Extranodal NK-T-cell lymphoma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Extra nodal NK-T-cell lymphoma, nasal type, can develop in either T cells or natural killer cells, but most often in the NK cells. Natural killer cells are a type of lymphocyte that are closely related to T cells and attack foreign cells. Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extra nodal NK-T-cell lymphoma, nasal type. Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into extranodal NK-T-cell lymphoma, nasal type and extranodal NK-T-cell lymphoma, extra nasal type. On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma. On microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of nonneoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.There are no established causes for extranodal NK-T-cell lymphoma. Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, and peripheral T cell lymphoma.Patients of all age groups may develop extranodal NK-T-cell lymphoma. Males are more commonly affected with extranodal NK-T-cell lymphoma than females. The majority of extranodal NK-T-cell lymphoma cases are reported in Asian, Central-American, and South-American countries.There are no established risk factors for extranodal NK-T-cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.

Classification

Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extra nodal NK-T-cell lymphoma, nasal type. Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into extranodal NK-T-cell lymphoma, nasal type and extranodal NK-T-cell lymphoma, extra nasal type.

Pathophysiology

Extra nodal NK-T-cell lymphoma, nasal type, can develop in either T cells or natural killer cells, but most often in the NK cells. Natural killer cells are a type of lymphocyte that are closely related to T cells and attack foreign cells. On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma. On microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of nonneoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.

Causes

There are no established causes for extranodal NK-T-cell lymphoma.

Differential Diagnosis

Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, and peripheral T cell lymphoma.

Epidemiology and Demographics

Patients of all age groups may develop extranodal NK-T-cell lymphoma. Males are more commonly affected with extranodal NK-T-cell lymphoma than females. The majority of extranodal NK-T-cell lymphoma cases are reported in Asian, Central-American, and South-American countries.

Risk Factors

There are no established risk factors for extranodal NK-T-cell lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.