Lymphoma and plasma cell neoplasms
T / NK cell disorders
Primary cutaneous acral CD8+ T cell lymphoma

Author: Carlos Z. Murga, M.D., Lauren B. Smith, M.D. (see Authors page)

Revised: 28 March 2017, last major update August 2016

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PubMed Search: Primary cutaneous acral CD8+ T cell lymphoma

Cite this page: Primary cutaneous acral CD8+ T cell lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonbacraltcell.html. Accessed August 20th, 2017.
Epidemiology
  • Few cases have been reported
Sites
  • Dermis of nose and ear
Etiology
  • Activated mature T lymphocytes, CD3+ / CD8+ type
Clinical features
  • Painful, slowly growing skin nodules
  • Male predilection
  • Median age of diagnosis is 54 years
  • Indolent course
  • No extradermal involvement
  • Favorable outcome after local excision
Case reports
Microscopic (histologic) description
  • Dense diffuse dermal infiltrate with sparing of the epidermis and presence of a thin grenz zone
  • The infiltrate is medium to large size atypical lymphocytes (blast-like) with irregular nuclear membranes, small nucleoli and scant cytoplasm; signet ring cells can be present
  • No fat rimming, angiodestruction or necrosis
Positive stains
Negative stains
Molecular / cytogenetics description
  • Clonal TCR gene rearrangements present by PCR testing
Differential diagnosis
  • Hydroa vacciniforme-like lymphoma:
    • Can show a similar infiltrate with CD8+ cytotoxic immunophenotype, however is a EBV+ T cell lymphoproliferative disease that occurs in children (mean age 8 years)
    • The atypical infiltrate is mainly located around adnexae and vessels with angiodestruction
  • Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma:
    • Features rapidly progressing skin papules or nodules with an aggressive clinical course
    • In contrast to primary cutaneous acral CD8+ TCL, is morphologically characterized by epidermotropism with a linear or pagetoid pattern of extension
  • Primary cutaneous gamma delta T cell lymphoma:
    • Usually the tumor cells are CD4- / CD8-, however a few cases are CD4- / CD8+
    • Characteristically this disease features CD56 and TCR gamma expression
    • Morphologically has epidermotropism and can show rimming of fat cells, similar to panniculitis-like T cell lymphoma