Sinus Histiocytosis with Massive Lymphadenopathy
Definition
-
Predominantly sinusoidal proliferation of S100 positive large histiocytic cells
Alternate / historical names
- Rosai-Dorfman Disease
- Juan-Ron Fever
Diagnostic Criteria
- Capsular thickening with fibrosis and distended sinuses
- Occasional cases with prominent germinal centers
- Microabscesses may be seen in the sinuses
- Large histiocytes with abundant cytoplasm
- S100 positive
- Predominantly intra-sinusoidal
- Large, round or oval vesicular nuclei
- Prominent nucleoli, single or multiple
- May show moderate atypia or multiple nuclei
- Emperipolesis prominent in nodes
- Intact, engulfed lymphocytes and plasma cells may be numerous
- Occasional engulfed neutrophils and RBCs
- Less prominent in extranodal disease
- Plasma cells prominent in medullary cords or adjacent soft tissue in extranodal cases
- May surround prominent vessels
- Extranodal cases may have less prominent sinuses
- Emperipolesis may be more difficult to find
- Stromal fibrosis, germinal centers and prominent plasma cells should prompt S100 stain and close search for characteristic histiocytes
- SHML has rarely been reported to coexist in the same node with lymphoma
- Occasional cases with prominent germinal centers
- Microabscesses may be seen in the sinuses
- S100 positive
- Predominantly intra-sinusoidal
- Large, round or oval vesicular nuclei
- Prominent nucleoli, single or multiple
- May show moderate atypia or multiple nuclei
- Intact, engulfed lymphocytes and plasma cells may be numerous
- Occasional engulfed neutrophils and RBCs
- Less prominent in extranodal disease
- May surround prominent vessels
- Emperipolesis may be more difficult to find
- Stromal fibrosis, germinal centers and prominent plasma cells should prompt S100 stain and close search for characteristic histiocytes
Yasodha Natkunam MD PhD
Ronald F Dorfman MBBCh FRCPath
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting:: May 1, 2006
