title: CD56/neural cell adhesion molecule expression in primary extranodal Ki-1/CD30+ lymphoma. Report of a pediatric case with simultaneous cutaneous and bone localizations.
abstract: We describe the clinicopathologic features of an unusual case of CD30+/CD50+ T-cell lymphoma in a child who presented with simultaneous primary extranodal cutaneous and bone localizations. The expression of CD56 (neural cell adhesion molecule, or NCAM) is rare in non-Hodgkin's lymphomas other than in a group of haematopoietic/lymphoid neoplasms of natural killer and natural killer-like T-cells, which usually involve extranodal sites and often pursue an aggressive clinical behavior. Coexpression of CD30 and CD56 in T-cell lymphomas is exceedingly rare, and its biological significance is unknown. Our patient responded well to an intensive chemotherapy regimen, and she is now in complete remission 4 years after discontinuation of chemotherapy. Expression of NCAM could be regarded as responsible, in part, for the extranodal localization of lymphoma cells; expression of CD56 also might contribute to the definition of a subset of CD30+ lymphomas with distinctive clinicopathologic features.
authors.affiliation: Research Unit, University of Pavia, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Paulli
authors.forename: E
authors.initials: E
authors.lastname: Boveri
authors.forename: R
authors.initials: R
authors.lastname: Rosso
authors.forename: M
authors.initials: M
authors.lastname: Aricò
authors.forename: S
authors.initials: S
authors.lastname: Kindl
authors.forename: A
authors.initials: A
authors.lastname: Viglio
authors.forename: E
authors.initials: E
authors.lastname: Berti
authors.forename: F
authors.initials: F
authors.lastname: Leithäuser
authors.forename: F
authors.initials: F
authors.lastname: Locatelli
authors.forename: U
authors.initials: U
authors.lastname: Gianelli
authors.forename: G
authors.initials: G
authors.lastname: Beluffi
authors.forename: A C
authors.initials: AC
authors.lastname: Feller
authors.forename: G
authors.initials: G
authors.lastname: Borroni
authors.forename: U
authors.initials: U
authors.lastname: Magrini
mesh_terms: D000469:Alkaline Phosphatase; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000188:drug therapy / Q000473:pathology*; D019002:CD56 Antigen / Q000032:analysis / Q000235:genetics*; D002675:Child, Preschool; D004396:Coloring Agents; D005260:Female; D005500:Follow-Up Studies; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007124:Immunoenzyme Techniques; D007694:Killer Cells, Natural / Q000473:pathology; D017728:Lymphoma, Large-Cell, Anaplastic / Q000188:drug therapy / Q000473:pathology*; D018396:Mucin-1 / Q000032:analysis; D019006:Neural Cell Adhesion Molecules / Q000032:analysis / Q000235:genetics*; D016133:Polymerase Chain Reaction; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis; D012074:Remission Induction; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology
chemical_list: D019002:CD56 Antigen; D004396:Coloring Agents; D018396:Mucin-1; D019006:Neural Cell Adhesion Molecules; D016692:Receptors, Antigen, T-Cell, gamma-delta; D000469:Alkaline Phosphatase