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特殊部位肾上腺外髓脂肪瘤的手术治疗及结果:单中心11例报告

Surgical management and outcome of Extra-adrenal myelolipomas at unusual locations: A report of 11 cases in a single center.

作者信息

Cao Jiashi, Huang Xing, Cui Na, Wang Xiang, You Chaoqun, Ni Xiangzhi, Gao Xin, Wang Jing, Liu Tielong

机构信息

Department of Orthopedics, No. 455 Hospital of the Chinese People's Liberation Army, The Navy Medical University, No. 338 Huaihai West Road, Shanghai 200052, China.

Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China.

出版信息

J Bone Oncol. 2022 Jun 7;35:100438. doi: 10.1016/j.jbo.2022.100438. eCollection 2022 Aug.

Abstract

PURPOSE

Extra-adrenal myelolipomas (EAMs) are rare benign tumors composed of both mature adipose and hematopoietic tissues with unclear etiology. There have been only sporadic case reports about the clinical characteristics and management of EAMs. Here we present our experience and practice in the clinical diagnosis and treatment of 11 consecutive patients with EAMs.

METHOD

We retrospectively reviewed 11 consecutive patients, who received surgeries in our department and were confirmed as having EAMs by postoperative histopathology from April 2016 to December 2021. Clinical information and follow-up data of all patients were collected and analyzed afterwards.

RESULTS

Of the 11 EAM patients (7 male and 4 female) with a mean age of 47.6 years, 3 were asymptomatic and 8 were symptomatic with a mean symptom duration of 6.07 months. EAMs were found in the thoracic spine in 4 cases, paravertebral mediastinal regions in 3 cases, ilium in 2 cases, humerus in 1 case, and rib in 1 case. All patients were initially misdiagnosed as other tumors by radiologists. All 11 patients received gross total excision or curettage with a mean intraoperative blood loss of 781.82 ± 1143.3 ml and a mean operation duration of 180.91 ± 98.41 min. Patients' Frankel scores and Karnofsky Performance Status score were improved or at least preserved postoperatively. No significant complications occurred postoperatively. All the 11 patients survived, and no local recurrence or distant metastasis occurred during the mean follow-up period of 42.0 months.

CONCLUSION

The surgical outcome and prognosis of EAMs are excellent and surgery can serve as the method of radical treatment.

摘要

目的

肾上腺外髓脂肪瘤(EAMs)是一种罕见的良性肿瘤,由成熟脂肪组织和造血组织组成,病因不明。关于EAMs的临床特征及治疗仅有零星病例报道。在此,我们介绍我们对11例连续性EAMs患者进行临床诊断和治疗的经验与做法。

方法

我们回顾性分析了2016年4月至2021年12月间在我科接受手术并经术后组织病理学确诊为EAMs的11例连续性患者。之后收集并分析了所有患者的临床信息及随访数据。

结果

11例EAMs患者(7例男性,4例女性),平均年龄47.6岁,3例无症状,8例有症状,平均症状持续时间为6.07个月。EAMs位于胸椎4例,椎旁纵隔区域3例,髂骨2例,肱骨1例,肋骨1例。所有患者最初均被放射科医生误诊为其他肿瘤。11例患者均接受了根治性切除或刮除术,术中平均失血量为781.82±1143.3ml,平均手术时间为180.91±98.41分钟。患者术后Frankel评分及卡氏功能状态评分均得到改善或至少得以维持。术后未发生严重并发症。11例患者均存活,在平均42.0个月的随访期内无局部复发或远处转移。

结论

EAMs的手术效果及预后良好,手术可作为根治性治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8aa0/9198452/8fdd62b86b3a/ga1.jpg

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