Secondary Malignant Fibrous Histiocytoma Following Refractory Langerhans Cell Histiocytosis

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概要

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• We describe a rare case of secondary malignant fibrous histiocytoma (MFH) following Langerhans cell histiocytosis (LCH). A 23-year-old Japanese male exhibited systemic lymphadenopathy, multiple lung tumors, and osteolytic changes in bilateral iliac bones in 1989. A biopsy specimen from the left iliac bone revealed an infiltration of S-100 protein-positive histiocyte-like cells intermingled with eosinophils, which confirmed the diagnosis of eosinophilic granuloma, a type of LCH. Although the patient was treated with prednisolone initially, the disease did not respond well and progressed gradually over time. The patient subsequently received multiple courses of chemotherapy and immunosuppressive therapy with many kinds of anticancer agents for 6 years. He also received radiotherapy totaling 136.8 Gy for lung tumors and osteolytic lesions of the pelvis. In 1997, because of the LCH refractoriness, biopsy was performed again from the right inguinal lymph node. Microscopic examinations demonstrated a mixture of spindle-shaped cells and histiocyte-like cells, which appeared to be in a storiform pattern. The tumor cells were immunohistologically positive for CD68 and vimentin, but negative for CD1a and S-100 protein. Therefore, the patient was diagnosed with MFH. Although chemotherapy was continued, the patient died of pneumonia during the neutropenic period following chemotherapy. Autopsy revealed systemic invasion of MFH and dissemination of mucormycosis. LCH was not detected histologically in any tissues. [J Clin Exp Hematopathol 49(1) : 33-37, 2009]

• 日本リンパ網内系学会の論文
• 2009-05-01

著者

• YAMAKAWA Mitsunori

  Department of Pathology, Yamagata University School of Medicine

• Ueda Takanori

  福井大学 血液腫瘍内科学

• Ueda T

  Department Of Hematology And Oncology University Of Fukui

• YAMAMOTO Shuji

  Department of Radiology, Osaka University Hospital

• ARAI Hajime

  Department of Neurosurgery, Juntendo University School of Medicine

• Yabe Aya

  Department Of Molecular Biology Institute Of Gerontology Nippon Medical School

• YOSHIDA AKIRA

  Department of Radiological Sciences, Hiroshima Prefectural College of Health Science

• Ueda T

  Department Of Urology Chiba Cancer Center

• Ueda T

  First Department Of Internal Medicine University Of Fukui

• Yamauchi T

  Department Of Hematology And Oncology University Of Fukui

• NAGOSHI Haruhisa

  Departments of Internal Medicine St. Marianna University Hospital

• Ueda Takanori

  Department of Cardiology, University of Fukui Hospital

• Yamauchi Takahiro

  福井大学 血液腫瘍内科学

• MISAKI Hirofumi

  Department of Hematology and Oncology, University of Fukui

• YAMAUCHI Takahiro

  Department of Hematology and Oncology, University of Fukui

• SUTOH Hidemasa

  Department of Hematology and Oncology, University of Fukui

• TSUTANI Hiroshi

  Department of Hematology and Oncology, University of Fukui

• EGUCHI Manabu

  Eguchi Naika Clinic

• NAIKI Hironobu

  Second Department of Pathology, University of Fukui

• BABA Hisatoshi

  Department of Orthopaedic Surgery, University of Fukui

• Naiki Hironobu

  Second Department Of Pathology University Of Fukui

• Baba Hisatoshi

  Department Of Orthopaedic Surgery University Of Fukui

• Baba Hisatoshi

  Department Of Orthopaedic Surgery Fukui Medical School

• Sutoh Hidemasa

  Department Of Hematology And Oncology University Of Fukui

• Misaki Hirofumi

  First Department Of Internal Medicine Fukui Medical University

• Misaki Hirofumi

  Department Of Hematology And Oncology University Of Fukui

• Yamauchi Takahiro

  Department Of Hematology And Oncology University Of Fukui

• Nagoshi Haruhisa

  Department Of Hematology And Oncology Yokohama City Seibu Hospital St. Marianna University

• Tsutani Hiroshi

  Department Of Hematology And Oncology University Of Fukui

• Yamakawa Mitsunori

  Department Of Pathology Faculty Of Medicine Yamagata University

• Yoshida Akira

  First Department Of Internal Medicine University Of Fukui

• Yoshida A

  Department Of Applied Biological Sciences School Of Agricultural Sciences Nagoya University

• Ueda Takahiro

  First Department Of Internal Medicine Fukui Medical University Idarubicin Study Group

• Arai Hajime

  Department Of Hematology And Oncology University Of Fukui

• Yoshida A

  First Department Of Internal Medicine University Of Fukui

• Ueda Takeshi

  Department Of Urology Chiba University Graduate School Of Medicine

• Yoshida Akira

  Department Of Cardiology Mitsubishi Kyoto Hospital

• Yamamoto Shuji

  Department Of Hematology And Oncology University Of Fukui

• Yoshida Akira

  Department Of Cardiology Kyoto National Hospital

• Yoshida Akira

  Department Of Agricultural Chemistry Nagoya University

• Yamakawa Mitsunori

  Department of Pathological Diagnostics Faculty of Medicine, Yamagata University

• Yamamoto Shuji

  Department of Applied Chemistry, Yamanashi University

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