title: Primary Cutaneous T-Cell Lymphomas Showing Gamma-Delta (γδ) Phenotype and Predominantly Epidermotropic Pattern are Clinicopathologically Distinct From Classic Primary Cutaneous γδ T-Cell Lymphomas.
abstract: Primary cutaneous gamma-delta (γδ) T-cell lymphoma is a rare disease that typically involves the dermis and subcutis. Cases of primary cutaneous T-cell lymphomas showing γδ phenotype and predominantly epidermotropic pattern (EγδTCL) are not well defined. In this series, cases of primary cutaneous T-cell lymphomas showing γδ phenotype were reviewed and classified as predominantly epidermotropic (EγδTCL) when >75% of lymphoma cells resided in the epidermis or predominantly dermal and/or subcutaneous (DSγδTCL). Clinical, pathologic, and immunophenotypic features were compared in 27 biopsies from 13 patients of EγδTCL and 13 biopsies from 7 patients of DSγδTCL. The lymphoma cells were diffusely positive for CD3 and T-cell receptor (TCR)γ, mostly positive for granzyme B and TIA-1, variably positive for CD8, CD7, and CD30, and negative for CD4 and TCRβ. Two patients with EγδTCL had dissemination to lymph nodes and 1 to the lung; 1 patient with DSγδTCL had gastrointestinal involvement. The median survival of patients with EγδTCL was not reached, and with a median follow-up of 19.2 months, 3/13 died. In contrast, the median survival of patients with DSγδTCL was 10 months, and after a median follow-up of 15.6 months, 5/5 died (P<0.01). EγδTCL is a rare presentation of cutaneous T-cell lymphoma that can be distinguished from DSγδTCL based on the extent of epidermotropism and has a better prognosis and longer median survival than DSγδTCL. However, although EγδTCL resembles mycosis fungoides clinically and histologically, a subset of EγδTCL is more likely to behave more aggressively than typical mycosis fungoides.
authors.affiliation: Departments of *Pathology ‡Hematopathology §Dermatology, The University of Texas MD Anderson Cancer Center, Houston, TX †University of Missouri- Kansas City School of Medicine, Kansas City, MO.
authors.forename: E Dean
authors.initials: ED
authors.lastname: Merrill
authors.forename: Rose
authors.initials: R
authors.lastname: Agbay
authors.forename: Roberto N
authors.initials: RN
authors.lastname: Miranda
authors.forename: Phyu P
authors.initials: PP
authors.lastname: Aung
authors.forename: Michael T
authors.initials: MT
authors.lastname: Tetzlaff
authors.forename: Ken H
authors.initials: KH
authors.lastname: Young
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Curry
authors.forename: Priyadharsini
authors.initials: P
authors.lastname: Nagarajan
authors.forename: Doina
authors.initials: D
authors.lastname: Ivan
authors.forename: Victor G
authors.initials: VG
authors.lastname: Prieto
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
authors.forename: Carlos A
authors.initials: CA
authors.lastname: Torres-Cabala
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D020405:Dermis / Q000473:pathology; D004817:Epidermis / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007223:Infant; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000276:immunology; D012878:Skin Neoplasms / Q000473:pathology*; D040521:Subcutaneous Tissue / Q000473:pathology; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D016692:Receptors, Antigen, T-Cell, gamma-delta