Aggressive Epidermotropic CD8(+) Cytotoxic T-Cell Lymphoma

 Often disseminated tumors, nodules, and papules

image Rapid spread to lungs, testes, brain, and oral cavity

image 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


• 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(+/-)

• Cytotoxic markers (+)

• Clonal for T-cell receptor gene gamma rearrangement

Top Differential Diagnoses

• Mycosis fungoides
image Is rarely CD8(+), but progression to tumor stage often takes years

• Lymphomatoid papulosis
image More benign clinical course with papules that spontaneously regress

Clinical Photograph of AECTCL
This is a clinical photograph of a large tumor on the posterior leg of a patient with aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL). As is typical of this disease, the lesion is extensively ulcerated. (Courtesy R. Willemze, MD.)

Low Magnification of AECTCL
At scanning magnification of an AECTCL, there is a dense dermal lymphoid infiltrate, which also involves the epidermis. The dermal infiltrate is angiocentric, which is a common infiltration pattern in AECTCL.

AECTCL With Epidermotropism
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.

Break-Apart *ALK* FISH Probe
This is a break-apart probe for the ALK gene by fluorescent in situ hybridization. The result is negative. No ALK gene rearrangement is seen in aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma.



• Aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL)


• Primary cutaneous AECTCL


• T-cell lymphoma consisting of CD8(+) cytotoxic αβ T cells prominently involving epidermis and exhibiting aggressive clinical behavior
image Provisional entity in 2008 WHO Classification of Hematopoietic and Lymphoid Tissues

image Previously included with cases of peripheral T-cell lymphoma, not otherwise specified

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



• Incidence
image Rare

– < 1% of cutaneous T-cell lymphomas

• Age
image Adults
– Median age: 53 years

• Sex
image M:F = 1.4:1.0


• Skin lesions
image Often tumors, nodules, and papules

– Less often patches or plaques

image Usually ulceration &/or necrosis

image Often widespread disseminated lesions

• Rapid extracutaneous spread
image Fairly frequent

image Lungs, testes, brain, and oral cavity involved

image Lymph nodes are usually spared


• Adjuvant therapy
image Often multiagent chemotherapy

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Aggressive Epidermotropic CD8(+) Cytotoxic T-Cell Lymphoma

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