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ENT · Head and neck tumours: Rosai-Dorfman disease
Sample itemA 12-year-old boy has massive painless bilateral cervical lymphadenopathy with fever and a raised ESR. Node biopsy shows sheets of S100-positive histiocytes engulfing intact lymphocytes. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Rosai-Dorfman disease
Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) presents with huge painless bilateral cervical nodes. Histiocytes show emperipolesis, are S100 positive and CD1a negative. Langerhans cell histiocytosis is CD1a positive.
Option by option
A. Hodgkin lymphoma
Hodgkin lymphoma shows Reed-Sternberg cells in a mixed infiltrate.
B. Rosai-Dorfman disease (correct)
Correct. Emperipolesis by S100-positive histiocytes in a boy with massive cervical nodes is Rosai-Dorfman disease.
C. Tuberculous lymphadenitis
Tuberculous lymphadenitis shows caseating granulomas with matted nodes.
D. Langerhans cell histiocytosis
Langerhans cell histiocytosis shows grooved nuclei, CD1a positivity and Birbeck granules.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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