Histiocytic Sarcoma: Review, Discussion of Transformation From B-Cell Lymphoma, and Differential Diagnosis

<jats:sec><jats:title>Context.—</jats:title><jats:p>Histiocytic sarcoma is a rare neoplasm of mature histiocytes with an aggressive clinical course that can arise de novo or from a low-grade B-cell lymphoma. In particular, chronic lymphocytic leukemia/small lymphocytic lymphoma is a very common malignancy in the Western hemisphere, and most cases of chronic lymphocytic leukemia/small lymphocytic lymphoma have an indolent course and behavior. However, 2% to 8% of chronic lymphocytic leukemia/small lymphocytic lymphoma cases transform. Histiocytic sarcomatous transformation is rare and portends poor prognosis.</jats:p></jats:sec><jats:sec><jats:title>Objective.—</jats:title><jats:p>To review the clinical features, morphology, and key points related to the differential diagnosis for histiocytic sarcoma. We discuss recent understanding of the biology underlying transformation.</jats:p></jats:sec><jats:sec><jats:title>Data Sources.—</jats:title><jats:p>University of Michigan case and review of pertinent literature about histiocytic sarcoma and morphologic differential diagnosis.</jats:p></jats:sec><jats:sec><jats:title>Conclusions.—</jats:title><jats:p>Histiocytic sarcoma is a rare histiocytic neoplasm that can arise as a result of transdifferentiation from low-grade B-cell lymphomas, and has a wide differential diagnosis including other histiocytic/dendritic cell neoplasms, myeloid neoplasms, lymphomas, melanoma, and carcinoma. However, some key morphologic and immunohistochemical features allow for accurate classification.</jats:p></jats:sec>