Primary Cutaneous B-Cell Lymphoma: An Update on Pathologic and Molecular Features
Marco Lucioni,
Sara Fraticelli,
Giuseppe Neri,
Monica Feltri,
Giuseppina Ferrario,
Roberta Riboni,
Marco Paulli
Affiliations
Marco Lucioni
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100 Pavia, Italy
Sara Fraticelli
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, 27100 Pavia, Italy
Giuseppe Neri
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100 Pavia, Italy
Monica Feltri
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100 Pavia, Italy
Giuseppina Ferrario
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100 Pavia, Italy
Roberta Riboni
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100 Pavia, Italy
Marco Paulli
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100 Pavia, Italy
Primary cutaneous B-cell lymphomas (PCBCLs) account for 25% of all primary cutaneous lymphomas. Three major types are currently recognized by the WHO classification: primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle centre lymphoma (PCFCL) (both considered indolent lymphomas) and primary cutaneous diffuse large B-cell lymphoma, leg-type (PCDLBCL-LT), which is, instead, a very aggressive disease. Nowadays, the PCBCL’s category also includes some rare entities such as intravascular B-cell lymphoma (IVBL) and the EBV+ mucocutaneous ulcer (EBVMCU). Furthermore, controversies still exist concerning the category of primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), because some cases may present with clinical and histological features between PCFCL and PCDLBCL-LT. Therefore, some authors proposed introducing another category called PCDLBCL, not otherwise specified (NOS). Regardless, PCBCLs exhibit distinct features and differ in prognosis and treatment from their nodal/systemic counterparts. Therefore, clinicopathologic analysis is a key diagnostic element in the work-up of these lymphomas.
