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CD56/NCAM-Positive Langerhans Cell Sarcoma: A Clinicopathologic Study of 4 Cases

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Abstract

This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive Langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100β, and langerin, the presence of which suggests derivation from Langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.

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Authors and Affiliations

  1. Departments of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya

    Takakazu Kawase, Michinori Ogura, Hirofumi Taji, Yoshitoyo Kagami & Yasuo Morishima

  2. Department of Pathology, Shizuoka Children’s Hospital, Shizuoka

    Minoru Hamazaki

  3. Kitami Red Cross Hospital, Hokkaido

    Yoshiaki Kawase

  4. Kumamoto National Hospital, Kumamoto

    Toshihiko Murayama

  5. Nagoya National Hospital, Nagoya

    Yoshio Mori

  6. Department of Pathology, Asahikawa Medical College, Hokkaido

    Hirokazu Nagai & Masatoshi Tateno

  7. Departments of Clinical Oncology, Aichi Cancer Center, Nagoya

    Takashi Oyama & Yoshikazu Kamiya

  8. First Department of Internal Medicine, Nagoya University School of Medicine, Nagoya, Japan

    Tomoki Naoe

  9. Division of Immunology, Aichi Cancer Center, Nagoya

    Toshitada Takahashi

  10. Department of Pathology and Molecular Diagnostics, Aichi Cancer Center, 1-1 Kanokoden, Chikusa-ku, 464-8681, Nagoya, Japan

    Shigeo Nakamura

Authors
  1. Takakazu Kawase
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  2. Minoru Hamazaki
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Correspondence to Shigeo Nakamura.

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Kawase, T., Hamazaki, M., Ogura, M. et al. CD56/NCAM-Positive Langerhans Cell Sarcoma: A Clinicopathologic Study of 4 Cases. Int J Hematol 81, 323–329 (2005). https://doi.org/10.1532/IJH97.04142

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  • DOI: https://doi.org/10.1532/IJH97.04142

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