title: Comparison of primary and secondary cutaneous CD56+ NK/T cell lymphomas.
abstract: CD56+ NK/T cell lymphoma (NKTL) frequently involves skin and subcutaneous tissue. The characteristics of primary cutaneous nasal-type CD56+ NKTLs and secondary cutaneous involvement of nasal CD56+ NKTLs have not been clearly separated. This retrospective study analyzed 15 cases of NKTL (10 primary CD56+ NKTLs and 5 secondary CD56+ NKTLs) for their clinicopathologic and immunophenotypic characteristics using CD3, CD4, CD20, CD45RO, CD56, TIA-1, CD30, and Ki-67 antigens. In situ hybridization for Epstein-Barr virus RNA (EBERISH) and PCR for T cell receptor (TCR) gamma gene rearrangement were also performed. Clinically, NKTL-P was seen with equal frequency among male (five cases) and female (five cases) patients and presented with subcutaneous nodules without epidermal changes (nine cases), whereas all cases of NKTL-S occurred in male patients and presented with nodules or plaques with distinct epidermal changes (five cases). Microscopically, initial NKTL-P lesions had the panniculitic patterns of small to medium-sized cells (nine cases). NKTL-S lesions were extensive in both subcutis and dermis, with larger and more pleomorphic tumor cells (four cases) that also showed signs epidermotropism (five cases). In initial biopsies of CD56+ NKTL-P, a minority of tumor cells showed signals for EBERISH and in biopsies of CD56+ NKTL-S, virtually every tumor cell showed signals. While all five patients with secondary CD56+ NKTL died of disease with widespread systemic involvement within 16 months after onset of skin lesions, 7 out of the 10 primary CD56+ NKTL patients survived more than 20 months after onset of skin lesions, with slow progression and episodic recurrences. The primary and secondary cutaneous CD56+ NKTLs showed considerable clinicopathologic differences, suggesting differences in pathogenesis.
authors.affiliation: Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
authors.forename: Sung-Eun
authors.initials: SE
authors.lastname: Chang
authors.forename: Ghil-Suk
authors.initials: GS
authors.lastname: Yoon
authors.forename: Jooryung
authors.initials: J
authors.lastname: Huh
authors.forename: Jee-Ho
authors.initials: JH
authors.lastname: Choi
authors.forename: Kyung-Jeh
authors.initials: KJ
authors.lastname: Sung
authors.forename: Kee-Chan
authors.initials: KC
authors.lastname: Moon
authors.forename: Jai-Kyoung
authors.initials: JK
authors.lastname: Koh
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019002:CD56 Antigen / Q000276:immunology*; D005260:Female; D005838:Genotype; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D007694:Killer Cells, Natural / Q000276:immunology*; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology* / Q000473:pathology
chemical_list: D019002:CD56 Antigen; D016692:Receptors, Antigen, T-Cell, gamma-delta