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目的:探讨胰腺浆液性微囊腺瘤的不典型临床影像表现及诊治策略。方法:回顾2008年7月至2019年10月北京协和医院收治的11例术前临床影像表现不典型的胰腺浆液性微囊腺瘤的患者资料，总结其临床表现、CT/MRI特点、术前临床诊断、手术方法、术后情况等。结果:共纳入11例不典型微囊腺瘤患者，中位年龄50(46～66)岁，女性7例，男性4例，伴有腰腹痛者8例，体重下降者6例。术前影像学检查发现病变位于头颈部者8例，伴有胰胆管扩张7例，可疑血管侵犯3例，存在胰腺实质萎缩2例，表现为富血供实性占位3例，与胰管相通2例。术前拟诊为胰腺恶性肿瘤者4例，神经内分泌肿瘤3例，实性假乳头状瘤3例，导管内乳头状黏液肿瘤1例。所有患者均接受手术治疗，远端胰腺切除3例(其中2例应用Kimura法保脾)，胰十二指肠切除术3例，全胰切除1例，保留十二指肠的胰头切除2例，局部切除2例。所有患者无术后早期死亡。术后并发B级胰瘘2例，生化漏2例，术后腹腔出血1例，胃排空延迟2例，均经积极治疗后治愈。患者术后中位住院时间18(7～63)d。结论:胰腺浆液性微囊腺瘤可伴有胆胰管扩张、实质萎缩，甚至血管侵犯等不典型侵袭性特征，掌握其不典型的临床影像表现有助于鉴别诊治。对于术前具有侵袭性影像学表现者，手术仍是主要的治疗方法之一。“,”Objective:To summarize the atypical manifestations and treatment strategies of serous microcystic neoplasm of the pancreas.Methods:Review the case data of 11 cases of pancreatic serous microcystic adenoma with atypical preoperative clinical imaging findings admitted to Peking Union Medical College Hospital from July 2008 to October 2019, and summarize their clinical manifestations, CT/MRI features, and preoperative clinical diagnosis, surgical methods, postoperative conditions.Results:The median age of the 11 patients was 50 (46-66) years old, 7 females, and 4 males. There were 8 cases with back pain and 6 cases with weight loss. The preoperative imaging examination found that the lesion was located in the head and neck in 8 cases, with pancreaticobiliary duct dilatation in 7 cases, suspected vascular invasion in 3 cases, and pancreatic parenchymal atrophy in 2 cases, 3 cases showed rich blood supply and solid space, and 2 cases were connected to the pancreatic duct. Preoperative diagnosis of pancreatic malignant tumors accounted for 4 cases, neuroendocrine tumors in 3 cases, solid pseudopapillary tumors in 3 cases, and intraductal papillary mucinous tumors in 1 case. All patients underwent surgical treatment, 3 cases of distal pancreatectomy (2 cases of using Kimura method to protect the spleen), 3 cases of pancreaticoduodenectomy, 1 case of total pancreatectomy, 2 cases of pancreatic head resection with duodenum preservation, 2 cases of local resection. All patients had no early postoperative deaths. There were 2 cases of grade B pancreatic fistula, 2 cases of biochemical leakage, 1 case of postoperative abdominal hemorrhage, and 2 cases of delayed gastric emptying, all of which were cured after active treatment. The median postoperative hospital stay was 18 (7-63) days.Conclusions:Pancreatic serous microcystic neoplasm could be accompanied by atypical features such as pancreatic/bile duct dilation, parenchyma atrophy, or even present vessel invasion. Understanding the atypical clinic and image features would help improving differential diagnosis and treatment. For those with invasive features, surgical exploration should be recommended.