Cutaneous Gamma-Delta T-cell Lymphoma

Cutaneous Gamma-Delta T-cell Lymphoma

Aaron Auerbach, MD, PhD

Cutaneous γδ T-cell lymphoma shows a large raised lesion with ulcer and satellite lesions. (Courtesy C. Sander, MD.)

Primary cutaneous γδ T-cell lymphoma involves the epidermis, dermis image, and subcutaneous tissue image with the most extensive disease in the dermis and subcutis. (Courtesy L. J. Medeiros, MD.)



  • Cutaneous gamma-delta T-cell lymphoma (CGDTCL)


  • Subcutaneous panniculitis-like T-cell lymphoma with γδ cells


  • T-cell lymphoma arising in the skin, which is composed of cytotoxic γδ T cells

    • Does not include subcutaneous panniculitis-like T-cell lymphoma composed of αβ cells

      • May encompass mucocutaneous γδ cell T-cell lymphoma, but further study is needed


Immunosuppression or Dysregulation of T Cells

  • Found in many of the patients

Chronic Antigenic Stimulation

  • Speculative, but possibly involved in pathogenesis

Cell of Origin

  • γδ T cells

    • Involved with mucosal and epithelial immune system function



  • Incidence

    • Rare tumor, < 100 cases reported in literature

      • < 1% of all cutaneous T-cell lymphomas

  • Age

    • Commonly adults

  • Gender

    • No gender preponderance


  • Mostly extremities

    • Sometimes mucosal sites, where normal γδ T cells are found

    • Metastasis common

      • Spread to lungs, liver, kidneys, oral mucosa, and brain

      • Usually not in bone marrow, lymph node, or spleen


  • 1 or multiple skin lesions

  • Patches or plaques due to epidermal infiltrates

    • Tumors or nodules due to dermal infiltrates

      • ± ulcerated epidermis

  • Hemophagocytic syndrome (HPS) may be present in 45% of cases

    • More often in subcutaneous lesions

      • Related to release of cytotoxic molecules

Laboratory Tests

  • Cytopenias

  • ↑ liver function tests


  • Adjuvant therapy

    • Multiagent chemotherapy ± radiotherapy

      • Poor response to allogenic stem cell transplant


  • Poor prognosis

    • 5-year survival: ˜ 11%

    • Subcutaneous disease is a poor prognostic indicator

      • Better prognosis if only disease in dermis or epidermis

    • HPS is a poor prognostic indicator


Histologic Features

  • 3 patterns of disease

    • Epidermotropic

      • Ranges from mild to marked

      • Can mimic mycosis fungoides or pagetoid reticulosis

    • Dermal

      • More dermal and epidermal involvement typically present than in subcutaneous panniculitis-like T-cell lymphoma

    • Subcutaneous

      • Lobules mostly involved

      • Septae less frequently involved and represents secondary spilling of T cells from lobules

      • Subcutis involvement can appear identical to subcutaneous panniculitis-like T-cell lymphoma of αβ cells, including rimming of fat cells

  • Often more than 1 pattern of disease in a patient

    • Different patterns of disease in a single biopsy or in different biopsies

  • Malignant T cells with nuclear atypia, hyperchromasia

  • Frequent necrosis/apoptosis and vascular invasion

    • Necrosis may be caused by released cytotoxic molecules

  • ↑ reactive histiocytes

    • Vacuolated foamy cytoplasm from imbibed material/lipid

    • With erythrophagocytosis or cytophagocytosis

Cytologic Features

  • Medium to large T cells with coarse, hyperchromatic-staining chromatin, sometimes vesicular nuclei, and variably prominent nucleoli



Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cutaneous Gamma-Delta T-cell Lymphoma

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