cutaneous marginal zone B-cell lymphoma

Cutaneous marginal zone B-cell lymphoma is a recently proposed entity and constitutes a cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT).

Although its molecular pathogenesis is currently unknown, an etiological link with Borrelia burgdorferi infection has been identified in European, but not in American or Asian cases.

Primary cutaneous marginal zone B-cell lymphomas from the three geographical regions have common clinicopathological features, however, moderate/marked tissue eosinophilia is a feature found almost exclusively in Asian cases. Borrelia infection and API2-MALT1 fusion are not significant in this tumor. (18820662)

Methylation of DAPK and p16(INK4a) genes is a frequent event in this lymphoma at its initial presentation, but may not be associated with tumor progression. (18820662)

Etiology

 Borrelia burgdorferi infection (?)

References

 Cutaneous marginal zone lymphomas have distinctive features and include 2 subsets. Edinger JT, Kant JA, Swerdlow SH. Am J Surg Pathol. 2010 Dec;34(12):1830-41. PMID: 21107089

 CD123-positive plasmacytoid dendritic cells in primary cutaneous marginal zone B-cell lymphoma: diagnostic and pathogenetic implications. Kutzner H, Kerl H, Pfaltz MC, Kempf W. Am J Surg Pathol. 2009 Sep;33(9):1307-13. PMID: 19718787

 Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathological study of cases from Asia, Germany, and the United States. Takino H, Li C, Hu S, Kuo TT, Geissinger E, Muller-Hermelink HK, Kim B, Swerdlow SH, Inagaki H. Mod Pathol. 2008 Sep 26. PMID: 18820662

 Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathologic study of 24 asian cases. Li C, Inagaki H, Kuo TT, Hu S, Okabe M, Eimoto T. Am J Surg Pathol. 2003 Aug;27(8):1061-9. PMID: 12883238

 Cutaneous lymphoid hyperplasia and cutaneous marginal zone lymphoma: comparison of morphologic and immunophenotypic features. Baldassano MF, Bailey EM, Ferry JA, Harris NL, Duncan LM. Am J Surg Pathol. 1999 Jan;23(1):88-96. PMID: 9888708