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.

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

Fava P.;Roccuzzo G.;Quaglino P.
2022-01-01

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.
2022
51
1
104109
104116
CBCL; DLBCL; IVLBCL; LT; NOS; PCBCL-FCL; PCDLBCL; PCMZL; TCRBCL
Fava P.; Roccuzzo G.; Alberti-Violetti S.; Grandi V.; Pileri A.; Pimpinelli N.; Berti E.; Quaglino P.
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/1863424
Citazioni
  • ???jsp.display-item.citation.pmc??? 5
  • Scopus 8
  • ???jsp.display-item.citation.isi??? 8
social impact