十二指肠节细胞性副神经节瘤的临床、影像及病理特征分析

Clinical,imaging and pathological features of duodenal gangliocytic paraganglioma

目的通过探讨十二指肠节细胞性副神经节瘤(DGP)的临床、影像及病理学特征,加强对DGP的认识并提高临床诊疗水平。方法回顾性分析山东第一医科大学附属省立医院2012年1月至2021年10月诊断的8例DGP患者的临床资料,总结其临床特点、影像学特征、病理学特征、治疗及预后。结果8例DGP患者中男性7例,女性1例,年龄37~57岁,中位年龄52岁。5例无临床症状的患者在健康查体或术前查体时偶然发现;3例患者因黑便就诊。CT影像学检查均表现为十二指肠局限性结节样增厚,增强扫描呈明显渐进性强化。超声内镜下表现为十二指肠黏膜下低回声病变。组织学上肿瘤由梭形细胞、上皮样细胞和神经节样细胞三种成分,梭形细胞表达神经丝蛋白(NF)、神经元特异性烯醇化酶(NSE)、S-100,上皮样细胞表达细胞角蛋白、NSE、突触素(Syn)、嗜铬素(CgA),神经节样细胞表达NSE、Syn、CgA及NF。2例行内镜黏膜剥离术,6例行外科手术切除。结论DGP是一种罕见的好发于十二指肠降段的良性神经内分泌肿瘤,影像学及内镜检查提示黏膜下病变,治疗以内镜下切除和外科手术局部切除为主,预后良好。

Objective To study the clinical,imaging and pathological features of duodenal gangliocytic paraganglioma(DGP).Methods The clinical,imaging and pathological data of patients with DGP treated at the Shandong Provincial Hospital Affiliated to Shandong First Medical University from January 2012 to October 2021 were retrospectively analyzed.Results Of 8 patients with DGP,there were 7 males and 1 female,with a median age of 52 years(range 37 to 57 years).Five patients were asymptomatic and they were diagnosed on physical examination followed by investigations.Three patients presented with black stools.CT examination showed localized nodular thickening of the duodenum,with enhanced scanning showing obvious progressive contrast enhancement.Endoscopic ultrasonography showed a hypoechoic submucosal lesion in duodenal wall.Histologically,the neoplasm composed of three different cell types which included Schwann cells,epithelioid cells,and ganglioid cells.The Schwann cells expressed NF,NSE and S-100 proteins;the epithelioid cells expressed CK,NSE,Syn and CgA proteins;while the ganglioid cells expressed NSE,Syn,CgA and NF proteins.Endoscopic submucosal dissection was performed in 2 patients and surgical resection was performed in 6 patients.Conclusion DGP is a rare benign neurogenic tumor which is most commonly found in the duodenum.It has a good prognosis.Imaging and endoscopic examinations demonstrated a submucosal mass.The main treatment are endoscopic resection and local surgical resection.