Abstract

Background: Epstein-Barr Virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is EBV-associated clonal B-cell proliferation often occurring in patients older than 50 years without any known immunocompromised condition. Comprehensive data on cutaneous EBV-positive DLBCL, NOS has been limited. Objectives: To describe cutaneous EBV-positive DLBCL, NOS regarding clinicopathological features, immunophenotypes, and prognosis. Methods: We conducted systematic review by searching four databases. We also searched the Asan Medical Center database for cases of cutaneous EBV-positive DLBCL, NOS. To further characterize this disease, primary cutaneous EBV-positive DLBCL, NOS was compared with primary cutaneous EBV-negative DLBCL, leg type cases, extracted from Asan Medical Center database. Results: We included a total of 22 cases consisting of 17 primary cutaneous EBV-positive DLBCL, NOS and 5 concurrent skin and extracutaneous EBV-positive DLBCL, NOS. Clinical morphology of primary cutaneous EBV-positive DLBCL, NOS appeared as nodule, papule, ulcer, maculopatch and plaque and showed more various forms than EBV-negative DLBCL, leg type. Disseminated skin lesions were more common in EBV-positive DLBCL, NOS than EBV-negative DLBCL, leg type and the frequency of T3 stage was significantly higher in EBV-positive DLBCL, NOS. Histopathologically, of 17 cases with primary cutaneous EBV-positive DLBCL, NOS, 9 cases showed polymorphic subtype and 8 cases were monomorphic subtype. The frequency of positivity for CD30 and BCL-6 were significantly higher in primary cutaneous EBV-positive DLBCL, NOS than EBV-negative DLBCL, leg type. Primary cutaneous EBV-positive DLBCL, NOS tended to have a worse prognosis than EBV-negative DLBCL, leg type. Conclusion: Cutaneous EBV-positive DLBCL, NOS presents distinct clinicopathological features and has poor prognosis in comparison with primary cutaneous EBV-negative DLBCL, leg type.

Financial Disclosure:
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