Lymph nodes & spleen, nonlymphoma
Lymph nodes-inflammatory / reactive disorders
Sinus histiocytosis with massive lymphadenopathy
Author: Nat Pernick, M.D.
Topic Completed: 1 June 2006
Minor changes: 10 September 2020
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Sinus histiocytosis with massive lymphadenopathy[TI] lymph node
Table of Contents
Definition / general | Terminology | Clinical features | Radiology images | Case reports | Treatment | Clinical images | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Pernick N. Sinus histiocytosis with massive lymphadenopathy. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodessinushistiocytosiswithmassivelymph.html. Accessed March 4th, 2021.
Definition / general
- 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
Terminology
- Also called Rosai-Dorfman disease
Clinical features
- 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
AFIP images
Disease in bone
Case reports
-
Breast:
- 43 year old woman with enlarging breast mass (Univ Pittsburgh Case #338)
- 45 year old woman presented with a right breast mass (Case of the Week #302)
- 75 year old woman with benign histiocytosis (Ned Tijdschr Geneeskd 2011;155:A3176)
- 32 year old woman with right hand and wrist weakness and a left parasagittal mass on MRI (Case of the Month #488)
- 38 year old man and 69 year old woman with dural based masses (University of Pittsburgh: Dural Based Masses [Accessed 1 August 2018])
- 48 year old woman with dural based intracranial tumor (Arch Pathol Lab Med 2001;125:1115)
- 50 year old woman with leptomeningeal disease (University of Pittsburgh: Acute Loss of Strength in Right Upper Extremity and Slurred Speech [Accessed 1 August 2018])
- 68 year old woman with isolated dural disease (AJNR Am J Neuroradiol 2003;24:515)
- 78 year old man and 47 year old man with relapsing intracranial disease treated with radiation (J Neurol Neurosurg Psychiatry 2001;71:538)
Lymph nodes:
- 16 month old boy with massive lymphadenopathy (J Clin Pathol 1973;26:463)
- 38 year old woman presenting with midline thyroid mass (Arch Pathol Lab Med 2003;127:e197)
- Associated with Hodgkin disease (Hum Pathol 1995;26:1378)
Treatment
- None in most cases
Clinical images
AFIP images
Skin lesion
Gross images
Contributed by Dr. Semir Vranic
Thigh lesion
Microscopic (histologic) description
-
Lymph node:
- Distinctive features are emperipolesis, plasma cells, distention of nodal sinuses
- 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
Microscopic (histologic) images
AFIP images
Lymph node: H&E and S100
Lymph node
Breast
Extranodal: H&E and S100
Eye
Case #302
45 year old woman with breast mass
Contributed by Dr. Semir Vranic
Subcutaneous right thigh
CD68
S100
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)
Cytology images
AFIP images
Touch imprint of lymph node
Molecular / cytogenetics description
- Not clonal
Differential diagnosis
- Langerhans cell histiocytosis: prominent eosinophils, smaller nuclei with central grooves, indistinct nucleoli, no lymphophagocytosis although may have erythrophagocytosis, CD1a+
- Leprosy
- Metastatic melanoma
- Prosthesis related changes
- Sinus histiocytosis: no emperipolesis, S100-
- Toxoplasmosis: see Leuk Lymphoma 2004;45:1037
