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肾上腺神经节细胞瘤:42例中国患者的特征与预后

Adrenal Ganglioneuroma: Features and Outcomes of 42 Cases in a Chinese Population.

作者信息

Xie Jing, Dai Jun, Zhou Wen-Long, Sun Fu-Kang

机构信息

Department of Urology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Ruijin Second Road No. 197, Shanghai, 200025, China.

出版信息

World J Surg. 2018 Aug;42(8):2469-2475. doi: 10.1007/s00268-018-4499-8.

Abstract

BACKGROUND

Adrenal ganglioneuromas (AGNs) are extremely rare benign neoplasms. This study is to share our experience regarding diagnostic and operative management of these tumors.

METHODS

Clinical details as well as follow-up data were retrospectively analyzed in 42 primary AGN patients who received operative resection at a tertiary referral hospital in China between May 2005 and July 2016.

RESULTS

The mean age of the patients at diagnosis was 35.3 years (range 13-59 years). Among the patients, 28 were asymptomatic. The mean preoperative size was 5.3 ± 2.2 cm (range 2.1-14.0 cm), and 17 cases were larger than 5 cm. All patients underwent operative resection as open adrenalectomy in 20 patients and laparoscopy in 22 patients. The mean tumor size on pathologic examination was 6.0 ± 2.6 cm (range 2.2-17.0 cm). Laparoscopic operation showed its advantages over open surgery on the postoperative duration (5.0 vs. 7.3 days, p < 0.001) and estimated blood loss (69.5 vs. 157.5 ml, p = 0.047). No patient had recurrence or metastasis during a median follow-up of 70.5 months (range 3.7-164.3 months).

CONCLUSIONS

We describe the largest AGN series reported to date. The preoperative diagnosis of AGN remains difficult despite of the progression of imaging examination. After complete resection, the prognosis of AGN patients is excellent. Laparoscopic approach has its advantages in the resection of AGNs.

摘要

背景

肾上腺神经节细胞瘤(AGNs)是极其罕见的良性肿瘤。本研究旨在分享我们对这些肿瘤的诊断和手术治疗经验。

方法

回顾性分析了2005年5月至2016年7月在中国一家三级转诊医院接受手术切除的42例原发性AGN患者的临床细节及随访数据。

结果

患者诊断时的平均年龄为35.3岁(范围13 - 59岁)。其中28例无症状。术前平均肿瘤大小为5.3±2.2 cm(范围2.1 - 14.0 cm),17例肿瘤直径大于5 cm。所有患者均接受了手术切除,20例行开放性肾上腺切除术,22例行腹腔镜手术。病理检查时肿瘤平均大小为6.0±2.6 cm(范围2.2 - 17.0 cm)。腹腔镜手术在术后住院时间(5.0天对7.3天,p < 0.001)和估计失血量(69.5 ml对157.5 ml,p = 0.047)方面显示出优于开放手术的优势。在中位随访70.5个月(范围3.7 - 164.3个月)期间,无患者出现复发或转移。

结论

我们描述了迄今为止报道的最大系列的AGN病例。尽管影像学检查有所进展,但AGN的术前诊断仍然困难。完全切除后,AGN患者的预后良好。腹腔镜手术在AGN切除术中具有优势。

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