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Presented by Ralph Hruban, M.D. and prepared by Whitney Green, M.D.
Case 3: This large solid mass was identified in the tail of the pancreas of a 60 some year old man.
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1. Question
Week 576: Case 3
This large solid mass was identified in the tail of the pancreas of a 60 some year old man.images/Case3_image1RH.jpg
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images/Case3_image5RH.jpgCorrect
Answer: Rosai-Dorfman disease
Histology: The mass lesion is composed of numerous foamy histiocytes, a mixed chronic inflammation, and fibrosis. No significant atypia is appreciated. Focal emperipolesis is noted. The cells are immunoreactive for S-100 protein and CD68.
Discussion: Rosai-Dorfman disease, also known as massive lymphadenopathy with sinus histiocytosis, is characterized by emperipolesis. Emperipolesis is the presence of an intact cell within the cytoplasm of another intact cell. The word is Greek in origin. Em=inside, Peri=around, and Polemai=wander about. Although first recognized in lymph nodes, a growing number of examples of extra-nodal Rosai-Dorfman disease have been reported. Several recent reports have linked Rosai-Dorfman disease to IgG4+ related diseases.
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Answer: Rosai-Dorfman disease
Histology: The mass lesion is composed of numerous foamy histiocytes, a mixed chronic inflammation, and fibrosis. No significant atypia is appreciated. Focal emperipolesis is noted. The cells are immunoreactive for S-100 protein and CD68.
Discussion: Rosai-Dorfman disease, also known as massive lymphadenopathy with sinus histiocytosis, is characterized by emperipolesis. Emperipolesis is the presence of an intact cell within the cytoplasm of another intact cell. The word is Greek in origin. Em=inside, Peri=around, and Polemai=wander about. Although first recognized in lymph nodes, a growing number of examples of extra-nodal Rosai-Dorfman disease have been reported. Several recent reports have linked Rosai-Dorfman disease to IgG4+ related diseases.
