Surgical Excision of Large Sacral Chordoma with Lumbopelvic Fixation

Ramadan Shamseldien
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Abstract

Background: Chordoma is slowly growing locally malignant destructive tumor originated as a remnant of notochord. It occur less than 5% of all bone tumors. It has a preferentiality to attack sacrum up to 50%, followed by base of the skull up to 40% and to less extent other vertebral regions up to 15%. Local pain, radiculopathy, and urinary incontinence are the usual presenting symptoms in case of Sacral Chordoma. Contrast enhanced MRI and CT are recommended studies to evaluate extent of both soft tissue invasion and displacement and bone destruction and calcification. This lesion is resistant to chemotherapy and weak response to radiotherapy make the surgical excision first line of treatment. Aggressive surgical excision and stabilization of lumbosacral spin and pelvis improve the local control in spite of risk of associated morbidity. Sacral Chordoma is reported to be poor prognosis. The optimal method for management of this lesion is debatable due to its rarity. Aim: To present a case of Sacral Chordoma with very large local extension and sacral destruction. Study design: Case report. Methods: our 60 years old male patient presented with lower lumbar and sacral swelling, bilateral gluteal pain, and obstructive uropathy. Slowly progressive within the last nine months prior to presentation; contrast enhanced MRI and CT lumbosacral spine, show a large sacral destructive heterogeneous mass lesion destructing the sacrum and intraspinal structures apart from first sacral segment. By CT guided biopsy at oncology institute diagnosed as Sacral Chordoma. He was subjected to gross surgical total excision, and posterior lumbosacral transpedicular screw fixation concomitant with iliac screw for sacral augmentation, then adjuvant radiation therapy. Results: After tumor excision and lumbopelvic fixation, the patient still incontinent, full motor power of both lower limbs. Complete course of adjuvant radiation. And after one year follow up the patient died due to bad general condition. Conclusion: Sacral Chordoma is infrequent lesion that affect male more than females. Gross surgical excision is the first line of treatment. Lumbopelvic stabilization is mandatory when extensive sacral destruction exist. Sacral Chordoma reported to be poor prognosis.
大骶脊索瘤手术切除联合腰盂固定术
背景:脊索瘤是一种生长缓慢的局部恶性破坏性肿瘤,起源于脊索的残余。它在所有骨肿瘤中所占比例不到5%。它偏爱攻击高达50%的骶骨,其次是高达40%的颅底,其他椎体区域较少,高达15%。局部疼痛、神经根病和尿失禁是骶脊索瘤的常见症状。对比增强MRI和CT被推荐用于评估软组织侵犯和移位以及骨破坏和钙化的程度。这种病变对化疗有抵抗力,对放疗反应弱,因此手术切除是治疗的第一线。尽管存在相关疾病的风险,但积极的手术切除和稳定腰骶部旋转和骨盆可以改善局部控制。据报道,骶脊索瘤预后不良。治疗这种病变的最佳方法是有争议的,因为它的罕见。目的:报告一例骶脊索瘤伴很大的局部延伸和骶骨破坏。研究设计:病例报告。方法:我们的60岁男性患者表现为下腰椎和骶骨肿胀,双侧臀痛和梗阻性尿病。在发病前的最后9个月内缓慢进展;腰骶骨MRI和CT增强显示,除第一骶段外,骶骨和椎管内结构均受到巨大的骶骨破坏性非均匀肿块损害。肿瘤研究所CT引导活检诊断为骶脊索瘤。患者行大手术全切除,腰骶后路经椎弓根螺钉固定联合髂螺钉行骶骨增强术,然后行辅助放疗。结果:经肿瘤切除及腰盂固定术后,患者仍能保持大小便失禁,双下肢运动能力完全。完成辅助放疗疗程。随访1年后,患者因一般情况不佳死亡。结论:骶脊索瘤是一种少见的病变,男性多于女性。大体手术切除是治疗的第一线。当广泛的骶骨破坏存在时,腰骨盆稳定是强制性的。据报道,骶脊索瘤预后不良。
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