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2014 ; 93
(3
): 165-175
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Rosai-Dorfman disease of the central nervous system: report of 6 cases and review
of the literature
#MMPMID24797172
Sandoval-Sus JD
; Sandoval-Leon AC
; Chapman JR
; Velazquez-Vega J
; Borja MJ
; Rosenberg S
; Lossos A
; Lossos IS
Medicine (Baltimore)
2014[May]; 93
(3
): 165-175
PMID24797172
show ga
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive
lymphadenopathy (SHML), is an uncommon benign idiopathic lymphoproliferative
disorder. The histologic hallmark of RDD is the finding of emperipolesis
displayed by lesional histiocytes. While RDD most commonly affects lymph nodes,
extranodal involvement of multiple organs has been reported, including the
central nervous system (CNS). However, CNS involvement in RDD is rare and is not
well characterized. As a result, therapeutic approaches to CNS involvement in RDD
are not well established. Herein we report 6 cases of RDD with isolated CNS
involvement and review the literature on RDD with CNS involvement. One of the
presented cases exhibited intramedullary involvement of the spinal cord--a very
rare form of RDD with CNS involvement.
|Adult
[MESH]
|Central Nervous System Diseases/*diagnosis/pathology/surgery
[MESH]