Preferred Label : Primary Cutaneous Gamma-Delta T-Cell Lymphoma;
NCIt synonyms : Cutaneous Gamma/Delta T-Cell Lymphoma; Peripheral Gamma-Delta T-Cell Lymphoma; Cutaneous Gamma Delta T-Cell Lymphoma; PCGD-TCL;
NCIt 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.;
Neoplastic status : Malignant;
ICD-O code : 9726/3;
Origin ID : C45340;
UMLS CUI : C1707547;
Automatic exact mappings (from CISMeF team)
- Disease excludes abnormal cell
- Disease excludes normal cell origin
- Disease may have findings
- Excludes anatomical site(s)
- Has associated anatomic sites
- Semantic type(s)
- UMLS correspondences (same concept)
- concept_is_in_subset
- disease_excludes_finding
- disease_has_abnormal_cell
- disease_has_finding
- disease_has_molecular_abnormality
- disease_has_normal_cell_origin
- disease_has_normal_tissue_origin
- disease_has_primary_anatomic_site
- disease_may_have_associated_disease
