Cutaneous Marginal Zone B-cell Lymphoma

Cutaneous Marginal Zone B-cell Lymphoma

Aaron Auerbach, MD, PhD

Low magnification of a primary cutaneous MZL shows a prominent, diffuse dermal lymphoid infiltrate, which infiltrates between collagen bundles image and is separated from the epidermis by a grenz zone image.

This is another case of cutaneous marginal zone B-cell lymphoma that is extending into the subcutaneous tissue with a nodular and diffuse growth pattern.



  • Primary cutaneous marginal zone lymphoma (PCMZL)


  • Primary cutaneous immunocytoma


  • Non-Hodgkin small B-cell lymphoma of centrocyte-like marginal zone cells

    • Recapitulates architecture of Peyer patches

      • Usually in sites without significant lymphoid tissue

    • Malignant B cells expanding marginal zones surrounding reactive follicles and colonizing them

  • May be difficult to diagnose because of nonspecificity of many of the morphologic features, absence of 1 or more classic features, and nature of the small biopsies


Infectious Agents

  • Borrelia burgdorferi DNA found in skin in some European cases, but not in other populations

    • Other infectious diseases noted in tissue other than skin

      • Helicobacter pylori implicated in gastric marginal zone lymphoma

      • Campylobacter jejuni implicated in small intestine marginal zone lymphoma

      • Chlamydia psittaci implicated in ocular marginal zone lymphoma

Autoimmune Diseases

  • Marginal zone lymphoma has been associated with patients who have autoimmune diseases

    • Sjögren syndrome and parotid gland marginal zone lymphoma

    • Hashimoto thyroiditis and thyroid marginal zone lymphoma



  • Age

    • Usually adults > 40 years old

  • Gender

    • No gender predominance

  • Ethnicity

    • No ethnic propensity


  • Mostly upper extremity

    • Also head and neck and torso


  • 1 or more plaques or nodules

    • Rarely ulcerates

    • Multiple nodules often seen in systemic marginal zone lymphoma with secondary spread to dermis


  • Excision or radiation therapy

    • If solitary or only a few lesions

      • Chemotherapy only considered for widespread disease, metastasis

  • Antibiotics

    • Sometimes therapy for Borrelia burgdorferi infection


  • Excellent

    • 5-year survival: 90-100%

    • But tendency to recur

      • Rarely transforms to large B-cell lymphoma

      • So look for sheets of large B cells for diagnosis of large B-cell lymphoma

      • Scattered large B cells are often seen in PCMZL


General Features

  • 1 or more firm red nodules on skin


Histologic Features

  • Nodular to diffuse dermal infiltrate

    • Spares epidermis with grenz zone

    • Sometimes involves subcutis

    • Malignant B cells expanding marginal zones surrounding reactive follicles and colonizing them

  • Lymphoepithelial lesions (very rare in skin)

  • Neoplastic cells

    • Sometimes monocytoid with ample pale/clear cytoplasm

    • Sometimes plasmacytoid with eccentric nucleus and pink cytoplasm

    • Variable numbers of plasma cells, neutrophils, and eosinophils

    • Mostly small in size with coarse chromatin and mild atypia

  • Dutcher bodies (intranuclear inclusions)

    • Sometimes seen

    • More often in marginal zone lymphoma than plasmacytoma

    • Russell bodies are cytoplasmic inclusions that may also be seen, but are less specific

  • Reactive germinal centers

    • Colonized by neoplastic marginal zone B cells

    • Still contain tingible body macrophages

    • Retain mantle zones

      • If tissue is small, may not see follicles

  • Follicular dendric cell meshworks

    • Often disrupted/loosened in colonized germinal centers

  • Lymphoepithelial lesions

    • 3 or more lymphocytes entering epithelial structure (usually a gland) and destroying it

    • Very rarely seen in skin

Cytologic Features

  • Small to medium-sized B cells with mild atypia, 1 or more inconspicuous nucleoli, and scant pale cytoplasm



Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cutaneous Marginal Zone B-cell Lymphoma
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