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


CONCLUSIONS
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.
Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland; Department of Dermatology, University Hospital, Zurich, Switzerland. Electronic address: kempf@kempf-pfaltz.ch.
Werner
W
Kempf
Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland; Department of Pathology, Medical Faculty in Pilsen, Charles University, Prague, Czech Republic.
Dmitry V
DV
Kazakov
Dermatopathologie Friedrichshafen, Friedrichshafen, Germany.
Arno
A
Rütten
Derma AG, Solothurn, Switzerland; Department of Dermatology, Ludwig-Maximilians-University, Munich, Germany.
Rudolf A
RA
Rupec
Cytopathos Laboratories, Bratislava, Slovak Republic.
Petr
P
Talarcik
National Oncology Department, Bratislava, Slovak Republic.
Veronika
V
Ballová
Department of Dermatology, University Hospital, Zurich, Switzerland.
Katrin
K
Kerl
Department of Dermatology, University Hospital, Zurich, Switzerland.
Reinhard
R
Dummer
Dermatologisches Ambulatorium, Stadtspital Triemli, Zurich, Switzerland.
Stephan
S
Lautenschlager
Molecular Biology Laboratory, Department of Pathology, University Hospital Zurich, Zurich, Switzerland.
Dieter R
DR
Zimmermann
Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland.
Marianne
M
Tinguely
D000328:Adult; D000368:Aged; D001478:Basal Cell Nevus Syndrome / Q000453:epidemiology; D015897:Comorbidity; D005260:Female; D006258:Head and Neck Neoplasms / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D017730:Ki-1 Antigen / Q000378:metabolism*; D016393:Lymphoma, B-Cell / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012535:Scalp; D012878:Skin Neoplasms / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology* / Q000628:therapy
D017730:Ki-1 Antigen
CD30; Gorlin–Goltz syndrome; PTCH; cutaneous lymphoma; diffuse large B-cell lymphoma; follicular lymphoma