title: Childhood CD4+/CD56+ hematodermic neoplasm: case report and review of the literature.
abstract: Recently, rare CD4+/CD56+ hematodermic neoplasm has been described as a distinct clinico-pathologic entity, with aggressive course and poor outcome. Skin is typically involved at presentation, but widespread dissemination to bone marrow is rapid. To date, no standardized therapeutic approach to this disease has been established. As its diffusion mainly concerns elderly patients, only a few paediatric cases have been documented. We report an additional case of CD4+/CD56+ hematodermic tumour that showed a good response to chemotherapy based on a lymphoma protocol. Moreover, we try to analyse features and outcome of a few other paediatric CD4+/CD56+ hematodermic tumours as they are reported in the literature.
authors.affiliation: Division of Paediatric Oncology, Department of Paediatric Sciences, Catholic University, Largo A. Gemelli 8 00168 Rome, Italy. ruggiero@rm.unicatt.it
authors.forename: Antonio
authors.initials: A
authors.lastname: Ruggiero
authors.forename: Palma
authors.initials: P
authors.lastname: Maurizi
authors.forename: Luigi Maria
authors.initials: LM
authors.lastname: Larocca
authors.forename: Annalisa
authors.initials: A
authors.lastname: Arlotta
authors.forename: Riccardo
authors.initials: R
authors.lastname: Riccardi
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D015704:CD4 Antigens / Q000032:analysis; D019002:CD56 Antigen / Q000032:analysis; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D003630:Daunorubicin / Q000008:administration & dosage; D003713:Dendritic Cells / Q000473:pathology; D003907:Dexamethasone / Q000008:administration & dosage; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D015704:CD4 Antigens; D019002:CD56 Antigen; D003561:Cytarabine; D014750:Vincristine; D003907:Dexamethasone; D003520:Cyclophosphamide; D015122:Mercaptopurine; D001215:Asparaginase; D011241:Prednisone; D008727:Methotrexate; D003630:Daunorubicin