How I Diagnose Primary Cutaneous Marginal Zone Lymphoma

Am J Clin Pathol. 2020 Sep 8;154(4):428-449. doi: 10.1093/ajcp/aqaa116.


Objectives: Primary cutaneous marginal zone lymphoma (PCMZL) is 1 of the 3 major subtypes of primary cutaneous B-cell lymphoma. The diagnosis of PCMZL may be challenging, as the differential diagnosis includes benign cutaneous lymphoproliferations as well as other primary or secondary cutaneous B-cell or T-cell lymphomas. This review describes our approach to the diagnosis of PCMZL.

Methods: Two cases are presented that illustrate how we diagnose each of the 2 subtypes of PCMZL. The clinicopathologic features of PCMZL and the ways in which these cases can be distinguished from both benign and other neoplastic entities are emphasized.

Results: A definitive diagnosis of PCMZL requires the incorporation of histologic and immunophenotypic features, molecular genetic studies in some cases, and just as importantly, clinical findings. Emerging data suggest that the heavy chain class-switched cases may be more like a clonal chronic lymphoproliferative disorder.

Conclusions: The 2 subtypes of PCMZL create different diagnostic challenges and require the use of a multiparameter approach. Although very indolent, it is important to distinguish PCMZLs from reactive proliferations, because they frequently recur and may require antineoplastic therapies. It is also critical to distinguish PCMZLs from other B- or T-cell lymphomas so that patients are properly evaluated and not overtreated.

Keywords: Extranodal marginal zone lymphoma; Lymphoma diagnosis; MALT lymphoma; Primary cutaneous marginal zone lymphoma; Skin.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Female
  • Humans
  • Lymphoma, B-Cell, Marginal Zone / diagnosis*
  • Lymphoma, B-Cell, Marginal Zone / pathology*
  • Middle Aged
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / pathology*