NCI Definition: A heterogenous category of nodal and extranodal mature T-cell lymphomas that do not correspond to any of the specifically defined entities of mature T-cell lymphoma in the 2017 WHO classification. Excluded from this category are tumors with a T follicular helper (TFH) cell phenotype. Variants include lymphoepithelioid lymphoma (Lennert lymphoma) and primary EBV-positive nodal T-cell or NK-cell lymphoma. The follicular variant included in the peripheral T-cell lymphomas, not otherwise specified, in the 2008 edition of the WHO classification has been moved to the category of angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin in the 2017 WHO update. The same is true for a proportion of cases previously designated as the T-zone variant, because they usually have a TFH-cell phenotype. (WHO 2017) [1]

Peripheral T-cell lymphoma, not otherwise specifieds most frequently harbor alterations in TET2, TP53, RHOA, DNMT3A, and CDKN2A [2].

Most Commonly Altered Genes in Peripheral T-Cell Lymphoma, Not Otherwise Specified

TET2 Mutation, RHOA Mutation, TP53 Mutation, RHOA G17V, and TET2fs are the most common alterations in peripheral T-cell lymphoma, not otherwise specified [2].

Top Alterations in Peripheral T-Cell Lymphoma, Not Otherwise Specified

Significant Genes in Peripheral T-Cell Lymphoma, Not Otherwise Specified




CARD11 +

CD79A +

CD79B +



Disease Details

Peripheral T-Cell Lymphoma, NOS, Peripheral T-cell lymphoma, NOS
Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
OncoTree Name
Peripheral T-Cell Lymphoma, NOS
OncoTree Code


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.