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.

Subcutaneous panniculitis‐like T‐cell lymphoma: Pediatric case series demonstrating heterogeneous presentation and option for watchful waiting

“…Most reports of spontaneous regression have occurred in AB SPTCL . Recently, there have been reports of favorable prognosis PCGD‐TCL; however the youngest patient in the series was 17 . Case 3 demonstrates a young patient with PCGD‐TCL who had spontaneous remission during a period of watchful waiting.…”

Managing Patients with Cutaneous B-Cell and T-Cell Lymphomas Other Than Mycosis Fungoides

“…More recently, as our ability to perform gamma staining by immunohistochemistry the has become more reliable, it has been observed that there may be a less aggressive form of cutaneous gamma/delta T-cell lymphoma that has histologic features and clinical behavior skin to SPTCL. 41–43 …”

Zebras and hen's teeth: recognition and management of rare T and NK lymphomas

“…62,63 More recently, as our ability to perform gamma staining by immunohistochemistry has become more reliable, it has been observed that there may be a less aggressive form of cutaneous gamma/delta T-cell lymphoma that has histologic features of SPTCL. [64][65][66] Given the poor prognosis, aggressiveness, and poor responsiveness to conventional-dose treatments, we and others prefer to manage most patients with aggressive cutaneous gamma/delta lymphomas patients similarly to other cytotoxic T-cell lymphomas (eg, HSTCL).…”