Department of Pathology, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan.
Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.
J Cutan Pathol. 2022 Apr;49(4):350-357. doi: 10.1111/cup.14169. Epub 2021 Nov 30.
Primary cutaneous gamma/delta T-cell lymphoma (PCDG-TCL) is aggressive, frequently presenting as multiple plaques, tumors, and/or subcutaneous nodules.
In this study, we conducted a retrospective study in a tertiary center in Taiwan to characterize this rare tumor.
We identified six patients. Five presented with a solitary lesion, including two with clinical impression of epidermal inclusion cyst or lipoma. Two of four evaluable cases exhibited epidermotropism, with one mimicking Pautrier microabscess. The neoplastic cells were pleomorphic and mostly medium- to large-sized. In all cases, the neoplastic cells expressed T-cell receptor (TCR)-γ and/or TCR-δ, with four co-expressing βF1. Two of these βF1+ cases co-expressed TCR-γ but not TCR-δ (two different clones). All were negative for Epstein-Barr virus (EBV), low stage, and treated with radiotherapy alone or combined chemotherapy and radiotherapy. In two patients, lymphoma relapsed in 3 and 7 months, respectively, and one patient died of the disease in 7 months. Four other patients were free of disease for 6 to 126 months.
PCGD-TCL cases in Taiwan are more commonly solitary, frequently with indolent courses. The two currently available TCR-δ clones alone might be insufficient to detect all tumors.
原发性皮肤 γ/δ T 细胞淋巴瘤（PCDG-TCL）侵袭性强，常表现为多个斑块、肿瘤和/或皮下结节。
本研究在台湾的一家三级中心进行了回顾性研究，以描述这种罕见的肿瘤。
我们共鉴定了 6 例患者。5 例患者表现为单发皮损，其中 2 例临床印象为表皮包涵囊肿或脂肪瘤。4 例可评估病例中的 2 例存在表皮亲润，其中 1 例类似于 Pautrier 微脓肿。肿瘤细胞多形性，大小中等至大型。所有病例均表达 T 细胞受体（TCR）-γ 和/或 TCR-δ，其中 4 例同时表达βF1。这 2 例βF1+病例中，有 1 例同时表达 TCR-γ但不表达 TCR-δ（两种不同的克隆）。所有病例均为 EBV 阴性，分期低，单独接受放疗或联合化疗和放疗。2 例患者分别在 3 个月和 7 个月时复发，1 例患者在 7 个月时死于该病。其他 4 例患者无疾病生存 6 至 126 个月。
台湾的 PCDG-TCL 病例更常见为单发，常呈惰性病程。目前可用的两种 TCR-δ 克隆可能不足以检测所有肿瘤。
