Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China; Beijing Neurosurgical Institute, Capital Medical University, Beijing, People's Republic of China.
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, People's Republic of China.
World Neurosurg. 2014 Nov;82(5):791-8. doi: 10.1016/j.wneu.2012.12.037. Epub 2013 Feb 13.
To investigate the clinical and pathologic characteristics of primary intradural retroclival chordoma and improve the understanding of this rare disease.
A retrospective study was conducted on six cases patients withof an intradural chordoma in the retroclival region who underwent surgery and were confirmed by pathology and imaging. Expression of brachyury, galectin-3, and Ki-67 in paraffin-embedded sections of the specimens was detected by streptavidin-peroxidase immunohistochemistry.
Lesions were located in the subdural prepontine cistern. Computed tomography scan showed that the bone of the skull base was not destroyed, and findings on magnetic resonance imaging varied, resulting in a misdiagnosis of 50% of the cases in the preoperative imaging. All six cases were classified by their pathology into the classical subtype. They presented a strong positive staining for brachyury and galectin-3, and the Ki-67 labeling index was between 2.5% and 8.2%. Three cases presented no signs of recurrence or regrowth, whereas in the other three patients recurrence or regrowth occurred at 7 ∼ 14 months after initial surgery. Two patients died of this disease.
Our study suggests that a positive staining for brachyury, galectin-3, and Ki-67 would be helpful for differential diagnosis, discriminating intradural retroclival chordoma from ecchordosis physaliphora and chordoid meningioma. Our study also shows that within intradural retroclival chordoma, there are significant prognostic differences. Tumors with an abundant blood supply, flake-like cellular arrangement, and a Ki-67 labeling index greater than 5% belong to a rapid-growth type and are prone to short-term recurrence and poorer prognosis.
探讨原发性椎管内硬膜下脊索瘤的临床病理特征，提高对这种罕见疾病的认识。
对 6 例经手术证实且病理和影像学检查均为椎管内硬膜下脊索瘤的患者进行回顾性研究。采用链霉亲和素-过氧化物酶免疫组化法检测石蜡切片中 brachyury、galectin-3 和 Ki-67 的表达。
病变位于桥前池硬膜下。CT 扫描显示颅底骨质无破坏，磁共振成像结果多样，导致术前影像学误诊率为 50%。所有 6 例均按其病理分为经典亚型。它们均对 brachyury 和 galectin-3 呈强阳性染色，Ki-67 标记指数为 2.5%～8.2%。3 例无复发或生长迹象，而另外 3 例在初次手术后 7～14 个月复发或生长。2 例患者死于该疾病。
我们的研究表明，bracyury、galectin-3 和 Ki-67 的阳性染色有助于鉴别诊断，将硬膜下脊索瘤与软骨黏液样纤维瘤和脊索样脑膜瘤区分开来。我们的研究还表明，在硬膜下脊索瘤中，存在显著的预后差异。具有丰富血供、片状细胞排列和 Ki-67 标记指数大于 5%的肿瘤属于快速生长型，易发生短期复发和预后较差。
