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胰腺实性假乳头状肿瘤的临床特征OA

Clinical characteristics of solid pseudopapillary tumor of the pancreas

中文摘要英文摘要

目的:胰腺实性假乳头状肿瘤是一种罕见的低度恶性肿瘤,无典型的症状和体征,术前诊断较困难.本研究总结胰腺实性假乳头状肿瘤的临床特点,探讨其诊断方式及治疗原则.方法:选取2010年1月至2023年5月在山西医科大学第一医院行手术治疗并经病理学证实为胰腺实性假乳头状肿瘤的42例患者.回顾性分析其人口学、影像学、病理学特征及治疗方式.结果:42例患者中男12例,女30例,主要临床表现为消化道症状.术前诊断为胰腺实性假乳头状肿瘤22例,胰腺癌1例,黏液性囊腺瘤3例,肉瘤1例,无功能胰岛细胞瘤1例,神经内分泌瘤2例,余诊断不明确.术前的影像学检查中CT及磁共振成像(magnetic resonance imaging,MRI)的诊断正确率显著高于二维超声检查.肿瘤标志物中以神经元特异性烯醇化酶(neuron specific enolase,NSE)升高最常见(8例),可能对胰腺实性假乳头状肿瘤有诊断价值.免疫组织化学检测示:β连环素、波形蛋白(Vimentin)、α1-抗胰蛋白酶(α1-antitrypsin,AAT)、分化簇10(cluster of differentiation 10,CD10)、突触素(synaptophysin,SYN)、NSE的阳性率分别为100%、93%、86%、82%、78%、66%.42例患者均随访,其中6例失访,余36例随访49.83(25.00~104.53)个月(包括1例术后4年肿瘤复发转移,2个月后死亡;1例术后2年复发,目前正接受化学治疗;余34例未见肿瘤复发及转移,均存活).结论:胰腺实性假乳头状肿瘤好发于女性,主要以消化系统症状为主,CT及MRI诊断正确率高,明确诊断需结合镜下观察和免疫组织化学检测,手术是有效的治疗方法.

Objective:Solid pseudopapillary tumor of the pancreas is a rare low-grade malignant tumor with no typical symptoms and signs,making preoperative diagnosis difficult.This study aims to summarize the clinical characteristics of solid pseudopapillary tumors of the pancreas and discuss its diagnostic methods and treatment principles. Methods:A total of 42 patients diagnosed with solid pseudopapillary tumors of the pancreas by pathological examination following surgical treatment at the First Hospital of Shanxi Medical University from January 2010 to May 2023 were selected.Their demographic,radiological,pathological characteristics,and clinical treatment modalities were retrospectively analyzed. Results:Among the 42 patients,there were 12 males and 30 females,mainly presenting with gastrointestinal symptoms.The preoperative diagnosis included 22 patients with solid pseudopapillary tumor of the pancreas,1 pancreatic cancer,3 mucinous cystadenoma,1 sarcoma,1 non-functional pancreatic islet cell tumor,2 neuroendocrine tumor,and the rest were unclear.The diagnostic accuracy of CT and magnetic resonance imaging(MRI)in preoperative imaging examinations was significantly higher than that of two-dimensional ultrasonography.Among tumor markers,elevated levels of neuron-specific enolase(NSE)were most common(8 patients),which may have diagnostic value for solid pseudopapillary tumors of the pancreas.Immunohistochemical detection showed positive rates of β-catenin,vimentin,α1-antitrypsin(AAT),cluster of differentiation 10(CD10),synaptophysin(SYN)and NSE were 100%,93%,86%,82%,78%,and 66%,respectively.Among the 42 follow-up patients,6 patients were lost to follow-up,and the remaining 36 were followed up for a median of 49.83(25.00 to 104.53)months,including 1 patient experienced tumor recurrence and metastasis 4 years after surgery and died 2 months later,1 patient experienced recurred 2 years after surgery,and the remaining 34 patients survived without tumor recurrence or metastasis. Conclusion:Solid pseudopapillary tumors of the pancreas predominantly affects females,and presents mainly with gastrointestinal symptoms.CT and MRI exhibit high diagnostic accuracy,while a definitive diagnosis requires microscopic observation and immunohistochemistry.Surgical treatment is an effective treatment.

闫凯峰;刘宏

山西医科大学第一临床医学院,太原 030001山西医科大学第一医院肝胆胰外科及肝脏移植中心,太原 030001

胰腺肿瘤胰腺实性肿瘤实性假乳头状肿瘤

pancreatic neoplasmsolid tumor of pancreassolid pseudopapillary tumor

《临床与病理杂志》 2024 (001)

23-30 / 8

山西省自然科学基金(201901D111350).This work was supported by the Natural Science Foundation of Shanxi Province,China(201901D111350).

10.11817/j.issn.2095-6959.2024.230283

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