Extranodal marginal zone lymphoma from ocular adnexae with subcutaneous involvement

Am J Dermatopathol. 2014 Nov;36(11):e189-93. doi: 10.1097/DAD.0000000000000050.


Extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue usually originates from cutaneous or mucosal surfaces. A rare site of involvement is the subcutaneous tissue of any location. Here, we describe a 58-year-old man who presented with bilateral extranodal MZL of mucosa-associated lymphoid tissue from ocular adnexae that involved subcutaneous tissue and subsequently extended to multiple anatomical locations in the head and neck, upper back, and arm. The neoplastic cells expressed B-cell markers, and the plasma cells expressed IgG4. The unusual pattern of infiltration of this extranodal MZL and the possible significance of IgG4 expression in this case are discussed.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers, Tumor / analysis
  • Biopsy
  • Eye Neoplasms / chemistry
  • Eye Neoplasms / drug therapy
  • Eye Neoplasms / immunology
  • Eye Neoplasms / pathology*
  • Humans
  • Immunoglobulin G / analysis
  • Immunohistochemistry
  • Lymphoma, B-Cell, Marginal Zone / chemistry
  • Lymphoma, B-Cell, Marginal Zone / drug therapy
  • Lymphoma, B-Cell, Marginal Zone / immunology
  • Lymphoma, B-Cell, Marginal Zone / pathology*
  • Male
  • Middle Aged
  • Neoplasm Staging
  • Neoplasms, Adnexal and Skin Appendage / chemistry
  • Neoplasms, Adnexal and Skin Appendage / drug therapy
  • Neoplasms, Adnexal and Skin Appendage / immunology
  • Neoplasms, Adnexal and Skin Appendage / secondary*
  • Subcutaneous Tissue / chemistry
  • Subcutaneous Tissue / immunology
  • Subcutaneous Tissue / pathology*
  • Treatment Outcome


  • Biomarkers, Tumor
  • Immunoglobulin G