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To investigate the clinicopathological characteristics, diagnosis, and treatment of primary seminal vesicle adenocarcinoma (SVAC).We analyzed the clinical data and clinicopathological characteristics of 4 cases of primary SVAC treated in the Department of Urology of the Second Hospital of Tianjin Medical University and reviewed relevant literature.All the 4 patients were treated by open radical resection of the seminal vesicle and prostate and pathologically diagnosed with SVAC. Preoperative prostatic biopsy had shown 1 of the cases to be negative, while preoperative CT and transrectal ultrasound had revealed a huge pelvic cystic neoplasm in another patient. Immunohistochemistry manifested that the 4 cases were all negative for prostate-specific antigen (PSA), prostatic acid phosphatase (PAP), and cytokeratin 20 (CK20), but positive for cancer antigen 125 (CA125) and CK7. All the patients recovered smoothly after surgery and experienced no recurrence or metastasis during 154, 41, 20, and 12 months of follow-up.Primary seminal vesicle carcinoma is extremely rare and presents in an advanced stage. Immunohistochemistry plays a valuable role in its differential diagnosis. Various combinations of radical surgery, radiotherapy, androgen-deprivation therapy, and chemotherapy are recommended for the treatment of the disease.目的: 探讨原发性精囊腺癌的病理学特点和诊疗方法。方法: 分析4例原发性精囊腺癌的临床资料和病理学特征，并行全面文献复习。结果: 4例患者均行开放性前列腺根治性切除术，病理确诊为原发性精囊腺癌：１例术前行前列腺穿刺活检阴性，行开放前列腺癌根治术后确诊为精囊腺癌：1例术前经CT及经直肠超声可见盆腔巨大囊性多分隔肿物，术中冰冻病理诊断为精囊腺癌，转行开放性前列腺根治术。2例术前血清PSA水平数值分别为20.15、40.75 μg/ml，余2例水平正常，4例术后血清PSA水平均正常。4例患者免疫组化结果提示PSA、PAP和CK20均阴性，而CA125和CK7阳性。4例术后恢复良好，分别随访154、41、20、12个月，未见复发转移。结论: 原发性精囊腺癌极其罕见，临床发现较晚。免疫组化对鉴别诊断价值很大。目前提倡多种方法诸如根治手术、放疗、雄激素剥夺疗法和化疗的综合治疗。.