Overview

NCI Definition: 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. 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. [1]

Nasal type extranodal NK/T-cell lymphomas most frequently harbor alterations in KDM6A, ZRSR2, TET2, STAT3, and SOCS1 [2].

Most Commonly Altered Genes in Nasal Type Extranodal NK/T-Cell Lymphoma

ZRSR2 Amplification, TET2 Mutation, TET2 E846K, STAT3 Mutation, and STAT3 K658Y are the most common alterations in nasal type extranodal NK/T-cell lymphoma [2].

Top Alterations in Nasal Type Extranodal NK/T-Cell Lymphoma

Significant Genes in Nasal Type Extranodal NK/T-Cell Lymphoma

ALK +

CD274 +

Disease Details

Synonyms
Extranodal NK/T lymphoma-nasal, Angiocentric T-Cell Lymphoma, Extranodal NK-/T-Cell Lymphoma, Nasal Type, RETICULOSIS, MALIGNANT, Extranodal NK/T-Cell Lymphoma, Nasal Type, Extranodal NK/T-cell lymphoma, nasal type
Parent(s)
Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
OncoTree Name
Extranodal NK-/T-Cell Lymphoma, Nasal Type
OncoTree Code
ENKL

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.