Topsakal Cahide, Bulut Serpil, Erol Fatih Serhat, Ozercan Ibrahim, Yildirim Hanefi
Department of Neurosurgery, Firat University, School of Medicine, Elazig, Turkey.
Neurol Med Chir (Tokyo). 2002 Apr;42(4):175-80. doi: 10.2176/nmc.42.175.
A 44-year-old woman presented with a thoracic chordoma with intrathoracic extension manifesting as complaints of lower extremity weakness, hypesthesia below the levels of T5-6, and sphincter incontinence. Almost total resection combined with anterior interbody fusion and stabilization was possible through a left transpleural transthoracic approach. She suffered recurrence after 2 years and was considered inoperable. Biopsy revealed a malignant chordoma with no sarcomatous differentiation. Chordoma is an uncommon malignant bone tumor originating from remnants of the embryonal notochord, occurring mostly along the axial skeleton, at the extremity of the vertebral spine, and is least common in the thoracic region. Differential diagnosis is problematic and biopsy is helpful particularly if considered inoperable. Thoracic chordomas of the malignant type manifest as cord or root compression. Classical malignant chordomas must be distinguished from chondroid, benign, or other types of chordomas, since the biological behavior and clinical features are distinct. However, the differential diagnosis cannot be based on histological examination, but long-term follow up is required. Most patients have extradural and intraspinal tissue extension at the time of diagnosis, which makes complete resection impossible. Aggressive surgery without violation of surgical borders is the best choice in the treatment of thoracic chordoma. Thoracic chordoma is a recurring neoplasm and is prone to dissemination and sarcomatous differentiation despite its slow-growing nature.
一名44岁女性因胸段脊索瘤伴胸内扩展就诊,表现为下肢无力、T5 - 6水平以下感觉减退及括约肌失禁。通过左经胸膜开胸入路,几乎实现了全切除,并结合前路椎间融合与稳定术。2年后她复发,被认为无法手术。活检显示为无肉瘤分化的恶性脊索瘤。脊索瘤是一种罕见的恶性骨肿瘤,起源于胚胎脊索的残余组织,大多沿中轴骨骼发生,位于脊柱末端,在胸段最为少见。鉴别诊断存在困难,活检尤其在考虑无法手术时很有帮助。恶性胸段脊索瘤表现为脊髓或神经根受压。经典的恶性脊索瘤必须与软骨样、良性或其他类型的脊索瘤相鉴别,因为它们的生物学行为和临床特征不同。然而,鉴别诊断不能仅基于组织学检查,还需要长期随访。大多数患者在诊断时已有硬膜外和椎管内组织侵犯,这使得完全切除不可能。在不违反手术边界的情况下积极手术是治疗胸段脊索瘤的最佳选择。胸段脊索瘤是一种复发性肿瘤,尽管生长缓慢,但易于播散和肉瘤分化。