Often disseminated tumors, nodules, and papules
Rapid spread to lungs, testes, brain, and oral cavity
Often treated with multiagent chemotherapy
•
Often spares lymph nodes
•
Rare compared to other cutaneous T-cell lymphomas, such as mycosis fungoides and anaplastic large cell lymphoma
Microscopic
•
Epidermotropic T-cell infiltrate
•
Variably sized T cells with moderate to marked atypia
•
May extend into dermis &/or subcutaneous tissue
•
Ulceration, necrosis, and angioinvasion are often identified
Ancillary Tests
•
CD8(+), CD3(+), CD2(-/+), CD5(-), CD7(+/-), βF1(+), CD4(-), LMP1(-), CD15(-/+), CD56(+/-)
•
Clonal for T-cell receptor gene gamma rearrangement
Top Differential Diagnoses
•
Mycosis fungoides
Is rarely CD8(+), but progression to tumor stage often takes years
•
Lymphomatoid papulosis
More benign clinical course with papules that spontaneously regress
TERMINOLOGY
Abbreviations
•
Aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL)
Synonyms
•
Primary cutaneous AECTCL
Definitions
•
T-cell lymphoma consisting of CD8(+) cytotoxic αβ T cells prominently involving epidermis and exhibiting aggressive clinical behavior
Provisional entity in 2008 WHO Classification of Hematopoietic and Lymphoid Tissues
Previously included with cases of peripheral T-cell lymphoma, not otherwise specified
Often separated from other CD8(+) lymphomas based on clinical behavior
CLINICAL ISSUES
Epidemiology
•
Incidence
Rare
–
< 1% of cutaneous T-cell lymphomas
•
Age
Adults
•
Sex
M:F = 1.4:1.0
Presentation
•
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
Treatment
•
Adjuvant therapy
Often multiagent chemotherapy