Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma.
J Cutan Pathol
; 43(12): 1121-1130, 2016 Dec.
Article
in En
| MEDLINE
| ID: mdl-27550169
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.Key words
Full text:
1
Database:
MEDLINE
Main subject:
Skin Neoplasms
/
CD4-Positive T-Lymphocytes
/
Lymphoma, T-Cell, Cutaneous
Type of study:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Language:
En
Journal:
J Cutan Pathol
Year:
2016
Type:
Article
Affiliation country:
Italy