2A90.6

ICD-11 MMS 2026-01

Extranodal NK/T-cell lymphoma, nasal type

Description

An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised by an often angiocentric and angiodestructive cellular infiltrate composed of Epstein-Barr Virus (EBV) positive NK/T cells. 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. It is also known as angiocentric T-cell lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic changes seen in this type of lymphoma. However, the latter term may also apply to lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive B-cell lymphoproliferative disorder.

Index terms

  • Extranodal NK/T-cell lymphoma, nasal type
  • angiocentric T-cell lymphoma
  • lethal midline granuloma
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  • malignant reticulosis
  • midline malignant reticulosis

Exclusions from above levels

  • 2E03 – Malignant neoplasm metastasis in bone or bone marrow
  • http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
  • http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

  • 2A90.0 – T-cell prolymphocytic leukaemia
  • 2A90.1 – T-cell large granular lymphocytic leukaemia
  • 2A90.2 – Chronic lymphoproliferative disorders of NK-cells
  • 2A90.3 – Aggressive NK cell leukaemia
  • 2A90.4 – Systemic Epstein-Barr Virus-positive T-cell lymphoma of childhood
  • 2A90.5 – Adult T-cell lymphoma or leukaemia, human T-cell lymphotropic virus type 1-associated
  • 2A90.7 – Enteropathy associated T-cell lymphoma
  • 2A90.8 – Hepatosplenic T-cell lymphoma
  • 2A90.9 – Angioimmunoblastic T-cell lymphoma
  • 2A90.A – Anaplastic large cell lymphoma, ALK-positive
  • 2A90.B – Anaplastic large cell lymphoma, ALK-negative
  • 2A90.C – Peripheral T-cell lymphoma, not otherwise specified

Foundation entity

Extranodal NK/T-cell lymphoma, nasal type – http://id.who.int/icd/entity/684005900

Allowed postcoordination axes

Part of: 2A90: Mature T-cell lymphoma, specified types, nodal or systemic › 02: Neoplasms