Lymph nodes - not lymphoma
Inflammatory disorders (noninfectious)
Sinus histiocytosis with massive lymphadenopathy

Topic Completed: 1 June 2006

Revised: 5 February 2019, last major update June 2006

Copyright: (c) 2003-2018,, Inc.

PubMed Search: Sinus histiocytosis with massive lymphadenopathy[TI] lymph node

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Cite this page: DePond W. Sinus histiocytosis with massive lymphadenopathy. website. Accessed January 24th, 2020.
Definition / general
  • Also called Rosai-Dorfman disease
  • Massive painless bilateral lymph node enlargement in neck, of unknown etiology
  • Rare; 25% involve extranodal sites (skin, upper respiratory tract, bone) but massive lymphadenopathy is usually also present
  • Associated with fever, leukocytosis, anemia, elevated sedimentation rate, polyclonal hypergammaglobulinemia
  • Usually age 20 years or less but can affect any age
  • Initial cases from South Africa; now from U.S., Western Europe, Africa and Caribbean
  • May spontaneously resolve; extranodal cases may persist for decades
  • May cause death due to amyloidosis, involvement of a vital organ or immunologic abnormalities
  • May coexist with lymphoma in same node (Mod Pathol 2000;13:414)
Radiology images

Images hosted on PathOut server:

Rosai-Dorfman disease: bone

Case reports
  • None in most cases
Clinical images

Images hosted on PathOut server:

Rosai-Dorfman disease: skin

Microscopic (histologic) description
  • Capsular and pericapsular inflammation and fibrosis
  • Dilated sinuses filled with large histiocytes containing intact lymphocytes (emperipolesis, lymphocytophagocytosis) and with extremely large, round, vesicular nuclei with a delicate nuclear membrane and prominent nucleoli
  • Histiocytes may exhibit atypia and be multinucleated
  • Polyclonal plasma cells and lymphocytes
  • Plasma cells frequently surround prominent high endothelial venules
  • Remaining node contains plasma cells with Russell bodies
  • No / rare internodal fibrosis
  • Distinctive features are emperipolesis, plasma cells, distention of nodal sinuses
Microscopic (histologic) images

Scroll to see all images:

Images hosted on PathOut server:

Contributed by Dr. Mark R. Wick:

Rosai-Dorfman disease


AFIP images:

Sinus histiocytosis with massive lymphadenopathy

Sinus histiocytosis with massive lymphadenopathy

Sinus histiocytosis with massive lymphadenopathy

Touch imprint of lymph node

Rosai-Dorfman disease: extranodal

Rosai-Dorfman disease: eye

Sinus histiocytosis
with massive

Rosai-Dorfman disease: extranodal S100

Images hosted on other servers:

Histiocytes fill a nodal sinus

Extensive replacement
of nodal architecture
by pale staining cells

Distended sinus contains lymphocytes with ingested lymphocytes

Thyroid nodule

18 year old man with enlarged lymph node, which was excised



Cytology description
  • Histiocytes with large nuclei and emperipolesis
  • Lymphocytes are within cytoplasmic vacuoles
  • Histiocytes may have atypia; also reactive lymphocytes (Diagn Cytopathol 2001;24:42)
Positive stains
Negative stains
Molecular / cytogenetics description
  • Not clonal
Differential diagnosis
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