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Primary cutaneous follicle center lymphoma with diffuse CD30 expression: a report of 4 cases of a rare variant


Kempf, Werner; Kazakov, Dmitry V; Rütten, Arno; Rupec, Rudolf A; Talarcik, Petr; Ballová, Veronika; Kerl, Katrin; Dummer, Reinhard; Lautenschlager, Stephan; Zimmermann, Dieter R; Tinguely, Marianne (2014). Primary cutaneous follicle center lymphoma with diffuse CD30 expression: a report of 4 cases of a rare variant. Journal of the American Academy of Dermatology, 71(3):548-54.

Abstract

BACKGROUND: CD30 is expressed in aggressive and Epstein-Barr virus-associated forms of B-cell non-Hodgkin lymphomas, but is rarely expressed by the majority of tumor cells in primary cutaneous B-cell lymphomas (CBCLs). The expression of CD30 in CBCLs may be at risk for misinterpretation as an unequivocal indicator of a highly aggressive form of the disease.
OBJECTIVE: We report 4 cases of low malignant primary cutaneous follicle center lymphoma (PCFCL) with diffuse and strong expression of CD30 by the majority of neoplastic cells.
RESULTS: The patients included 3 men and 1 woman with tumors on the scalp (3 patients) and chest wall (1 patient). The histologic examinations revealed a mixed, diffuse, and follicular growth pattern with CD20(+), bcl-6(+), and bcl-2(-) tumor cells. Seventy percent to 90% of the tumor cells expressed CD30. Clonal rearrangement of immunoglobulin heavy chain genes was found in 1 of 4 cases. None of the 3 cases yielded positivity for Epstein-Barr virus RNA.
LIMITATIONS: The study is limited by the small number of patients.
CONCLUSIONS: This rare variant of CD30(+) PCFCL needs be distinguished from CD30(+) aggressive B-cell lymphomas. CD30 in this variant of CBCLs may serve as a therapeutic target for anti-CD30 antibody-based strategies.

Abstract

BACKGROUND: CD30 is expressed in aggressive and Epstein-Barr virus-associated forms of B-cell non-Hodgkin lymphomas, but is rarely expressed by the majority of tumor cells in primary cutaneous B-cell lymphomas (CBCLs). The expression of CD30 in CBCLs may be at risk for misinterpretation as an unequivocal indicator of a highly aggressive form of the disease.
OBJECTIVE: We report 4 cases of low malignant primary cutaneous follicle center lymphoma (PCFCL) with diffuse and strong expression of CD30 by the majority of neoplastic cells.
RESULTS: The patients included 3 men and 1 woman with tumors on the scalp (3 patients) and chest wall (1 patient). The histologic examinations revealed a mixed, diffuse, and follicular growth pattern with CD20(+), bcl-6(+), and bcl-2(-) tumor cells. Seventy percent to 90% of the tumor cells expressed CD30. Clonal rearrangement of immunoglobulin heavy chain genes was found in 1 of 4 cases. None of the 3 cases yielded positivity for Epstein-Barr virus RNA.
LIMITATIONS: The study is limited by the small number of patients.
CONCLUSIONS: This rare variant of CD30(+) PCFCL needs be distinguished from CD30(+) aggressive B-cell lymphomas. CD30 in this variant of CBCLs may serve as a therapeutic target for anti-CD30 antibody-based strategies.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:September 2014
Deposited On:16 Oct 2014 19:21
Last Modified:05 Apr 2016 18:25
Publisher:Elsevier
ISSN:0190-9622
Publisher DOI:https://doi.org/10.1016/j.jaad.2014.03.027
PubMed ID:24836545

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