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




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(+/-)

• 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

image
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.)


image
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.

image
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.

image
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.


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
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


CLINICAL ISSUES


Epidemiology




• Incidence
image Rare

– < 1% of cutaneous T-cell lymphomas

• Age
image Adults
– Median age: 53 years

• Sex
image M:F = 1.4:1.0


Presentation




• 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


Treatment




• 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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