Primary cutaneous B-cell lymphoma other than marginal zone: clinicopathologic analysis of 161 cases: Comparison with current classification and definition of prognostic markers.
Categorization of primary cutaneous B-cell lymphomas (PCBCL) other than marginal zone (MZL) represents a diagnostic challenge with relevant prognostic implications. The 2008 WHO lymphoma classification recognizes only primary cutaneous follicular center cell lymphoma (PCFCCL) and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), whereas the previous 2005 WHO/EORTC classification also included an intermediate form, namely PCDLBCL, other. We conducted a retrospective, multicentric, consensus-based revision of the clinicopathologic characteristics of 161 cases of PCBCL other than MZL. Upon the histologic features that are listed in the WHO classification, 96 cases were classified as PCFCCL and 25 as PCDLBCL-LT; 40 further cases did not fit in the former subgroups in terms of cytology and/or architecture, thus were classified as PCDLBCL, not otherwise specified (PCDLBCL-NOS). We assigned all the cases a histogenetic profile, based on the immunohistochemical detection of CD10, BCL6, and MUM1, and a "double hit score" upon positivity for BCL2 and MYC. PCDLBCL-NOS had a clinical presentation more similar to PCFCCL, whereas the histology was more consistent with the picture of a diffuse large B-cell lymphoma, as predominantly composed of centroblasts but with intermixed a reactive infiltrate of small lymphocytes. Its behavior was intermediate between the other two forms, particularly when considering only cases with a "non-germinal B-cell" profile, whereas "germinal center" cases resembled PCFCCL. Our data confirmed the aggressive behavior of PCDLBC-LT, which often coexpressed MYC and BCL2. The impact of single factors on 5-year survival was documented, particularly histogenetic profile in PCDLBCL and BCL2 translocation in PCFCCL. Our study confirms that a further group-PCDLBCL-NOS-exists, which can be recognized through a careful combination of histopathologic criteria coupled with adequate clinical information.
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Marco
M
Lucioni
Department of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico and Università degli Studi di Milano-Bicocca, Milan, Italy.
Emilio
0000-0001-6753-4910
E
Berti
Section of Hematology-Oncology, Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Luca
L
Arcaini
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Giorgio A
GA
Croci
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Aldo
A
Maffi
Scientific Direction, Biometry and Statistics, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Catherine
C
Klersy
Pathologic Anatomy and Histopathology, Department of Biomedical Sciences and Public Health, Polytechnic University of Marche Region, United Ancona Hospitals, Torrette, Ancona, Italy.
Gaia
G
Goteri
Pathology Unit, Department of Laboratory Medicine, Azienda Ospedaliera Città della Salute e della Scienza di Torino, Turin, Italy.
Carlo
C
Tomasini
Dermatologic Clinic, Department of Medical Sciences, University of Turin, Turin, Italy.
Pietro
P
Quaglino
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Roberta
R
Riboni
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Mariarosa
M
Arra
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Elena
E
Dallera
Division of Dermatology, University of Florence Medical School, Florence, Italy.
Vieri
V
Grandi
Dermatologic Clinic, University of Padova, Padova, Italy.
Mauro
M
Alaibac
Division of Pathology, Azienda Ospedaliero-Universitaria Maggiore della Carità, Novara, Italy.
Antonio
A
Ramponi
Department of Hematology and Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Sara
S
Rattotti
Division of Hematology, Osp A Businco, Cagliari, Italy.
Maria Giuseppina
MG
Cabras
Division of Hematology, Department of Translational Medicine, Amedeo Avogadro University of Eastern Piedmont, Azienda Ospedaliero-Universitaria Maggiore della Carità, Novara, Italy.
Silvia
S
Franceschetti
Anatomic Pathology Division, San Martino University Hospital, Genova, Italy.
Giulio
G
Fraternali-Orcioni
Department of Surgical and Morphological Sciences, Faculty of Medicine and Surgery, University of Insubria, Varese, Italy.
Nicola
N
Zerbinati
Hematology and Bone Marrow Transplantation Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan, Milano, Italy.
Francesco
0000-0002-2991-3474
F
Onida
Institute of Pathology, Ospedale S. Maria di Terni and University of Perugia, Perugia, Italy.
Stefano
S
Ascani
Dermatologic Clinic, Department of Medical Sciences, University of Turin, Turin, Italy.
Maria Teresa
MT
Fierro
Clinic of Hematology, United Ancona Hospitals, Torrette, Ancona, Italy.
Serena
S
Rupoli
Synlab, Italia, Brescia, Italy.
Marcello
M
Gambacorta
Institute of Hematology "Seràgnoli", University of Bologna, Bologna, Italy.
Pier Luigi
PL
Zinzani
Division of Dermatology, University of Florence Medical School, Florence, Italy.
Nicola
N
Pimpinelli
Division of Pathological Anatomy, Department of Surgery and Translational Medicine, University of Florence School of Human Health Sciences, Firenze, Italy.
Marco
M
Santucci
Unit of Anatomic Pathology, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. marco.paulli@unipv.it.
Marco
M
Paulli
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000378:metabolism*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D050835:Interferon Regulatory Factors / Q000378:metabolism; D016393:Lymphoma, B-Cell / Q000145:classification* / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015260:Neprilysin / Q000378:metabolism; D011379:Prognosis; D051560:Proto-Oncogene Proteins c-bcl-6 / Q000378:metabolism; D016271:Proto-Oncogene Proteins c-myc / Q000378:metabolism; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000145:classification* / Q000378:metabolism / Q000473:pathology*; D016019:Survival Analysis; D000099218:Interferon Regulatory Factor-4
D014408:Biomarkers, Tumor; D050835:Interferon Regulatory Factors; D015260:Neprilysin; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc; D000099218:Interferon Regulatory Factor-4; C084103:BCL6 protein, human; C489427:MYC protein, human
BCL2; cutaneous lymphoma; follicular lymphoma; large cell lymphoma; leg type
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