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.
Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma / S. Alberti Violetti, C.A. Torres Cabala, R. Talpur, L. Corti, D. Fanoni, L. Venegoni, E. Berti, M. Duvic. - In: JOURNAL OF CUTANEOUS PATHOLOGY. - ISSN 0303-6987. - 43:12(2016 Aug 23), pp. 1121-1130.
|Titolo:||Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma|
ALBERTI VIOLETTI, SILVIA (Primo)
BERTI, EMILIO (Penultimo)
|Parole Chiave:||CD4+ small medium pleomorphic lymphoma; PD1; array comparative genomic hybridization; cutaneous T-cell lymphoma; mycosis fungoides|
|Settore Scientifico Disciplinare:||Settore MED/35 - Malattie Cutanee e Veneree|
|Data di pubblicazione:||23-ago-2016|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1111/cup.12806|
|Appare nelle tipologie:||01 - Articolo su periodico|
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