摘要
目的·探讨原发于口腔的外周T细胞淋巴瘤-非特指型(peripheral T-cell lymphoma,not otherwise specified,PTCLNOS)的临床表现和免疫表型特征。方法·回顾性分析2020年8月至2024年8月就诊于上海交通大学医学院附属第九人民医院口腔黏膜病科、口腔为首发部位、确诊为PTCL-NOS的患者的病史和病理资料,并检索PubMed、Web of Science、Embase、Scopus和中国知网共5个数据库,结合2014年1月至2024年9月国际上相关病例报道,对临床特点、病理学特点进行分析归纳。结果·共20例口腔PTCL-NOS病例纳入研究。其中男性11例(55.0%),女性9例(45.0%);患者首诊年龄25~77岁,平均(52.53±12.94)岁;好发部位为舌部(25.0%)、腭部(25.0%)及颊部(20.0%);19例(95.0%)不伴B症状。纳入病例免疫表型阳性表达情况为CD3(19/19)、CD4(11/13)、CD8(7/12)、CD2(5/6)、CD7(5/5)、TIA-1(6/7)、GB(9/13)、perforin(4/6),EBER均阴性表达(8/8)。85%的病例Ki-67增殖指数≥60%。结论·口腔PTCL-NOS极为罕见,临床进展极具侵袭性,口腔表现为深而大的黏膜溃疡,基底高低不平,可扪及结节。病理特征具有异质性,免疫表型检测有助于早期诊断和分型。口腔科医师需加深对该疾病的认识,以避免错过此类恶性疾病的早期诊断与治疗。
Objective·To investigate the clinical manifestations and immunophenotypic features of peripheral T-cell lymphoma,not otherwise specified(PTCL-NOS),involving the oral cavity.Methods·The medical histories and pathology records of patients diagnosed with oral PTCL-NOS in the Department of Oral Mucosal Diseases of Shanghai Ninth People's Hospital,Shanghai Jiao Tong University School of Medicine,between August 2020 and August 2024 were retrospectively analyzed.In addition,5 databases,including PubMed,Web of Science,Embase,Scopus,and CNKI,were searched,and relevant cases reported internationally from January 2014 to September 2024 were reviewed.Results·A total of 20 oral PTCL-NOS cases were included,comprising 11 males(55.0%)and 9 females(45.0%).The patients'ages at initial diagnosis ranged from 25 to 77 years,with a mean age of(52.53±12.94)years.The most common sites were the tongue(25.0%),palate(25.0%),and buccal mucosa(20.0%).Nineteen cases(95.0%)had no B symptoms.The cases were positive for CD3(19/19),CD4(11/13),CD8(7/12),CD2(5/6),CD7(5/5),TIA-1(6/7),GB(9/13),perforin(4/6).EBER expression was negative(8/8).The Ki-67 proliferation index was≥60% in 85% of cases.Conclusions·Oral PTCL-NOS is extremely rare and has an aggressive clinical behavior.The oral manifestation presents as deep and large mucosal ulcers with uneven bases,and nodules can be palpable.The pathological features are heterogeneous.Immunophenotype detection is useful for early diagnosis and classification.It is essential for stomatologists to enhance their awareness of this malignancy to avoid delayed diagnosis and treatment.
作者
黄润语
张春叶
张颖
赵峥岩
杨扬
吴岚
HUANG Runyu;ZHANG Chunye;ZHANG Ying;ZHAO Zhengyan;YANG Yang;WU Lan(Department of Oral Mucosal Diseases,Shanghai Ninth People's Hospital,Shanghai Jiao Tong University School of Medicine/College of Stomatology,Shanghai Jiao Tong University/National Center for Stomatology/National Clinical Research Center for Oral Diseases/Shanghai Key Laboratory of Stomatology/Shanghai Research Institute of Stomatology,Shanghai 200011,China;Department of Oral Pathology,Shanghai Ninth People's Hospital,Shanghai Jiao Tong University School of Medicine/College of Stomatology,Shanghai Jiao Tong University/National Center for Stomatology/National Clinical Research Center for Oral Diseases/Shanghai Key Laboratory of Stomatology/Shanghai Research Institute of Stomatology,Shanghai 200011,China;Department of Periodontal and Mucosal Diseases,Affiliated Stomatological Hospital of Xuzhou Medical University,Xuzhou 221002,China)
出处
《上海交通大学学报(医学版)》
北大核心
2025年第5期653-660,共8页
Journal of Shanghai Jiao tong University:Medical Science
基金
上海市科学技术委员会基金(21S21902000)
上海市卫生健康委员会项目[(ZY(2021-2023)-0207-01-04,2022CX009)]
上海交通大学医学院附属第九人民医院交叉基金(JYJC202111)。
关键词
口腔特征
外周T细胞淋巴瘤
非特指型
临床病理
oral feature
peripheral T-cell lymphoma(PTCL)
not otherwise specified(NOS)
clinical pathology