title: Clinicopathological analysis of 17 primary cutaneous T-cell lymphoma of the γδ phenotype from Japan.
abstract: Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is an aggressive lymphoma consisting of clonal proliferation of mature activated γδ T-cells of a cytotoxic phenotype. Because primary cutaneous γδ T-cell lymphoma is a rare disease, there are few clinicopathological studies. In addition, T-cell receptor (TCR) γδ cells are typically immunostained in frozen sections or determined by TCRβ negativity. We retrospectively analyzed 17 primary cutaneous T-cell lymphomas of the γδ phenotype (CTCL-γδ) in a clinicopathological and molecular study using paraffin-embedded sections. Among 17 patients, 11 had CTCL-γδ without subcutaneous panniculitis-like T-cell lymphoma (SPTCL) features and six had CTCL-γδ with SPTCL features. Immunophenotypically, some significant differences were found in CD8 and CD56 positivity between our patient series of CTCL-γδ patients with SPTCL features and SPTCL-γδ patients described in the previous literature. A univariate analysis of 17 CTCL-γδ patients showed that being more than 60 years old, presence of visceral organ involvement, and small-to-medium cell size were poor prognostic factors. In addition, the 5-year overall survival rate was 42.4% for the CTCL-γδ patients without SPTCL features and 80.0% for those with SPTCL features. Consequently, there was a strikingly significant difference in overall survival among SPTCL, CTCL-γδ with SPTCL features and CTCL-γδ without SPTCL features (P = 0.0005). Our data suggests that an indolent subgroup may exist in CTCL-γδ. Studies on more cases, including those from other countries, are warranted to delineate the clinicopathological features and the significance in these rare lymphomas.
authors.affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.forename: Seiichi
authors.initials: S
authors.lastname: Kato
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Sato
authors.forename: Naoko
authors.initials: N
authors.lastname: Asano
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Ogino
authors.forename: Kimio
authors.initials: K
authors.lastname: Hashimoto
authors.forename: Yukie
authors.initials: Y
authors.lastname: Tashiro
authors.forename: Shogo
authors.initials: S
authors.lastname: Takeuchi
authors.forename: Taro
authors.initials: T
authors.lastname: Masunari
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Hiramatsu
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Maeda
authors.forename: Mitsune
authors.initials: M
authors.lastname: Tanimoto
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007564:Japan; D016410:Lymphoma, T-Cell, Cutaneous / Q000378:metabolism / Q000401:mortality* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016612:Paraffin Embedding; D011379:Prognosis; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000378:metabolism / Q000401:mortality* / Q000473:pathology*
chemical_list: D011948:Receptors, Antigen, T-Cell
keywords: Cutaneous γδ T-cell lymphoma; T-cell receptor αβ; T-cell receptor γδ; indolent clinical behavior; subcutaneous panniculitis-like T-cell features