NCI Definition: An aggressive cutaneous lymphoma of mature, activated gamma/delta T-lymphocytes. It usually presents with disseminated plaques and nodules. Involvement of mucosal sites is frequent. However, involvement of lymph nodes, spleen, or bone marrow is uncommon. Morphologically, there are three patterns of cutaneous involvement: epidermotropic, dermal, and subcutaneous. Often, more than one pattern may co-exist in a single biopsy specimen, or may be present in different biopsy specimens from the same patient. The lymphocytic infiltrate is composed of medium- to large-sized lymphocytes expressing CD56. Most cases lack both CD4 and CD8, although CD8 may be present in some cases. This group of lymphomas includes cases previously known as subcutaneous panniculitis-like T-cell lymphoma with a gamma/delta phenotype. [1]

Primary cutaneous gamma-delta T-cell lymphomas most frequently harbor alterations in ROBO1, CDKN2B, CDKN2A, XPO1, and TSC1 [2].

Most Commonly Altered Genes in Primary Cutaneous Gamma-Delta T-Cell Lymphoma

CDKN2B Loss, CDKN2A Loss, XPO1 Amplification, TSC1 S505L, and TSC1 Mutation are the most common alterations in primary cutaneous gamma-delta T-cell lymphoma [2].

Top Alterations in Primary Cutaneous Gamma-Delta T-Cell Lymphoma

Disease Details

Cutaneous Gamma/Delta T-Cell Lymphoma, Primary Cutaneous Gamma Delta T-Cell Lymphoma, Peripheral Gamma-Delta T-Cell Lymphoma
Primary Cutaneous T-Cell Non-Hodgkin Lymphoma
OncoTree Name
Primary Cutaneous Gamma Delta T-Cell Lymphoma
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.