Histologische Diagnostik, Kempf und Pfaltz, Zürich, Switzerland.
Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
J Eur Acad Dermatol Venereol. 2021 Mar;35(3):658-668. doi: 10.1111/jdv.16969. Epub 2020 Dec 22.
Cutaneous peripheral T-cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm.
The European Organization for Research and Treatment of Cancer cutaneous lymphoma taskforce (EORTC CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyse their clinical, histological, immunophenotypic features and outcome.
Retrospective analysis of clinical data and histopathological features by an expert panel.
Cutaneous PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumours or disseminated papulo-nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3 /CD4 /CD8 and CD3 /CD4 /CD8 . Moreover, 18% of the cases exhibited an aberrant expression of the B-cell marker CD20 by the tumour cells. All solitary tumours were located on the limbs and presented a high expression of GATA-3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow-up of 36 months (mean value; range 3-144). The survival rates were 61% after 3 years (CI, 43-85%) and 54% after 5 years (CI, 36-81%). Small to medium-sized morphology of tumour cells was associated with a better outcome than medium to large or large tumour cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA-3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first-line treatment for solitary lesions.
Cutaneous PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS.
未特指的皮肤外周 T 细胞淋巴瘤（PTL NOS）是一种侵袭性但特征较差的肿瘤。
欧洲癌症研究与治疗组织皮肤淋巴瘤工作组（EORTC CLTF）研究了 30 例原发性皮肤 PTL NOS 患者的 33 份活检，以分析其临床、组织学、免疫表型特征和结果。
通过专家小组对临床数据和组织病理学特征进行回顾性分析。
皮肤 PTL NOS 临床上表现为单发或播散性快速生长的溃疡性肿瘤或播散性丘疹结节性病变。组织学上，真皮和皮下组织常发现弥漫性或结节性浸润，表皮浸润很少见，且仅为轻度和局限性。不常见的表型很常见，例如 CD3/CD4/CD8 和 CD3/CD4/CD8。此外，18%的病例肿瘤细胞表达异常的 B 细胞标志物 CD20。所有单发肿瘤均位于四肢，高表达 GATA-3，但这与预后无关，因此不能作为预后因素。在 36 个月的随访中（平均值；范围 3-144），30 例患者中有 10 例（33%）死于淋巴瘤，预后一般较差。3 年后的生存率为 61%（CI，43-85%），5 年后为 54%（CI，36-81%）。肿瘤细胞的小至中等大小形态与较好的预后相关，而中等至大或大肿瘤细胞则与预后较差相关。年龄、性别、临床分期、CD4/CD8 表型和 GATA-3 表达与预后无关。化疗是最常见的治疗方式，但手术切除和/或放疗可能是单发病变的一线治疗方法。
皮肤 PTL NOS 在大多数患者中表现出侵袭性病程，与初始表现、年龄和表型无关。细胞形态学被确定为预后因素。这些数据表明，PTL NOS 需要更有效的治疗方式。
