9708/3 Subcutaneous panniculitis-like T-cell lymphoma


Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C86.3
           

/span>ubcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue. It is composed of atypical lymphoid cells of varying size, typically with karyorrhexis of tumour cells and associated fat necrosis. In contrast to the third edition of the WHO classification, cases expressing the γδ T-cell receptor are excluded, and now reclassified as primary cutaneous gamma-delta T-cell lymphoma.

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Skin
ICD-O-3 topography code: C44
ICD10: C86.3
           

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a cytotoxic T-cell lymphoma that preferentially infiltrates subcutaneous tissue. It is composed of atypical lymphoid cells, typically with karyorrhexis of tumour cells and associated fat necrosis
6
 
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



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SPTCL is a rare lymphoma, representing <1% of all non-Hodgkin lymphomas. Approximately 20% of patients are under the age of 20 (median, 35 years) and present with multiple subcutaneous nodules. Up to 20% of patients may have associated autoimmune disease, most commonly systemic lupus erythematosus (SLE)
7
Click to access Pubmed
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ (2005)
WHO-EORTC classification for cutaneous lymphomas.
Blood 105: 3768-85



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In contrast to the third edition of the WHO classification
8
 
Jaffe ES, Harris NL, Stein H, Vardiman JW (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues.
3rd Edition
IARC Press: Lyon 2001



9
Click to access Pubmed
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ (2005)
WHO-EORTC classification for cutaneous lymphomas.
Blood 105: 3768-85



, cases expressing the gamma-delta T-cell receptor are excluded and now 10
 
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



reclassified as primary cutaneous gamma-delta T-cell lymphoma.