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

American journal of neurodegenerative disease Pub Date : 2024-08-25 eCollection Date: 2024-01-01 DOI:10.62347/EKNJ6411
Prabodh Kantiwal, Aakarsh Aggarwal, Sandeep K Yadav, Nitesh Gahlot, Abhay Elhence
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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.

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一名脊柱侧弯后残余瘫痪病人体内被忽视的异常巨大骶脊索瘤--罕见病例。
脊索瘤是一种罕见的恶性肿瘤,发病率为每年每 10 万人口中 0.1 例。骶尾部是最常见的受累部位。在此,我们报告一例骶尾部脊索瘤病例,患者男性,32 岁,已知为小儿麻痹症后左下肢残余瘫痪,主诉下背部和臀部疼痛 2 年,臀部肿胀 1 年,肿胀逐渐增大 1 年,伴有体重减轻。核磁共振成像显示,S3至S5椎体和尾椎椎体上有一个界限不清的溶解性膨胀性信号强度改变病灶,大小为13.2 × 16.2 × 14厘米(ap × tr × cc),邻近有大分叶异质软组织成分,并显示多处粗大钙化。病灶向前方移位并与直肠相邻,血液供应来自双侧髂内动脉分支。根据计划,患者接受了广缘切除术(骶骨中段切除术,R0边缘,保留S2和右侧S3神经根)。组织学分级报告为 G2,中度分化,高级别。病理分期为 pT3a。术后患者的神经功能状况与术后相同,出院时医生建议他完全负重行走,并自行间断导尿和服用泻药。他接受了常规随访,症状改善良好。
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