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19例肾上腺神经鞘瘤的临床特征及腹膜后腹腔镜切除术

Clinical features and retroperitoneal laparoscopic resection of adrenal schwannoma in 19 patients.

作者信息

Li Shu-Qiang, Zhang Yu-Shi, Shi Jie, Li Han-Zhong

出版信息

Endocr Pract. 2015 Apr;21(4):323-9. doi: 10.4158/EP14453.OR.

Abstract

OBJECTIVE

To explore the diagnostic and therapeutic principles of the rare adrenal schwannoma.

METHODS

We objectively analyzed the detailed clinical data of 19 patients with adrenal schwannoma treated in a large comprehensive medical center in China between January 2009 and March 2014, including general information, clinical manifestations, imaging, adrenal-related endocrine tests, treatment, pathology, and follow-up records.

RESULTS

Patients included 16 females and 3 males ranging from 23 to 66 years of age (mean, 48.3 ± 12.6 years), of whom 15 were discovered incidentally. Four patients reported mild abdominal discomfort. Each patient had one unilateral lesion, ranging in diameter from 4.0 to 8.8 cm (mean, 6.2 ± 1.2 cm). Lesions were hypoechoic in 16 patients and cystic-solid in 3 patients. Computed tomography (CT) scanning revealed soft tissue density, mostly with calcifications and clear boundaries. Average values in normal and enhanced CT were 28.7 ± 7.2 Hounsfield units (HU) and 59.7 ± 5.4 HU, respectively. Indicators of adrenal-related endocrine tests were normal, except that catecholamines levels were slightly low in 2 patients with excessively large lesions. All patients underwent uncomplicated retroperitoneal laparoscopic surgery, with subsequent pathology confirmation of adrenal schwannoma. The patients have been followed for 6 months to 4 years, with no recurrence.

CONCLUSION

Our results demonstrate that the onset of adrenal schwannoma is always occult, without typical clinical manifestations and adrenal-related endocrine abnormalities, and the pre-operative diagnosis depends primarily on imaging features of hypoechoic calcifications and mild enhancement. Diagnosis can be confirmed by postoperative pathology. Even with large diameter lesions, retroperitoneal laparoscopic surgery is still the preferred approach, with good prognosis.

摘要

目的

探讨罕见肾上腺神经鞘瘤的诊断与治疗原则。

方法

我们客观分析了2009年1月至2014年3月在中国一家大型综合医疗中心接受治疗的19例肾上腺神经鞘瘤患者的详细临床资料,包括一般信息、临床表现、影像学检查、肾上腺相关内分泌检查、治疗、病理及随访记录。

结果

患者包括16例女性和3例男性,年龄在23至66岁之间(平均48.3±12.6岁),其中15例为偶然发现。4例患者报告有轻度腹部不适。每位患者均有一个单侧病变,直径范围为4.0至8.8厘米(平均6.2±1.2厘米)。16例患者病变为低回声,3例为囊实性。计算机断层扫描(CT)显示软组织密度,大多伴有钙化且边界清晰。平扫CT和增强CT的平均值分别为28.7±7.2亨氏单位(HU)和59.7±5.4 HU。肾上腺相关内分泌检查指标均正常,除2例病变过大患者的儿茶酚胺水平略低。所有患者均顺利接受了腹膜后腹腔镜手术,术后病理证实为肾上腺神经鞘瘤。患者已随访6个月至4年,无复发。

结论

我们的结果表明,肾上腺神经鞘瘤发病隐匿,无典型临床表现和肾上腺相关内分泌异常,术前诊断主要依赖于低回声钙化及轻度强化的影像学特征。术后病理可确诊。即使病变直径较大,腹膜后腹腔镜手术仍是首选方法,预后良好。

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