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原发性肾上腺淋巴瘤的临床病理学特征、治疗及患者预后分析OA

Analysis of clinicopathological characteristics,treatment and patient prognosis of primary adrenal lymphoma

中文摘要英文摘要

背景与目的:原发性肾上腺淋巴瘤(primary adrenal lymphoma,PAL)是一种罕见的恶性肿瘤,关于其临床病理学特征、治疗和患者预后的分析较为少见.将PAL与其他肾上腺肿块进行鉴别诊断时较为困难.鉴于PAL的罕见性及诊断复杂性,本研究回顾性分析PAL的临床病理学特征及治疗结局,旨在为鉴别诊断及个体化治疗提供参考.方法:本研究为一项单中心、回顾性观察性研究.收集2013年1月—2024年10月在甘肃省人民医院诊断为PAL的患者的临床资料.所有病例诊断均符合第5版世界卫生组织(World Health Organization,WHO)淋巴造血组织肿瘤分类标准及Ann Arbor分期系统,本研究已通过甘肃省人民医院伦理委员会审批(伦理批号:2025-590),并豁免知情同意.纳入标准:① 初诊患者,入院前未进行过任何针对肿瘤的放疗、化疗或免疫治疗;②经病理学检查确诊为PAL;③ 结内或其他部位无淋巴瘤,原发病灶位于肾上腺;④ 临床及病理学资料完整.排除标准:① 存在结内或其他部位的淋巴瘤且伴有肾上腺转移者;② 临床及病理学资料不全.收集的数据包括人口学特征、临床表现、实验室指标、影像学资料及治疗方案.病理学标本经10%中性福尔马林固定,采用EnVision两步法进行免疫组织化学染色.疗效评估参照《淋巴瘤诊疗指南(2022年版)》,分为完全缓解(complete response,CR)、部分缓解(partial response,PR)、疾 病 稳 定(stable disease,SD)及 疾 病 进 展(progressive disease,PD).通过电话随访进行预后追踪,截至时间为2024年12月31日.生存分析指标包括无进展生存期(progression-free survival,PFS)和总生存期(overall survival,OS).采用SPSS 27.0软件进行统计学分析,应用Kaplan-Meier法绘制生存曲线,P<0.05为差异有统计学意义.结果:10例患者中,男性6例,女性4例,中位年龄66岁.10例患者的临床首发症状均不典型,多表现为乏力、腰腹疼痛等症状.影像学检查显示,单侧或双侧肾上腺占位性病变.10例患者中3例诊断为T细胞淋巴瘤,其中2例为外周T细胞淋巴瘤(非特指型),1例为间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)-间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL),另外7例诊断为B细胞淋巴瘤,其中6例为弥漫大B细胞淋巴瘤,1例为EB病毒(Epstein-Barr virus,EBV)+大B细胞淋巴瘤.1例外周T细胞淋巴瘤患者和1例ALK-ALCL患者经过CHOP-E方案初始治疗后,ALK-ALCL患者PR,外周T细胞淋巴瘤患者因病情进展死亡;另1例外周T细胞淋巴瘤患者确诊后14天因噬血综合征死亡.7例B细胞淋巴瘤患者经过R-CHOP方案初始治疗后,6例患者PR,1例患者在R-CHOP方案初始治疗后接受自体骨髓造血干细胞移植术,达到CR.所有患者随访1~63个月,中位随访时间为32个月,患者预后相对较好.结论:PAL是一种罕见的恶性肿瘤,好发于中老年男性,诊断时需结合多方面因素,包括临床表现、乳酸脱氢酶升高及病理学特征,联合化疗为其主要治疗方式,患者预后与病理组织学类型相关.

Background and purpose:Primary adrenal lymphoma(PAL)is a rare and malignant tumor.Analysis of its clinicopathological characteristics,treatment,and prognosis is relatively rare.PAL is difficult to diagnose accurately compared with other adrenal masses.Given the rarity and diagnostic complexity of PAL,this study retrospectively analyzed its clinicopathological characteristics and therapeutic outcomes,aiming to provide a comprehensive reference for differential diagnosis and personalized treatment strategies.Methods:This study was designed as a single-center,retrospective observational study.We collected clinical data from patients diagnosed with PAL at Gansu Provincial People's Hospital between January 2013 and October 2024.The diagnosis of all cases was confirmed according to the 5th edition of the World Health Organization(WHO)Classification of Haematolymphoid Tumours and the Ann Arbor staging system.The study protocol was approved by the ethics committee of Gansu Provincial People's Hospital(approval number:2025-590),and informed consent was waived.Inclusion criteria:① Patients were newly diagnosed and had not received any prior antitumor therapy(including radiotherapy,chemotherapy or immunotherapy)before admission;② Diagnosis of PAL was confirmed by histopathological examination;③ No evidence of lymph node or extranodal lymphoma,with the primary tumor lesion located in the adrenal gland;④ Complete clinical and pathological data were available.Exclusion criteria:① Patients with lymph node or extranodal lymphoma accompanied by adrenal metastasis;② Patients with incomplete clinical or pathological data.Data extracted included demographic characteristics,clinical manifestations,laboratory parameters,imaging findings and therapeutic regimens.Pathological specimens were fixed in 10%neutral buffered formalin.Immunohistochemical staining was performed using the EnVision two-step method.Treatment response was evaluated according to the"Diagnosis and treatment guideline for lymphoma(2022 version)",categorized as complete response(CR),partial response(PR),stable disease(SD)and progressive disease(PD).Prognostic follow-up was conducted via telephone interviews until December 31,2024.Survival endpoints included progression-free survival(PFS)and overall survival(OS).Statistical analysis was performed using SPSS 27.0 software.Survival curves were plotted using the Kaplan-Meier method,with statistical significance set at P<0.05.Results:Among the 10 patients,6 were male and 4 were female,with a median age of 66 years.All 10 patients exhibited atypical initial clinical symptoms,often manifesting as fatigue,abdominal and back pain,etc.Imaging studies revealed solitary or bilateral adrenal nodular lesions.Of the 10 patients,3 were diagnosed with T-cell lymphoma,including 2 peripheral T-cell lymphomas of unspecified type and 1 ALK-anaplastic large cell lymphoma(ALCL).The remaining 7 were diagnosed with B-cell lymphoma,including 6 diffuse large B-cell lymphomas and 1 Epstein-Barr virus(EBV)+large B-cell lymphoma.One patient with peripheral T-cell lymphomaand one patient with ALK-ALCL received initial treatment with the CHOP-E regimen.The ALK-ALCL patient achieved PR,whereas the PTCL patient died of disease progression.Another peripheral T-cell lymphoma patient died of hemophagocytic lymphohistiocytosis 14 d after diagnosis.Of the 7 patients with B-cell lymphoma,6 achieved PR following initial treatment with the R-CHOP regimen,and one underwent autologous bone marrow hematopoietic stem cell transplantation after initial treatment with R-CHOP,achieving CR.All patients were followed for 1 to 63 months,with a median follow-up period of 32 months.The prognosis for these patients was relatively favorable.Conclusion:PAL is a rare type of malignant tumor that predominantly affects middle-aged and elderly men.Diagnosis requires consideration of multiple factors,including clinical manifestations,elevated lactate dehydrogenase levels,and pathological characteristics.Combined chemotherapy is the primary treatment approach.The prognosis of patients is related to the type of pathological tissue.

赵佳;孙海燕;陈昱彤;陈姚旭;王卓;冯友繁;梁小芹

甘肃中医药大学第一临床医学院,甘肃 兰州 730000||甘肃省人民医院病理科,甘肃 兰州 730000甘肃中医药大学第一临床医学院,甘肃 兰州 730000||甘肃省人民医院病理科,甘肃 兰州 730000甘肃中医药大学第一临床医学院,甘肃 兰州 730000||甘肃省人民医院病理科,甘肃 兰州 730000甘肃中医药大学第一临床医学院,甘肃 兰州 730000||甘肃省人民医院病理科,甘肃 兰州 730000甘肃省人民医院病理科,甘肃 兰州 730000甘肃省人民医院血液科,甘肃 兰州 730000甘肃中医药大学第一临床医学院,甘肃 兰州 730000||甘肃省人民医院病理科,甘肃 兰州 730000

医药卫生

肾上腺侵袭性B细胞淋巴瘤T细胞淋巴瘤治疗罕见病预后

Adrenal glandAggressive B-cell lymphomaT-cell lymphomaTherapyRare diseasePrognosis

《中国癌症杂志》 2026 (5)

488-495,8

10.19401/j.cnki.1007-3639.2026.05.007

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