Cutaneous B-cell lymphomas: Update on diagnosis, risk-stratification, and management

Paolo Fava; Gabriele Roccuzzo; Silvia Alberti-Violetti; Vieri Grandi; Alessandro Pileri; Nicola Pimpinelli; Emilio Berti; Pietro Quaglino

doi:10.1016/j.lpm.2022.104109

Cutaneous B-cell lymphomas: Update on diagnosis, risk-stratification, and management

Presse Med. 2022 Mar;51(1):104109. doi: 10.1016/j.lpm.2022.104109. Epub 2022 Jan 11.

Authors

Paolo Fava¹, Gabriele Roccuzzo², Silvia Alberti-Violetti³, Vieri Grandi⁴, Alessandro Pileri⁵, Nicola Pimpinelli⁴, Emilio Berti³, Pietro Quaglino¹

Affiliations

¹ Clinic of Dermatology, Department of Medical Sciences, University of Turin, Turin, Italy.
² Clinic of Dermatology, Department of Medical Sciences, University of Turin, Turin, Italy. Electronic address: gabriele.roccuzzo@unito.it.
³ UOC Dermatologia, Dipartimento di Medicina Interna, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
⁴ Unit of Dermatology, Department of Surgery and Translational Medicine, University of Florence Medical School, Florence, Italy.
⁵ Unit of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

PMID: 35026390
DOI: 10.1016/j.lpm.2022.104109

Abstract

PCBCLs are a group of Non-Hodgkin's B-cell lymphomas originating in and usually confined to the skin, representing approximately one fourth of primary cutaneous lymphomas (PCL). Their current classification system has been the result of the joint World Health Organization (WHO) - European Organization for Research and Treatment of Cancer (EORTC) consensus in 2018. To date, several types of PCBCLs have been described in the scientific literature, with different clinical presentation and prognosis. Primary cutaneous follicle-center lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma (PCMZL) are the most common forms, with a typical indolent course. On the contrary, primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is less common, yet more aggressive, with a reported 5-year overall survival of approximatively 50%. In this review, we outline the PCBCLs defining diagnostic criteria, report the features of the less common subtypes and summarize the noteworthy therapeutical options currently available in this field.

Keywords: CBCL; DLBCL; IVLBCL; LT; NOS; PCBCL-FCL; PCDLBCL; PCMZL; TCRBCL.

Publication types

Review

MeSH terms

Humans
Lymphoma, B-Cell* / diagnosis
Lymphoma, B-Cell* / pathology
Lymphoma, B-Cell* / therapy
Lymphoma, Large B-Cell, Diffuse* / diagnosis
Lymphoma, Large B-Cell, Diffuse* / pathology
Lymphoma, Large B-Cell, Diffuse* / therapy
Prognosis
Skin Neoplasms* / diagnosis
Skin Neoplasms* / pathology
Skin Neoplasms* / therapy