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Presented by Dr. Cimino-Mathews and prepared by Dr. Armen Khararjian.
This case talks about:
A 30 year-old female presents with bilateral axillary masses
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1. Question
Week 632: Case 2
A 30 year-old female presents with bilateral axillary masses.
Correct
Answer: D. Rosai-Dorfman disease
Histology: The core needle biopsy of the axillary mass reveals primarily adipose tissue with nodular aggregates of chronic inflammatory cells including lymphocytes, plasma cells and histiocytes. No granulomas or acute inflammation are identified. No epithelial elements are identified. No distinct lymph node architecture is identified. Close examination reveals that there are scattered large histiocytes with abundant cytoplasm with intra-cytoplasmic, intact lymphocytes (emperipolesis). Immunostain for the S-100 protein and CD68 are positive in the histiocytes and further highlight the emperipolesis.
Discussion: The patient’s core needle biopsies reveal bilateral, extranodal Rosai-Dorfman disease; this disorder is also called “sinus histiocytosis with mass lymphadenopathy” when occurring within the lymph nodes, because as the name suggests, involved lymph nodes display dilated sinuses and enlargement! Rosai-Dorfman disease is presumed to be a reactive, non-clonal histiocytosis characterized the presence of histiocytes with emperopolesis (that is, the presence of intact, normal-appearing lymphocytes within the cytoplasm of the histiocytes). The histiocytes in Rosai-Dorfman disease are immunoreactive for both CD68 and S-100 protein, which is a characteristic features because non-Rosai-Dorfman histiocytes are CD68+ but negative for S100 protein. The histiocytes in Rosai-Dorman disease are also negative for CD1a (a marker of Langerhans cell histiocytosis) and CD21 (a marker of dendritic cell neoplasms). Extranodal Rosai-Dorfman disease can essentially involve any area of the body and has been reported in both the axilla and breast.
References
1. Eisen RH, Buckley PF, Rosai J. Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 1990;7:74-82.
2. Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am J Surg Pathol. 1997 Jun;21(6):664-8.
3. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
4. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer 1972;30:1174-88.Incorrect
Answer: D. Rosai-Dorfman disease
Histology: The core needle biopsy of the axillary mass reveals primarily adipose tissue with nodular aggregates of chronic inflammatory cells including lymphocytes, plasma cells and histiocytes. No granulomas or acute inflammation are identified. No epithelial elements are identified. No distinct lymph node architecture is identified. Close examination reveals that there are scattered large histiocytes with abundant cytoplasm with intra-cytoplasmic, intact lymphocytes (emperipolesis). Immunostain for the S-100 protein and CD68 are positive in the histiocytes and further highlight the emperipolesis.
Discussion: The patient’s core needle biopsies reveal bilateral, extranodal Rosai-Dorfman disease; this disorder is also called “sinus histiocytosis with mass lymphadenopathy” when occurring within the lymph nodes, because as the name suggests, involved lymph nodes display dilated sinuses and enlargement! Rosai-Dorfman disease is presumed to be a reactive, non-clonal histiocytosis characterized the presence of histiocytes with emperopolesis (that is, the presence of intact, normal-appearing lymphocytes within the cytoplasm of the histiocytes). The histiocytes in Rosai-Dorfman disease are immunoreactive for both CD68 and S-100 protein, which is a characteristic features because non-Rosai-Dorfman histiocytes are CD68+ but negative for S100 protein. The histiocytes in Rosai-Dorman disease are also negative for CD1a (a marker of Langerhans cell histiocytosis) and CD21 (a marker of dendritic cell neoplasms). Extranodal Rosai-Dorfman disease can essentially involve any area of the body and has been reported in both the axilla and breast.
References
1. Eisen RH, Buckley PF, Rosai J. Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 1990;7:74-82.
2. Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am J Surg Pathol. 1997 Jun;21(6):664-8.
3. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
4. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer 1972;30:1174-88.
