title: CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.
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, S100beta, 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.
authors.affiliation: Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya 464-8681, Japan.
authors.forename: Takakazu
authors.initials: T
authors.lastname: Kawase
authors.forename: Minoru
authors.initials: M
authors.lastname: Hamazaki
authors.forename: Michinori
authors.initials: M
authors.lastname: Ogura
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kawase
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Murayama
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Mori
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Nagai
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Tateno
authors.forename: Takashi
authors.initials: T
authors.lastname: Oyama
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Kamiya
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Taji
authors.forename: Yoshitoyo
authors.initials: Y
authors.lastname: Kagami
authors.forename: Tomoki
authors.initials: T
authors.lastname: Naoe
authors.forename: Toshitada
authors.initials: T
authors.lastname: Takahashi
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Morishima
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000328:Adult; D019002:CD56 Antigen / Q000032:analysis*; D005260:Female; D005334:Fever / Q000209:etiology; D006646:Histiocytosis, Langerhans-Cell / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology; D010146:Pain / Q000209:etiology; D012509:Sarcoma / Q000276:immunology* / Q000473:pathology* / Q000628:therapy
chemical_list: D019002:CD56 Antigen