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

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.

Keywords: CD30; Gorlin–Goltz syndrome; PTCH; cutaneous lymphoma; diffuse large B-cell lymphoma; follicular lymphoma.