10.5826/dpc.1401a19
text/xml
2160-9381
3417
en
Vieri Grandi
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Giant Primary Cutaneous Follicle Centre B-Cell Lymphoma 
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Image Letter
2024-01-31
2023-04-10
2024-01-31
2024-02-21
e2024019
Dermatol Pract Concept
Vieri Grandi
2024/01/31
10.5826/dpc.1401a19
e2024019
Department of Health Sciences, Section of Dermatology, University of Florence, P. Palagi Hospital, Florence, Italy
2160-9381
en
e2024019
Skala SL, Hristov B, Hristov AC. Primary Cutaneous Follicle Center Lymphoma. Arch Pathol Lab Med. 2018;142(11):1313-1321. DOI: 10.5858/arpa.2018-0215-RA. PMID: 30407851.
Giant Primary Cutaneous Follicle Centre B-Cell Lymphoma 
https://dpcj.org/index.php/dpc/article/download/3417/2546
                 Giant Primary Cutaneous Follicle Centre
                            B-Cell Lymphoma
            Alberto Corrà1, Isabella Ciardetti1, Andrea Gemignani1, Eleonora Gherardi1,
                                  Nicola Pimpinelli1, Vieri Grandi1


1 Department of Health Sciences, Section of Dermatology, University of Florence, P. Palagi Hospital, Florence, Italy



Citation: Corrà A, Ciardetti I, Gemignani A, Gherardi E, Pimpinelli N, Grandi V. Giant Primary Cutaneous Follicle Centre B-Cell
Lymphoma. Dermatol Pract Concept. 2024;14(1):e2024019. DOI: https://doi.org/10.5826/dpc.1401a19
Accepted: May 29, 2023; Published: January 2024
Copyright: ©2024 Corrà et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Grandi Vieri, Department of Health Sciences, Section of Dermatology, University of Florence, P. Palagi Hospital,
Viale Michelangelo 41, 50141, Florence, Italy. E-mail: Vieri.grandi@unifi.it




Case Presentation                                                         Teaching Point
A 67-year-old man presented with firm, smooth, violaceous                 PCFCL accounts for 12% of cutaneous lymphomas and typ-
scalp nodules in the frontal-parietal region, which had been              ically occurs between the ages of 50 and 70 [1]. It manifests
present for 4 years. The lesions exhibited slow growth and                as slow-growing erythematous papules, plaques, or tumors
had merged to form a mass with telangiectasia, necrosis,                  primarily on the head-neck region or trunk.
ulceration, and crusting. Histological evaluation showed a                    Histologically, PCFCL is characterized by the prolifer-
dense follicular proliferation separated from the epidermis by            ation of centrocytes, centroblasts, and a few reactive lym-
a grenz zone, consisting of enlarged centrocytes intermixed               phocytes. The immunophenotype of neoplastic cells shows
with centroblasts. Immunohistochemistry was positive for                  positive expression of B‐cell markers such as CD20, CD79a,
Bcl-6, CD20, CD79a, PAX-5, while CD10, Bcl-2, CD34,                       and Bcl‐6, while lacking CD5 or CD43 expression, which
CD38, CD68, IRF4/MUM1, CD4 were negative. Subsequent                      distinguishes it from systemic follicular lymphomas. These
staging CT scan showed infiltration of the periosteum, bone,              markers are also helpful in differentiating PCFCL from
and dura mater, but no evidence of nodal or extracutane-                  primary cutaneous diffuse large B-cell lymphoma, leg type
ous visceral disease was found. A bone marrow biopsy did                  (which is positive for Bcl-2 and MUM1) and primary cuta-
not reveal any signs of lymphoma. We made a diagnosis of                  neous marginal zone lymphoma (which is positive for Bcl-2
primary cutaneous follicle center B-cell lymphoma (PCFCL).                but negative for Bcl-6 and CD10) [2].
The patient was treated with the R-CHOP (rituximab, cy-                       Although PCFCL has an excellent prognosis, long-­standing
clophosphamide, doxorubicin, vincristine and prednisone)                  untreated masses can invade deeper structures, leading to in-
regimen and achieved complete remission after 3 cycles. No                creased morbidity and mortality. Nevertheless, even bulky and
relapses were observed during 18 months of follow-up.                     neglected disease can show rapid responses to treatment.




Image Letter | Dermatol Pract Concept. 2024;14(1):e2024019                                                                               1
Figure 1. (A,B) Pictures of the giant mass of the scalp affecting the fronto-parietal area, in frontal (A) and lateral (B) view. (C) Detail of the
scalp mass. It is deducible that the mass resulted from the confluence of nodules, as an isolated tumor is visible in the lower part of the pic-
tures. Telangiectasia, crusting, necrosis with ulceration and fibrin deposits are also appreciable.




References                                                                  2. Skala SL, Hristov B, Hristov AC. Primary Cutaneous Follicle
                                                                               Center Lymphoma. Arch Pathol Lab Med. 2018;142(11):1313-
 1. Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the              1321. DOI: 10.5858/arpa.2018-0215-RA. PMID: 30407851.
    WHO-EORTC classification for primary c­utaneous lympho-
    mas. Blood. 2019;133(16):1703-1714. DOI: 10.1182/blood
    -2018-11-881268. PMID: 30635287. PMCID: PMC6473500.




2                                                                                 Image Letter | Dermatol Pract Concept. 2024;14(1):e2024019
