9719/3 Extranodal NK/T-cell lymphoma, nasal type



Definitions

Skin
ICD-O-3 topography code: C44
ICD10: C86.0
           

An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterized by an often angiocentric and angiodestructive cellular infiltrate composed of EBV positive NK/T cells. Most cases appear to be genuine NK-cell neoplasms, some cases show a cytotoxic T-cell phenotype.
The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes 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



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Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C86.0
           

A predominantly extranodal lymphoma characterized by vascular damage and destruction, prominent necrosis, cytotoxic phenotype and association with Epstein-Barr virus (EBV). It is designated "NK/T" (instead of "NK"), because while most cases appear to be genuine NK-cell neoplasms, some cases show a cytotoxic T-cell phenotype.







Nasopharynx
ICD-O-3 topography code: C11
ICD10: C86.0
           

An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterized by an often angiocentric and angiodestructive cellular infiltrate composed of EBV positive NK/T cells. Most cases appear to be genuine NK-cell neoplasms, some cases show a cytotoxic T-cell phenotype.
The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes
4
 
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



.



Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C86.0
           

/span> predominantly extranodal lymphoma characterized by vascular damage and destruction, prominent necrosis, cytotoxic phenotype and association with Epstein-Barr virus (EBV). It is designated "NK/T" (instead of "NK"), because while most cases appear to be genuine NK-cell neoplasms, some cases show a cytotoxic T-cell phenotype.


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