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目的总结腹膜后节细胞神经瘤(GN)的临床特点。方法回顾性收集2013年5月至2016年7月期间于徐州医科大学附属医院行手术并经术后病理学检查证实为腹膜后GN的18例患者的临床资料,结合相关文献报道,总结腹膜后GN的临床症状、影像学表现、病理学特征、治疗及预后。结果 (1)临床症状:18例患者中,8例患者无任何症状,经体检发现;6例表现为腹部疼痛不适;1例表现为下腹部坠胀不适;3例因高血压病就诊。(2)影像学表现:18例患者均行彩超检查,16例表现为边界清楚的低回声团块,2例形态不规则;12例回声均匀,6例回声欠均匀。17例患者行CT平扫,表现为均匀低密度14例,密度不均匀3例;3例可见散在点状钙化。15例患者行CT动态增强扫描,动、静脉期均无明显强化2例,动脉期瘤体无明显强化8例,静脉期见瘤体不均质强化11例。3例患者行MRI平扫,3例T1WI图像均表现为均匀低信号;1例T2WI图像呈明显高信号,2例T2WI图像呈中等强度高信号。(3)手术及预后:15例患者行腹腔镜下腹膜后肿瘤切除术,3例行开放性手术,肿瘤均完整切除,所有患者术后恢复良好且无明显并发症发生。术后所有患者均获得随访,随访时间6~32个月,中位数为20个月,随访期间暂未发现肿瘤复发或远处转移。结论 GN的临床表现无特异性,影像学检查是术前肿瘤诊断最为重要的辅助性检查,但病理学检查仍为GN诊断的金标准。GN行肿瘤根治性切除后的预后良好。
Objective To summarize the clinical features of retroperitoneal ganglioneuroma (GN). Methods The clinical data of 18 patients who underwent surgery at the Affiliated Hospital of Xuzhou Medical University between May 2013 and July 2016 were retrospectively collected and confirmed as retroperitoneal GN by postoperative pathological examination. Clinical symptoms, imaging findings, pathological features, treatment and prognosis of GN. Results (1) Clinical symptoms: Among the 18 patients, 8 patients had no symptoms and were found by physical examination; 6 patients showed abdominal pain discomfort; 1 patient showed abdominal distension discomfort; 3 patients were treated by hypertension. (2) Imaging findings: 18 patients underwent color Doppler ultrasound examination, 16 cases showed clear boundary hypoechoic mass, 2 cases of irregular shape; 12 cases of echo uniform, 6 cases of echo less uniform. Seventeen patients underwent CT scan, which showed uniform low density in 14 cases and uneven density in 3 cases. Scattered punctate calcification was seen in 3 cases. Fifteen patients underwent CT dynamic contrast-enhanced scanning. There were no significant enhancement in arterial and venous phase in 2 cases, no significant enhancement in arterial phase in 8 cases and heterogeneous enhancement in 11 cases in venous phase. Three patients underwent MRI plain scan and three cases of T1WI images showed a uniform low signal; one T2WI image was significantly high signal, two T2WI images showed moderate high intensity signal. (3) Surgery and prognosis: Laparoscopic retroperitoneal nephrectomy was performed in 15 patients and open surgery was performed in 3 patients. Neoplasms were completely resected. All patients recovered well without significant complications. All patients were followed up for 6 to 32 months with a median of 20 months. No tumor recurrence or distant metastasis was found during follow-up. Conclusion The clinical manifestations of GN are nonspecific. Imaging examination is the most important auxiliary examination for preoperative tumor diagnosis, but the pathological examination is still the gold standard for the diagnosis of GN. The prognosis of GN after radical resection is good.