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Presented by Jonathan Epstein, M.D. and prepared by Angelique W. Levi, M.D.
Case 6: 66-year-old female with a right upper arm soft tissue mass.
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Week 10: Case 6
66-year-old female with a right upper arm soft tissue mass. An immunostain for S-100 protein is included below.Correct
Answer: Rosai-Dorfman disease
Histology: The lesion consists of an inflammatory process involving adipose tissue and collagen. The inflammatory cell component is predominantly composed of small mature appearing lymphocytes admixed with plasma cells. In areas, the lymphoplasmacytic infiltrate demonstrates a perivascular distribution. There is also clustering of lymphocytes which at higher magnification appear to represent histiocytes which have engulfed lymphocytes (emperipolesis). This finding is highlighted in an S-100 stain.
Discussion: Small lymphocytic lymphoma would show less of a polymorphous appearance and would uncommonly present as a soft tissue mass. Similarly, this would be an unusual mode of presentation for Hodgkin’s lymphoma and no cells suggestive of Reed-Sternberg cells were identified. Inflammatory malignant fibrous histiocytomas may show a prominent inflammatory component often containing neutrophils. However, inflammatory malignant fibrous histiocytomas are usually overtly malignant with prominent pleomorphism and increased mitoses. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) most typically occurs in lymph nodes and in other organs. However, it may uncommonly occur in soft tissue. Emperipolesis tends to be less conspicuous in soft tissue sites as compared to lymph nodes. Diagnosis can be confirmed in that the majority of cases will show S-100 protein and histiocytic differentiation within the characteristic histiocytes. Typically, Rosai-Dorfman disease pursues a benign course although it may be associated with immunologic abnormalities. Treatment tends to be empirical and usually directed towards palliation of symptoms. In cases where the disease compresses vital structures it may be necessary for either surgery or radiation to alleviate symptoms.Incorrect