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脊柱侧凸:椎管内节细胞神经瘤的一种不常见临床表现。

Scoliosis: an unusual clinical presentation of paraspinal ganglioneuroma.

机构信息

Division of Spine Surgery, Department of Orthopedic Surgery, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Zhongshan Road 321, Nanjing, 210008, Jiangsu Province, China.

出版信息

Spine Deform. 2022 Sep;10(5):1185-1195. doi: 10.1007/s43390-022-00511-x. Epub 2022 Apr 29.

Abstract

PURPOSE

To comprehensively present the clinical characteristics and treatment strategies in patients with scoliosis secondary to ganglioneuroma (S-GN).

METHODS

Six patients with S-GN treated surgically at a median age of 12 years were retrospectively reviewed and the median follow-up period was 6 years (4-14 years). The radiological features of GN and the associated scoliosis were evaluated. The surgical strategies and the corresponding outcomes were investigated.

RESULTS

All patients had a delayed diagnosis age of GN than scoliosis (12 vs. 9 years). GN was located at the posterior mediastinum in four patients (66.7%) and at retroperitoneum in two, respectively. Tumor occupancies were frequently detected on the X-ray films for four patients (66.7%), being uniformly on the convexity of the main curve. All patients complained of rapid progressive deformities during the growth period. Five patients (83.3%) received total tumor resections, one accepted partial resection. Deformity correction was implemented for all patients with an average rate of 66.4% on the main curve. No recurrence of the GN was detected for all totally tumor-resected patients at the latest follow-up.

CONCLUSION

S-GN is often misdiagnosed clinically. Paravertebral mass neighboring the apex of scoliosis can be meticulously detected from the X-ray films. Total tumor resection should be aggressively performed if possible. The deformity correction could be satisfactorily obtained and the risk of recurrence of the GN was relatively low.

摘要

目的

全面呈现神经节细胞瘤继发脊柱侧凸(S-GN)患者的临床特征和治疗策略。

方法

回顾性分析了 6 例在 12 岁时接受手术治疗的 S-GN 患者,中位随访时间为 6 年(4-14 年)。评估了 GN 和相关脊柱侧凸的影像学特征。研究了手术策略和相应的结果。

结果

所有患者的 GN 诊断年龄均晚于脊柱侧凸(12 岁 vs. 9 岁)。4 例患者(66.7%)GN 位于后纵隔,2 例位于腹膜后。4 例患者(66.7%)的 X 线片上经常发现肿瘤占位,均位于主曲线凸侧。所有患者在生长期间均出现快速进展性畸形。5 例患者(83.3%)接受了肿瘤全切除术,1 例接受了部分切除术。所有患者均进行了畸形矫正,主曲线平均矫正率为 66.4%。所有完全肿瘤切除的患者在末次随访时均未发现 GN 复发。

结论

S-GN 临床上常被误诊。从 X 线片上可以仔细检测到邻近脊柱侧凸顶点的椎旁肿块。如果可能,应积极进行肿瘤全切除术。可以获得满意的畸形矫正,GN 复发的风险相对较低。

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