Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma

Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma

Aaron Auerbach, MD, PhD

This is a clinical photograph of a large tumor on the posterior leg of a person with AECTCL. As is typical of this disease, the lesion is extensively ulcerated. (Courtesy R. Willemze, MD.)

This is a medium magnification of a case of AECTCL with considerable epidermotropism image. The T cells in the epidermis are medium in size with moderate atypia and coarse chromatin.



  • Aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL)


  • Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma


  • T-cell lymphoma consisting of CD8(+) cytotoxic αβ T cells prominently involving the epidermis and exhibiting aggressive clinical behavior

    • Provisional entity in the 2008 WHO Classification of Hematopoietic and Lymphoid Tissues

    • Previously included in cases of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS)

    • Often separated from other CD8(+) lymphomas based on clinical behavior



  • Incidence

    • Rare

      • < 1% of cutaneous T-cell lymphomas

  • Age

    • Adults

      • Median age: 53

  • Gender

    • Male:female = 1.4:1.0


  • Skin lesions

    • Often tumors, nodules, and papules

      • Less often patches or plaques

    • Usually ulceration &/or necrosis

    • Often widespread disseminated lesions

  • Rapid extracutaneous spread

    • Fairly frequent

    • Lungs, testes, brain, and oral cavity involved

    • Lymph nodes are usually spared


  • Adjuvant therapy

    • Often multiagent chemotherapy


  • Aggressive clinical course

    • 32-month median survival

    • No difference in prognosis if tumor cells are smaller or larger

    • 1 study suggests that CD2(−)/CD7(+) cases have worse prognosis


Histologic Features

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma
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