title: Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma.
abstract: BACKGROUND
  Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma (CD4+ PCSM-TCL) is a rare lymphoproliferative disorder with a favorable prognosis. Distinguishing it from other cutaneous lymphomas is often a challenge.
  
  
  METHODS
  We retrospectively collected CD4+PCSM-TCL cases from two centers (MD Anderson Cancer Center, USA and University of Milan, Italy) and evaluated their clinicopathological features. Array-comparative genomic hybridization (aCGH) analysis was performed on 11 cases.
  
  
  RESULTS
  A total of 62 patients were identified. Single lesions were the most common clinical presentations (79%). Five patients (8%) showed multiple MF-like plaques. All patients' disease had an indolent course. The infiltrate was nodular and diffuse, multinodular or superficial but in all cases, it was characterized by small/medium pleomorphic CD4+/CD279(PD1+) lymphocytes grouped in clusters and 'pseudorosettes' around B-cells. aCGH analysis showed no significant genomic abnormalities. Single lesions were mainly treated with surgical excision (91%) and/or radiotherapy (95%) with low rate of relapse (12%). For multiple lesions, topical steroids, nitrogen mustard and phototherapy were mainly used but the rate of relapse was high (69%).
  
  
  CONCLUSIONS
  CD4+PCSM-TCL is characterized by heterogeneous clinical presentations. The arrangement of atypical cells in clusters or pseudorosettes is a useful criterion for diagnosis. The absence of significant genomic alterations is in agreement with its indolent behavior.
authors.affiliation: UOC Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Alberti-Violetti
authors.affiliation: Department of Pathology, Dermatopathology Section, University of Texas, MD Anderson Cancer Center, Houston, TX, USA.|Department of Dermatology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Carlos A
authors.initials: CA
authors.lastname: Torres-Cabala
authors.affiliation: Department of Dermatology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Rakhshandra
authors.initials: R
authors.lastname: Talpur
authors.affiliation: UOC Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Corti
authors.affiliation: UOC Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: Fanoni
authors.affiliation: Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.
authors.forename: Luigia
authors.initials: L
authors.lastname: Venegoni
authors.affiliation: UOC Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.|Dipartimento di Scienze della Salute, Università degli Studi di Milano-Bicocca, Milan, Italy.
authors.forename: Emilio
authors.initials: E
authors.lastname: Berti
authors.affiliation: Department of Dermatology, University of Texas, MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D055028:Comparative Genomic Hybridization; D062109:Dermatologic Surgical Procedures; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D016133:Polymerase Chain Reaction; D011878:Radiotherapy; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents
keywords: CD4+ small medium pleomorphic lymphoma; PD1; array comparative genomic hybridization; cutaneous T-cell lymphoma; mycosis fungoides