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一名患有脊髓灰质炎后遗肢体麻痹的患者出现极其巨大的骶骨脊索瘤——罕见病例情况。

Exceptionally giant neglected sacral chordoma in a post-poliotic residual paralysis patient - a rare case scenario.

作者信息

Kantiwal Prabodh, Aggarwal Aakarsh, Yadav Sandeep K, Gahlot Nitesh, Elhence Abhay

机构信息

Department of Orthopaedics Surgery, All India Institute of Medical Sciences Jodhpur, Rajasthan, India.

出版信息

Am J Neurodegener Dis. 2024 Aug 25;13(3):13-22. doi: 10.62347/EKNJ6411. eCollection 2024.

DOI:10.62347/EKNJ6411
PMID:39308697
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11411203/
Abstract

Chordoma is a rare malignant tumour with an incidence of 0.1 case per 1 lakh population per year. The sacrococcygeal region is the most common site to be involved. Herein, we are reporting a case of sacral chordoma, who is a 32-year-old male patient, a known case of post-polio residual paralysis on the left lower limb, who presented with complaint of pain in the lower back and gluteal region for 2 years with swelling in the gluteal region for 1 year, which was gradually increasing in size for 1 year with associated weight loss. MRI revealed an ill-defined lytic expansile altered signal intensity lesion involving S3 to S5 and coccygeal vertebral bodies measuring 13.2 × 16.2 × 14 cm (ap × tr × cc) with adjacent large lobulated heterogeneous soft tissue component and showed multiple coarse calcifications. The lesion anteriorly displaced and abutted the rectum and was deriving its blood supply from branches of bilateral internal iliac arteries. The patient was planned and underwent wide-margin resection (middle sacrectomy with R0 margins with preservation of both S2 and right S3 nerve roots). Histologic Grade was reported to be G2, moderately differentiated, high grade. Pathologic stage classification was reported as pT3a. Postoperatively patient had the same neurological status and was discharged on advice to do full weight bearing walking and self-intermittent catheterisation and laxatives. He was on routine follow up and improved well symptomatically.

摘要

脊索瘤是一种罕见的恶性肿瘤,每年发病率为每10万人中有0.1例。骶尾区域是最常受累的部位。在此,我们报告一例骶骨脊索瘤病例,患者为一名32岁男性,已知患有左下肢小儿麻痹后遗症,因下背部和臀区疼痛2年、臀区肿胀1年就诊,肿胀在1年中逐渐增大,并伴有体重减轻。磁共振成像(MRI)显示一个边界不清的溶骨性膨胀性信号强度改变病灶,累及S3至S5及尾椎椎体,大小为13.2×16.2×14厘米(前后径×左右径×上下径),伴有相邻的大分叶状不均匀软组织成分,并显示多处粗大钙化。病灶向前移位并紧邻直肠,其血供来自双侧髂内动脉分支。该患者计划并接受了广泛边缘切除术(保留S2和右侧S3神经根的R0边缘骶骨中段切除术)。组织学分级报告为G2,中度分化,高级别。病理分期分类报告为pT3a。术后患者神经状态相同,经建议进行全负重行走、自我间歇性导尿和服用泻药后出院。他接受常规随访,症状改善良好。

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Exceptionally giant neglected sacral chordoma in a post-poliotic residual paralysis patient - a rare case scenario.一名患有脊髓灰质炎后遗肢体麻痹的患者出现极其巨大的骶骨脊索瘤——罕见病例情况。
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本文引用的文献

1
Chordoma: Genetics and Contemporary Management.脊索瘤:遗传学与当代管理。
Int J Mol Sci. 2024 May 28;25(11):5877. doi: 10.3390/ijms25115877.
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Radiotherapy after gross-total resection and subtotal resection of spinal chordoma: a SEER database analysis of overall survival outcomes.脊髓脊索瘤行大体全切和次全切除术后的放疗:基于 SEER 数据库的总生存结果分析。
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Chordoma-Current Understanding and Modern Treatment Paradigms.脊索瘤——当前的认识与现代治疗模式
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The Role of Radiotherapy for Chordoma Patients Managed With Surgery: Analysis of the National Cancer Database.手术治疗脊索瘤患者中放射治疗的作用:国家癌症数据库分析。
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Evaluating the Role of Adjuvant Radiotherapy in the Management of Sacral and Vertebral Chordoma: Results from a National Database.评估辅助放疗在骶骨和脊柱脊索瘤治疗中的作用:来自国家数据库的结果
World Neurosurg. 2019 Jul;127:e1137-e1144. doi: 10.1016/j.wneu.2019.04.070. Epub 2019 Apr 14.
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Giant sacrococcygeal chordoma: A case report.巨大骶尾部脊索瘤:一例报告。
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Sacral chordoma : A review of literature.骶骨脊索瘤:文献综述
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Cryosurgery in the excision of a giant local recurrent sacral chordoma: a case report and literature review.冷冻手术切除巨大局部复发性骶骨脊索瘤:病例报告及文献复习。
Eur Spine J. 2018 Dec;27(12):3059-3063. doi: 10.1007/s00586-017-5438-3. Epub 2018 Jan 25.
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First report of clinical responses to immunotherapy in 3 relapsing cases of chordoma after failure of standard therapies.标准治疗失败后3例复发性脊索瘤免疫治疗临床反应的首次报告。
Oncoimmunology. 2017 Jun 21;6(8):e1338235. doi: 10.1080/2162402X.2017.1338235. eCollection 2017.
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Safety and Local Control of Radiation Therapy for Chordoma of the Spine and Sacrum: A Systematic Review.脊柱和骶骨脊索瘤放射治疗的安全性与局部控制:一项系统评价
Spine (Phila Pa 1976). 2016 Oct 15;41 Suppl 20(Suppl 20):S186-S192. doi: 10.1097/BRS.0000000000001831.