title: Langerhans cell histiocytosis of an adult with tumors in liver and spleen.
abstract: We describe a 58-year-old male with multiple histiocytic tumors in the liver and spleen. Multiple tumors in the liver and spleen were seen by image analysis, and splenectomy showed a large splenic tumor with a small nodule and a swelling lymph node in the hilus. Histological features of the tumors in the liver and spleen revealed proliferation of histiocytic cells with large and clear cytoplasm and a horseshoe-shaped nucleus. Immunohistochemical studies revealed the presence of S-100 protein and CD1a antigen in the tumor cells, and neither lymphocytic marker nor lysozyme was detected. No definite Birbeck granules were seen ultrastructurally, thus the tumor cells could be classified into Langerhans cell type without Birbeck granules. Administration of adriamycin, vincristine, cyclophosphamide and prednisolone reduced size and number of the liver tumors, and the histiocytic cells could not be detected in repeatedly biopsied tissue from liver tumor. We present the clinical, immunohistological and cytological features in a visceral type of adult Langerhans cell histiocytosis, which responded well to chemotherapy.
authors.affiliation: Third Department of Internal Medicine and Hospital Pathology, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji Kamigyo-ku, Kyoto, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Yagita
authors.forename: M
authors.initials: M
authors.lastname: Iwai
authors.forename: M
authors.initials: M
authors.lastname: Yagita-Toguri
authors.forename: H
authors.initials: H
authors.lastname: Kimura
authors.forename: M
authors.initials: M
authors.lastname: Taniwaki
authors.forename: S
authors.initials: S
authors.lastname: Misawa
authors.forename: T
authors.initials: T
authors.lastname: Okanoue
authors.forename: K
authors.initials: K
authors.lastname: Kashima
authors.forename: Y
authors.initials: Y
authors.lastname: Tsuchihashi
mesh_terms: D018949:Antigens, CD1 / Q000378:metabolism; D006644:Histiocytes / Q000648:ultrastructure; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009418:S100 Proteins / Q000378:metabolism; D013160:Splenic Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D018949:Antigens, CD1; C419393:CD1a antigen; D009418:S100 Proteins