Clinicopathologic, immunophenotypic, and molecular characterization of primary cutaneous follicular B-cell lymphoma

Objectives: To determine the clinicopathologic, immunophenotypic, and molec ular characteristics of primary follicular cutaneous B-cell lymphoma (CBCL) as defined by the revised European-American lymphoma classification. Design: A retrospective survey of the medical records, an immunohistochemic al study of archival biopsy specimens. and molecular studies of preserved D NA of all patients with follicle center lymphoma-follicular (FCL-F) primary CBCL from 1987 to 1997. Setting: A single-center outpatient specialty clinic at an academic medical center. Patients: Twenty-one patients (68% of all new primary CBCL cases), includin g 14 men and 7 women (age range, 33-88 years; mean, 55 years). Results: The head and neck region was the most frequent primary site. Follo wing treatment, recurrences were relatively frequent, but the overall morta lity rate during 1.0 to 11.3 years (mean, 6.3 years) of follow-up was 4.8%. Immunohistochemical analysis for B- and T-cell lineages was helpful in enh ancing the folliclelike structures. CD10, bcl-2, and CD43 were expressed by the neoplastic cells in 9 (47%) of 19 cases, 4 (21%) of 19 cases, and 2 (1 3%) of 16 cases, respectively. Immunohistochemical detection of cytoplasmic immunoglobulin light chains, using steaming in EDTA as the antigen-retriev al technique, was successful in 12 (71%) of 17 cases. The 1g heavy-chain ge ne rearrangements, using the Southern blot technique, detected clonality in 17 (94%) of 18 cases. The bcl-2 gene rearrangements were detected in only 2 (13%) of 15 of the primary cutaneous FCL-F cases, compared with 9 (75%) o f 12 of the primary nodal FCL-F cases (P=.002). Conclusions: Primary cutaneous FCL-F is a relatively common subtype of CBCL , with a relatively indolent course. It has many features in common with pr imary nodal FCL-F. except for low rates of bcl-2 expression and bcl-2 gene rearrangements.