赵佳 1孙海燕 1陈昱彤 1陈姚旭 1王卓 2冯友繁 3梁小芹1
作者信息
- 1. 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
- 2. 甘肃省人民医院病理科,甘肃 兰州 730000
- 3. 甘肃省人民医院血液科,甘肃 兰州 730000
- 折叠
摘要
Abstract
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.关键词
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Key words
Adrenal gland/Aggressive B-cell lymphoma/T-cell lymphoma/Therapy/Rare disease/Prognosis分类
医药卫生
