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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  TCR β 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  CD 8 and  CD 56 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.

Clinicopathological analysis of 17 primary cutaneous T‐cell lymphoma of the γδ phenotype from J apan