Case Histories

Subcutaneous Panniculitis-like T-cell lymphoma
Dennis B. Cornfield, MD
Chief, Section of Hematopathology & Clinical Laboratory Medicine Department of Pathology & Laboratory Medicine

 

Presenting Case
  • Patient is a 26 year-old woman who presented for:
  • Incision and debridement of 9 cm area of induration and erythema, left lateral thigh
  • Initial microscopic diagnosis: inflammation and fat necrosis
  • Patient subsequently had excision of a 2.7 cm subcutaneous mass, left groin

    • Interstitial infiltration of adipose tissue
    by mononuclear cells    		  
    Infiltrate consists of lymphocytes with irregular
    nuclear contours and some scattered histiocytes.
    Lymphocytes tend to form rim around fat cells.
         
         

    Lymphocytes are predominantly CD3+
    T-cells    		  
    T lymphocytes express the cytotoxic molecule Granzyme B (shown)
    as well as T-cell receptor alpha/beta (not shown)

    Molecular Analysis: Positive for a clonal T-cell receptor gene rearrangement, consistent with the presence of a monoclonal population of T lymphocytes.

    Diagnosis:

    Subcutaneous panniculitis-like T-cell lymphoma, TCR α/β type

    Treatment:
  • Ontak (denileukin diftitox): recombinant fusion protein consisting of amino acid sequences of IL-2 and diphtheria toxin
  • Given days 1-5 i-v q3 wks x 4 courses
  • Patient was NED by PE, labs, and PET/CT at 2 months follow up

    • WHO-EORTC classification of cutaneous lymphomas with primary cutaneous manifestations

    Cutaneous T-cell and NK-cell lymphomas
    Mycosis Fungoides
    MF variants and subtypes
    Sezary syndrome
    Adult T-cell leukemia/lymphoma
    Primary cutaneous CD30+ lymphoproliferative disorders
       – Primary cutaneous anaplastic large cell lymphoma
       – Lymphomatoid papulosis
    Subcutaneous panniculitis-like T-cell lymphoma (present case)
    Extranodal NK/T-cell lymphoma, nasal type
    Primary cutaneous peripheral T-cell lymphoma, rare subtypes:
       – 1° cutan. aggressive epidermotropic CD8+ T-cell lymphoma (provisional)
       – Cutaneous γδ T-cell lymphoma
       – Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (provisional)

     

    Interesting Case Features
    1. This is a rare type of lymphoma (<1%) which usually occurs in young adults.
    2. Diagnosis is often delayed because the clinical presentation and histologic findings mimic
    inflammation of fat (panniculitis)
    3. Cases with the alpha/beta type of T-cell receptor (TCR) comprise the majority of cases
    and have a considerably better survival than those derived from T-cells with the
    gamma/delta TCR.

     

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