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Aggressive sporadic histiocytic sarcoma with immunoglobulin heavy chain gene rearrangement and t(14;18)

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Abstract

Histiocytic sarcoma (HS) is a rare but aggressive malignant neoplasm of histiocytic lineage with a poor prognosis. Immunohistochemically, the neoplastic cells are positive for CD163, CD68, and lysozyme, and negative for B and T cell markers. However, molecular studies on the origin of the neoplastic cells remain inconclusive. A 54-year-old woman was admitted to our hospital because of painful swelling of the left knee. Examination revealed generalized lymphadenopathy and splenomegaly. HS was diagnosed according to morphologic and immunohistochemical features observed on biopsy of the left inguinal lymph node. The tumor demonstrated a clonal immunoglobulin heavy chain gene rearrangement and a clonal cytogenetic abnormality including t(14;18) which was confirmed by fluorescence in situ hybridization analysis showing the IgH/BCL2 fusion gene. The neoplastic cells were negative for PAX5, a B cell associated transcription factor, and positive for CEBPβ, a transcription factor mediating macrophage and myeloid differentiation. Positron emission tomography showed disseminated areas of increased 18F-fluorodeoxyglucose uptake in multiple lymph nodes, the liver, spleen, both lungs, both kidneys, and many bony sites. The patient received localized irradiation therapy followed by chemotherapy, she failed to respond and died of the disease progression. The case findings suggest lineage promiscuity or plasticity related to the pathogenesis of HS.

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Acknowledgments

We would like to thank Dr. Shigeo Nakamura, Professor of Department of Pathology and Clinical Laboratories, Nagoya University School of Medicine, for the pathological diagnosis.

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Authors and Affiliations

  1. Department of Hematology, National Hospital Organization Hokkaido Cancer Center, 4-2-3-54 Kikusui, Shiroishi-ku, Sapporo, 003-0804, Japan

    Eiko Hayase, Mitsutoshi Kurosawa, Masakatsu Yonezumi & Sachiko Suzuki

  2. Department of Diagnostic Pathology, National Hospital Organization Hokkaido Cancer Center, 4-2-3-54 Kikusui, Shiroishi-ku, Sapporo, 003-0804, Japan

    Hiroaki Suzuki

Authors
  1. Eiko Hayase
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  2. Mitsutoshi Kurosawa
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  3. Masakatsu Yonezumi
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  4. Sachiko Suzuki
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  5. Hiroaki Suzuki
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Correspondence to Eiko Hayase.

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Hayase, E., Kurosawa, M., Yonezumi, M. et al. Aggressive sporadic histiocytic sarcoma with immunoglobulin heavy chain gene rearrangement and t(14;18). Int J Hematol 92, 659–663 (2010). https://doi.org/10.1007/s12185-010-0704-8

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  • DOI: https://doi.org/10.1007/s12185-010-0704-8

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