9766/1 Angiocentric immunoproliferative lesion


Synonyms

Lymphomatoid granulomatosis

Definitions

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

/span>ymphomatoid granulomatosis (LYG) is an angiocentric and angiodestructive lymphoproliferative disease involving extra?nodal sites, composed of Epstein-Barr virus (EBV)-positive B cells admixed with reactive T cells, which usually predominate. The lesion has a spectrum of histological grade and clinical aggressiveness, which is related to the proportion of large B cells.

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Bronchus and lung
ICD-O-3 topography code: C34

Lymphomatoid granulomatosis (LYG) is a rare extranodal neoplastic lymphoproliferative disease characterized by an angiocentric arrangement of tumor cells which is associated with angiodestruction. Tumour cells are Epstein-Barr virus (EBV)-positive B cells admixed with reactive T cells
2
 
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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Wester populations and males are more frequently affected and immunodeficiency is a risk factor. Predisposing conditions include allogeneic organ transplantation, Wiskott-Aldrich syndrome, human immunodeficiency virus (HIV) infection and X-linked lymphoproliferative syndrome .



Skin
ICD-O-3 topography code: C44

Lymphomatoid granulomatosis (LYG) is a rare extranodal neoplastic lymphoproliferative disease characterized by an angiocentric arrangement of tumor cells which is associated with angiodestruction. Tumour cells are Epstein-Barr virus (EBV)-positive B cells admixed with reactive T cells
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



.
Wester populations and males are more frequently affected and immunodeficiency is a risk factor. Predisposing conditions include allogeneic organ transplantation, Wiskott-Aldrich syndrome, human immunodeficiency virus (HIV) infection and X-linked lymphoproliferative syndrome .