Werner Kempf1, Dmitry V Kazakov2, Arno Rütten3, Rudolf A Rupec4, Petr Talarcik5, Veronika Ballová6, Katrin Kerl7, Reinhard Dummer7, Stephan Lautenschlager8, Dieter R Zimmermann9, Marianne Tinguely10. 1. Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland; Department of Dermatology, University Hospital, Zurich, Switzerland. Electronic address: kempf@kempf-pfaltz.ch. 2. Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland; Department of Pathology, Medical Faculty in Pilsen, Charles University, Prague, Czech Republic. 3. Dermatopathologie Friedrichshafen, Friedrichshafen, Germany. 4. Derma AG, Solothurn, Switzerland; Department of Dermatology, Ludwig-Maximilians-University, Munich, Germany. 5. Cytopathos Laboratories, Bratislava, Slovak Republic. 6. National Oncology Department, Bratislava, Slovak Republic. 7. Department of Dermatology, University Hospital, Zurich, Switzerland. 8. Dermatologisches Ambulatorium, Stadtspital Triemli, Zurich, Switzerland. 9. Molecular Biology Laboratory, Department of Pathology, University Hospital Zurich, Zurich, Switzerland. 10. Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland.
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.
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.