Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) leg type is an aggressive type of CBCL, characterized by skin lesions mainly on the legs and a predominance of diffuse sheets of centroblasts and immunoblasts. Given the poor prognosis, old age at onset, frequent relapses, and extracutaneous spread, PCDLBCL, leg type is a distinct type of cutaneous lyphoma. Here the clinicopathology of extranodal lymphomas will be discussed

Chen, Ding-Bao

Atlas of Genetics and Cytogenetics in Oncology and Haematology

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 Leukaemia Section 
Review 
Atlas Genet Cytogenet Oncol Haematol. 2018; 22(11) 439 
Atlas of Genetics and Cytogenetics 
in Oncology and Haematology 
OPEN ACCESS JOURNAL AT INIST-CNRS 
Primary cutaneous DLBCL, leg type 
Ding-Bao Chen 
Department of Pathology, Peking University People's Hospital, Beijing 100044, People's Republic of 
China; cdingbao@163.com 
Published in Atlas Database: September 2017 
Online updated version : http://AtlasGeneticsOncology.org/Anomalies/DLBCLPrimCutanID1245.html 
Printable original version : http://documents.irevues.inist.fr/bitstream/handle/2042/68967/09-2017-DLBCLPrimCutanID1245.pdf 
DOI: 10.4267/2042/68967
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. 
© 2018 Atlas of Genetics and Cytogenetics in Oncology and Haematology 
Abstract 
Primary cutaneous diffuse large B-cell lymphoma 
(PCDLBCL) leg type is an aggressive type of CBCL, 
characterized by skin lesions mainly on the legs and 
a predominance of diffuse sheets of centroblasts and 
immunoblasts. Given the poor prognosis, old age at 
onset, frequent relapses, and extracutaneous spread, 
PCDLBCL, leg type is a distinct type of cutaneous 
lyphoma. Here the clinicopathology of extranodal 
lymphomas will be discussed. 
KEYWORDS 
Diffuse large B-cell lymphoma; Cutaneous 
lymphoma; Primary cutaneous, leg type 
Clinics and pathology 
Disease 
PCDLBCL, leg type is composed exclusively of 
large transformed B-cells, most commonly arising in 
the leg. PCDLBCL, leg type was first reported as a 
subgroup of PCFCL in 1987 based on its particular 
histological feature and more aggressive behavior. In 
1996, the term "primary cutaneous large B-cell 
lymphoma of the leg" was proposed, which was used 
to classify it as a distinctive subgroup in the EORTC 
classification. In the last WHO-EORTC 
classification, this entity was finally defined 
PCDLBCL, leg type to reflect the predominant but 
not exclusive anatomic location of the lesions 
(Swerdlow, et al, 2008. Selva, et al, 2017). 
Phenotype/cell stem origin 
Tumor cells express B-cell markers (CD20, CD79a, 
and PAX5). Cytoplasmic IgM expression seems to 
be a sensitive marker of PCDLBCL, leg type. BCL-
2, MUM1/IRF4, and FOX-P1 are strongly expressed 
regardless of the location of the skin lesions. A study 
suggests that cell-of origin is germinal center 
experienced and superantigen driven selected B-cell, 
in a stage between germinal center B-cell and plasma 
cell (Swerdlow, et al , 2008. Selva, et al, 2017). 
Epidemiology 
PCDLBCL, LT represents 5%-10% of all PCBCLs 
commonly presenting in elderly females 
(male:female 1:3-4), with a peak incidence in the 
seventh decade of life (Swerdlow, et al , 2008. Selva, 
et al, 2017). 
Clinics 
Patients present solitary or multiple, rapidly 
growing, red to bluish-red firm tumors on one or 
both legs, usually below the knee. In 10%-15% of 
cases, the lesions are localized at other sites such as 
the trunk, head-neck, and upper arms. Multiple 
lesions may be disseminated or aggregated. 
Extracutaneous sites are often disseminated, most 
commonly in lymph nodes, bone marrow, and 
Primary cutaneous DLBCL, leg type  
 
Atlas Genet Cytogenet Oncol Haematol. 2018; 22(11) 440 
 
nervous system (Swerdlow, et al , 2008. Selva, et al, 
2017). 
Pathology 
Histologically, there is a diffuse and dense infiltrate 
throughout the dermis that often involves the 
subcutis but is separated from the epidermis by a 
Grenz zone. The infiltrate mainly consists of 
confluent sheets of large B-cells with roundish 
nuclei, prominent nucleoli, and open chromatin 
resembling centroblasts and immunoblasts. 
Anaplastic cells are occasionally seen. Mitotic 
figures are frequently observed. T-cells are rare. 
PCDLBCL, leg type is usually characterized by a 
high proliferation rate (>70%) and BCL-6, CD5, 
CD10, CD30, CD138, and cyclin D1 negativity. 
 
Figure 1.  Primary cutaneous DLBCL, leg type. There is a foci and sheets infiltration of lymphoid cells 
throughout the dermis. A Grenz zone can be seen separated from the epidermis. (HE staining). 
 
Figure 2.  The lymphoid cells are medium to large in size, with oval nuclei and distinct nucleoli. (HE staining) 
Primary cutaneous DLBCL, leg type  
 
Atlas Genet Cytogenet Oncol Haematol. 2018; 22(11) 441 
 
 
Figure 3.  The cells are positive for CD20. (immunostaining) 
 
Figure 4.  Ki67 immunostaining shows a high proliferation rate. (immunostaining). 
Treatment 
PCDLBCL, leg type is often managed as a systemic 
lymphoma depending on its staging and location, 
number of skin lesions, and general status of the 
patient. Immunochemotherapy with R-CHOP with 
or without involved-field radiotherapy as the first-
line therapy for single, localized, or generalized 
lesions is recommended. The use of local 
radiotherapy or rituximab as a single agent can be 
considered in particular cases. In relapsed cases, the  
 
use of protocols for relapsed systemic DLBCLs is 
recommended (Swerdlow, et al ,2008. Selva, et al, 
2017. Wilcox, 2016). Targeting of tumor cells and 
myeloid-derived suppressor cells using anti-PD-1/ 
anti -PD-L1 or anti-CD33 antibodies might be a 
worthwhile new approach to treat PCDLBCL, leg 
type (Mitteldorf, et al. 2017). 
Primary cutaneous DLBCL, leg type  
 
Atlas Genet Cytogenet Oncol Haematol. 2018; 22(11) 442 
 
Prognosis 
The prognosis of PCDLBCL, LT is poor and 
characterized by frequent relapses and 
extracutaneous spreading. The 5-year survival rate is 
40%-55%. A location on the leg and multiple skin 
lesions are negative prognostic factors. Factors that 
have been reported to be linked to a worse prognosis 
are the high expression of MUM1 and FOX-P1, and 
deletion of the CDKN2A locus on chromosome 
9p21 (Swerdlow, et al, 2008. Selva, et al, 2017). 
Cytogenetics 
Cytogenetic studies have revealed a frequent 
inactivation of CDKN2B (p15(INK4b)) and 
CDKN2A (p16(INK4a)) as a result of promoter 
hypermethylation (respectively, 11% and 44% of all 
PCDLBCLs) and chromosomal imbalances in up to 
85% of PCDLBCL, leg type (mainly gains of 
chromosome 2q, 3, 7p, 12q, 18q and losses of 6q, 
13,14,17p, 19). Translocations of MYC, BCL6, and 
IGH genes have been demonstrated by fluorescence 
in situ hybridization in PCDLBCL, leg type. 
PCLBCL leg type exhibits a unique mutational 
landscape, combining highly recurrent hotspot 
mutations in genes involved in NF-kb and B-cell 
signaling pathways, which provides a rationale for 
using selective inhibitors of the B-cell receptor 
(Swerdlow, et al, 2008. Selva, et al, 2017. 
Mareschal, et al. 2017). 
References 
Mareschal S, Pham-Ledard A, Viailly PJ, Dubois S, 
Bertrand P, Maingonnat C, Fontanilles M, Bohers E, Ruminy 
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E, Verneuil L, Vergier B, Tilly H, Joly P, Frebourg T, Beylot-
Barry M, Merlio JP, Jardin F. Identification of Somatic 
Mutations in Primary Cutaneous Diffuse Large B-Cell 
Lymphoma, Leg Type by Massive Parallel Sequencing. J 
Invest Dermatol. 2017 Sep;137(9):1984-1994 
Mitteldorf C, Berisha A, Pfaltz MC, Broekaert SMC, Schön 
MP, Kerl K, Kempf W. Tumor Microenvironment and 
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This article should be referenced as such:  
Chen DB. Primary cutaneous DLBCL, leg type. 
Atlas Genet Cytogenet Oncol Haematol. 2018; 
22(11):439-442. 


Primary cutaneous DLBCL, leg type

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Primary cutaneous DLBCL, leg type

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