Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma

Silvia Alberti-Violetti, Carlos A. Torres-Cabala, Rakhshandra Talpur, Laura Corti, Daniele Fanoni, Luigia Venegoni, Emilio Berti, Madeleine Duvic

Research output: Contribution to journalArticle

18 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1121-1130
Number of pages10
JournalJournal of Cutaneous Pathology
Volume43
Issue number12
DOIs
Publication statusPublished - Dec 1 2016

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T-Cell Lymphoma
Non-Hodgkin's Lymphoma
Comparative Genomic Hybridization
Skin
Recurrence
Mechlorethamine
Phototherapy
Lymphoproliferative Disorders
Italy
Lymphoma
B-Lymphocytes
Radiotherapy
Steroids
Lymphocytes
Neoplasms

Keywords

  • array comparative genomic hybridization
  • CD4+ small medium pleomorphic lymphoma
  • cutaneous T-cell lymphoma
  • mycosis fungoides
  • PD1

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. / Alberti-Violetti, Silvia; Torres-Cabala, Carlos A.; Talpur, Rakhshandra; Corti, Laura; Fanoni, Daniele; Venegoni, Luigia; Berti, Emilio; Duvic, Madeleine.

In: Journal of Cutaneous Pathology, Vol. 43, No. 12, 01.12.2016, p. 1121-1130.

Research output: Contribution to journalArticle

Alberti-Violetti, Silvia ; Torres-Cabala, Carlos A. ; Talpur, Rakhshandra ; Corti, Laura ; Fanoni, Daniele ; Venegoni, Luigia ; Berti, Emilio ; Duvic, Madeleine. / Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma. In: Journal of Cutaneous Pathology. 2016 ; Vol. 43, No. 12. pp. 1121-1130.
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AU - Corti, Laura

AU - Fanoni, Daniele

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N2 - 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.

AB - 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.

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