9709/3 Cutaneous T-cell lymphoma, NOS


Synonyms

Cutaneous lymphoma, NOS

Definitions

Skin
ICD-O-3 topography code: C44
ICD10: C84.5
           

Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma CTCL) is characterized by proliferation of epidermotropic CD8-positive cytotoxic T-cells and aggressive clinical behaviour 1
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



2
Click to access Pubmed
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R (1999)
Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior.
Am J Pathol 155: 483-92



.
This lymphoma accounts for less than 1% of all CTCL and occurs mainly in adults. Most patients present with localized or disseminated eruptive papules, nodules and tumours with central ulceration and necrosis. Dissemination to other sites may occur, incl. lung, testis, central nervous system and oral mucosa
3
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



4
Click to access Pubmed
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R (1999)
Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior.
Am J Pathol 155: 483-92



.



Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C84.5
           

/span>his provisional entity is a cutaneous T-cell lymphoma (CTCL) characterized by proliferation of epidermotropic CD8-positive cytotoxic T-cells and aggressive clinical behaviour. Differentiation from other types of CTCL expressing a CD8-positive cytotoxic T-cell phenotype is based on the clinical presentation, clinical behaviour and certain histological features, such as marked epidermotropism with epidermal necrosis.

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