Wobser Marion, Reinartz Theresa, Roth Sabine, Goebeler Matthias, Rosenwald Andreas, Geissinger Eva
Department of Dermatology, University Hospital Würzburg, Würzburg, Germany.
Institute of Pathology and Comprehensive Cancer Center Mainfranken, University of Würzburg, Würzburg, Germany.
Oncol Ther. 2016;4(2):199-210. doi: 10.1007/s40487-016-0026-y. Epub 2016 Aug 18.
Cytotoxic CD8+ T-cell lymphomas are only rarely encountered and thus remain only poorly characterized. Our aim was to collect and correlate clinical and histological data of CD8+ skin lymphoma infiltrates to obtain a proper subtype assignment of CD8+ skin lymphoma infiltrates and to derive putative prognostic markers thereof.
Formalin-fixed and paraffin-embedded (FFPE) tissue of 35 patients with CD8+ cytotoxic cutaneous T-cell lymphoma infiltrates was retrieved from the archives of the Institute of Pathology and the Department of Dermatology, University Hospital Wuerzburg, dating back from 1998 until 2015. Cytological, histological, immunohistochemical and molecular genetic features were assessed and correlated with respective clinical data.
The identified cases of CD8+ cytotoxic atypical lymphoproliferative infiltrates of the skin ( = 35) comprised 13 cases of mycosis fungoides (MF)/Sézary syndrome (SS), 4 cases of subcutaneous panniculitis-like T-cell lymphoma (SPTCL), 5 cases of primary cutaneous acral CD8+ lymphoma [formerly indolent CD8+ lymphoid proliferation (ILP)] and 1 case of aggressive epidermotropic primary cutaneous T-cell lymphoma (AECTCL). Moreover, nine cases were classified as primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) and three cases as systemic PTCL-NOS. Multiple skin lesions, a high proliferative index and especially a final subtype attribution to AECTCL or systemic PTCL-NOS were associated with a worse survival. Coexpression of CD68 by tumor cells was exclusively observed in indolent acral CD8+ T-cell lymphoma and thus indicated an invariably benign clinical course. No further distinctive markers could be derived from our analysis.
Cutaneous infiltrates of CD8+ cytotoxic T-cell lymphoma comprise clinically and histologically heterogeneous entities of either primary cutaneous T-cell lymphomas or secondary infiltrates of otherwise systemic peripheral T-cell lymphomas. A thorough clinicopathological correlation with respective staging examinations remains the mainstay for correct subtype assignment and proper prognostication as long as no better markers have been defined.
细胞毒性CD8+ T细胞淋巴瘤极为罕见,因此其特征仍知之甚少。我们的目的是收集CD8+皮肤淋巴瘤浸润的临床和组织学数据并进行关联分析,以正确划分CD8+皮肤淋巴瘤浸润的亚型,并推导其潜在的预后标志物。
从维尔茨堡大学医院病理学研究所和皮肤科存档中检索了35例CD8+细胞毒性皮肤T细胞淋巴瘤浸润患者的福尔马林固定石蜡包埋(FFPE)组织,时间跨度为1998年至2015年。对细胞学、组织学、免疫组化和分子遗传学特征进行评估,并与各自的临床数据相关联。
所鉴定的皮肤CD8+细胞毒性非典型淋巴增殖浸润病例(n = 35)包括13例蕈样肉芽肿(MF)/赛塞里综合征(SS)、4例皮下脂膜炎样T细胞淋巴瘤(SPTCL)、5例原发性皮肤肢端CD8+淋巴瘤[原惰性CD8+淋巴细胞增殖(ILP)]和1例侵袭性亲表皮性原发性皮肤T细胞淋巴瘤(AECTCL)。此外,9例被分类为原发性皮肤外周T细胞淋巴瘤,未另行指定(PTCL-NOS),3例为系统性PTCL-NOS。多发皮肤病变、高增殖指数,尤其是最终归为AECTCL或系统性PTCL-NOS的亚型与较差的生存率相关。肿瘤细胞共表达CD68仅在惰性肢端CD8+ T细胞淋巴瘤中观察到,因此表明临床病程始终良性。我们的分析未得出其他明显的标志物。
CD8+细胞毒性T细胞淋巴瘤的皮肤浸润包括原发性皮肤T细胞淋巴瘤或其他系统性外周T细胞淋巴瘤的继发性浸润,在临床上和组织学上均为异质性实体。只要尚未确定更好的标志物,与相应分期检查进行全面的临床病理关联分析仍是正确亚型划分和准确预后评估的主要方法。