Rosai-dorfman disease of the central nervous system

Report of 6 cases and review of the literature

Jose D. Sandoval-Sus, Ana C. Sandoval-Leon, Jennifer Chapman-Fredricks, Jose Velazquez-Vega, Maria J. Borja, Shai Rosenberg, Alexander Lossos, Izidore Lossos

Research output: Contribution to journalArticle

41 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)165-175
Number of pages11
JournalMedicine (United States)
Volume93
Issue number3
DOIs
StatePublished - Jan 1 2014

Fingerprint

Sinus Histiocytosis
Central Nervous System
Emperipolesis
Histiocytes
Lymphoproliferative Disorders
Spinal Cord
Lymph Nodes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Rosai-dorfman disease of the central nervous system : Report of 6 cases and review of the literature. / Sandoval-Sus, Jose D.; Sandoval-Leon, Ana C.; Chapman-Fredricks, Jennifer; Velazquez-Vega, Jose; Borja, Maria J.; Rosenberg, Shai; Lossos, Alexander; Lossos, Izidore.

In: Medicine (United States), Vol. 93, No. 3, 01.01.2014, p. 165-175.

Research output: Contribution to journalArticle

Sandoval-Sus, Jose D. ; Sandoval-Leon, Ana C. ; Chapman-Fredricks, Jennifer ; Velazquez-Vega, Jose ; Borja, Maria J. ; Rosenberg, Shai ; Lossos, Alexander ; Lossos, Izidore. / Rosai-dorfman disease of the central nervous system : Report of 6 cases and review of the literature. In: Medicine (United States). 2014 ; Vol. 93, No. 3. pp. 165-175.
@article{784e1d433f6c40aca082d7f49cb6f876,
title = "Rosai-dorfman disease of the central nervous system: Report of 6 cases and review of the literature",
abstract = "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.",
author = "Sandoval-Sus, {Jose D.} and Sandoval-Leon, {Ana C.} and Jennifer Chapman-Fredricks and Jose Velazquez-Vega and Borja, {Maria J.} and Shai Rosenberg and Alexander Lossos and Izidore Lossos",
year = "2014",
month = "1",
day = "1",
doi = "10.1097/MD.0000000000000030",
language = "English",
volume = "93",
pages = "165--175",
journal = "Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries",
issn = "0025-7974",
publisher = "Lippincott Williams and Wilkins",
number = "3",

}

TY - JOUR

T1 - Rosai-dorfman disease of the central nervous system

T2 - Report of 6 cases and review of the literature

AU - Sandoval-Sus, Jose D.

AU - Sandoval-Leon, Ana C.

AU - Chapman-Fredricks, Jennifer

AU - Velazquez-Vega, Jose

AU - Borja, Maria J.

AU - Rosenberg, Shai

AU - Lossos, Alexander

AU - Lossos, Izidore

PY - 2014/1/1

Y1 - 2014/1/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84900429715&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84900429715&partnerID=8YFLogxK

U2 - 10.1097/MD.0000000000000030

DO - 10.1097/MD.0000000000000030

M3 - Article

VL - 93

SP - 165

EP - 175

JO - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

JF - Medicine; analytical reviews of general medicine, neurology, psychiatry, dermatology, and pediatries

SN - 0025-7974

IS - 3

ER -