occurs with lymphadenopathy in 43% of the cases. Isolated extranodal disease occurs in about 23% of the cases and affects skin (12%), paranasal sinuses (11%), soft tissues (9%), bone (9%), salivary glands (5%), central nervous system (5%), oral cavity (3%), kidney (2%), lower respiratory tract (2%), larynx (1%) and orbit (5-10%) (5,8,9).…”
Section: Introductionmentioning
confidence: 99%
“…Isolated central nervous system (CNS) involvement is extremely rare (2,6,4,10,13,15, [17][18][19][21][22][23]28,33,35,36,38,46). In a large series of RDD including 200 cases there were only 3 cases with intracranial manifestations (9).…”
Section: Discussionmentioning
confidence: 99%