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Clinicopathological and molecular study of primary cutaneous CD4+ small/medium‐sized pleomorphic T‐cell lymphoma
Author(s) -
AlbertiVioletti Silvia,
TorresCabala Carlos A,
Talpur Rakhshandra,
Corti Laura,
Fai Daniele,
Venegoni Luigia,
Berti Emilio,
Duvic Madeleine
Publication year - 2016
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.12806
Subject(s) - comparative genomic hybridization , pathology , lymphoma , medicine , cutaneous lymphoma , biology , mycosis fungoides , chromosome , gene , biochemistry
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.