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Presented by Ralph Hruban, M.D. and prepared by Shien Micchelli, M.D.
Case 2: This adult patient was incidentally found to have lymphadenopathy.
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Question 1 of 1
1. Question
Week 275: Case 2
This adult patient was incidentally found to have lymphadenopathy.images/7_10_06_2a.jpg
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images/7_10_06_2e.jpgCorrect
Answer: Rosai-Dorfman disease
Histology: The sinus of this lymph node is effaced by histiocytic cells with vesicular nuclei. Focal emperiopolesis was noted.
Discussion: Rosai-Dorfman Disease, also known as sinus histiocytosis with massive lymphadenopathy, occurs in men more commonly than in women. While it can occur at any age, most are < 20 years old. The classical presentation is bilateral cervical lymphadeopathy, but 40% of Rosai-Dorfman Disease is extra-nodal. The patients often have a polyclonal hypergammaglobulinemia. By light microscopy the lymph nodes characteristically have a thickened capsule and the sinuses are dilated by histocyte-like cells with vesicular nuclei. Emperiopolesis (the presence of lymphocytes with the cytoplasm of cells) is a characteristic feature. As in Langerhans cell granulomatosis, the cells label for S-100 protein. In contrast to Langerhans cell granulomatosis, however, the cells in Rosai-Dorfman Disease are CD-1 negative.
Incorrect
Answer: Rosai-Dorfman disease
Histology: The sinus of this lymph node is effaced by histiocytic cells with vesicular nuclei. Focal emperiopolesis was noted.
Discussion: Rosai-Dorfman Disease, also known as sinus histiocytosis with massive lymphadenopathy, occurs in men more commonly than in women. While it can occur at any age, most are < 20 years old. The classical presentation is bilateral cervical lymphadeopathy, but 40% of Rosai-Dorfman Disease is extra-nodal. The patients often have a polyclonal hypergammaglobulinemia. By light microscopy the lymph nodes characteristically have a thickened capsule and the sinuses are dilated by histocyte-like cells with vesicular nuclei. Emperiopolesis (the presence of lymphocytes with the cytoplasm of cells) is a characteristic feature. As in Langerhans cell granulomatosis, the cells label for S-100 protein. In contrast to Langerhans cell granulomatosis, however, the cells in Rosai-Dorfman Disease are CD-1 negative.
