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Presented by Dr. Pedram Argani and prepared by Dr. Kevan Salimian
This is an 87 year old male with a retroperitoneum mass.
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1. Question
This is an 87 year old male with a retroperitoneum mass. Choose the correct diagnosis.
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Answer: Well differentiated liposarcoma
Histologic Description: This an inflammation-rich lesion which contains large neoplastic cells with large irregular nucleoli and abundant cytoplasm in a background of predominantly chronic inflammation with lymphocytes and plasma cells. These large cells resemble those of Hodgkin lymphoma or Rosai Dorfman disease. However, in the adjacent fat, one can appreciate occasionally atypical cells which, in the retroperitoneum, raise the differential diagnosis of well differentiated liposarcoma. Immunohistochemistry for MDM2 supports the diagnosis of well differentiated liposarcoma, inflammatory type in this case. Myogenin immunostain did not label these cells; instead, it labeled regenerating skeletal muscle cells. Reference Argani P et al. Am J Surg Pathol 1997; 21:884-895.
Differential Diagnosis: The atypia in the fat surrounding the lesion essentially excludes the differential diagnosis of Hodgkin lymphoma, Rosai Dorfman disease and sclerosing low grade lymphoma. The majority of inflammatory cells in inflammatory well-differentiated liposarcomas are, in our experience T-lymphocytes. The neoplastic cells of Hodgkin disease typically have less abundant cytoplasm, and while emperilopoiesis is often seen in well-differentiated inflammatory liposarcoma, it is less prevalent than in Rosai Dorfman disease.
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Answer: Well differentiated liposarcoma
Histologic Description: This an inflammation-rich lesion which contains large neoplastic cells with large irregular nucleoli and abundant cytoplasm in a background of predominantly chronic inflammation with lymphocytes and plasma cells. These large cells resemble those of Hodgkin lymphoma or Rosai Dorfman disease. However, in the adjacent fat, one can appreciate occasionally atypical cells which, in the retroperitoneum, raise the differential diagnosis of well differentiated liposarcoma. Immunohistochemistry for MDM2 supports the diagnosis of well differentiated liposarcoma, inflammatory type in this case. Myogenin immunostain did not label these cells; instead, it labeled regenerating skeletal muscle cells. Reference Argani P et al. Am J Surg Pathol 1997; 21:884-895.
Differential Diagnosis: The atypia in the fat surrounding the lesion essentially excludes the differential diagnosis of Hodgkin lymphoma, Rosai Dorfman disease and sclerosing low grade lymphoma. The majority of inflammatory cells in inflammatory well-differentiated liposarcomas are, in our experience T-lymphocytes. The neoplastic cells of Hodgkin disease typically have less abundant cytoplasm, and while emperilopoiesis is often seen in well-differentiated inflammatory liposarcoma, it is less prevalent than in Rosai Dorfman disease.
