Sun H Ibrahim, Guduk Mustafa, Gucyetmez Bulent, Yapicier Ozlem, Pamir M Necmettin
Acibadem University, School of Medicine, Department of Neurosurgery, Istanbul, Turkey.
Turk Neurosurg. 2018;28(2):174-178. doi: 10.5137/1019-5149.JTN.18587-16.1.
Chordomas are rare, slow growing but locally aggressive malignancies of the axial skeleton. Skull base chordomas, due to their intricate anatomical localization, pose significant challenges to managing physicians. In classical and chondroid chordomas, the disease course cannot be reliably determined using only morphological criteria. Brachyury (T Gene) was shown to play a central role in chordoma pathogenesis and several studies also showed that this gene also carries potential as a prognostic biomarker. This study aims to correlate Brachyury expression with the clinical course in surgically treated skull base chordomas.
Chordoma tumor samples from 14 patients with skull base chordomas, diagnosed using histopathological and immunohistochemistry criteria (epithelial membrane antigen (EMA), S100, pan cytokeratin (panCK)) were retrospectively analyzed for Brachyury expression using immunohistochemistry. Brachyury expression was graded using a 4 point semi-quantitative scoring system. Focal (grade II) and diffuse staining (grade III) were considered as overexpression. Patient recurrence-free survival and total survival were compared between Brachyury overexpressing and non-overexpressing groups using Kaplan-Meier survival analysis.
Among the stained tumor samples, 85.7% were positive for brachyury expression. In both groups, there was one sample that was negative. We did not observe any significant difference among the groups for staining, grade and percentage of brachyury positive cells.
Brachyury expression in tumor samples is not a sensitive indicator of prognosis in chordomas.
脊索瘤是一种罕见的、生长缓慢但具有局部侵袭性的中轴骨恶性肿瘤。颅底脊索瘤由于其复杂的解剖定位，给治疗医生带来了重大挑战。在经典型和软骨样脊索瘤中，仅使用形态学标准无法可靠地确定疾病进程。研究表明，短尾型蛋白（T基因）在脊索瘤发病机制中起核心作用，多项研究还表明该基因也具有作为预后生物标志物的潜力。本研究旨在将短尾型蛋白的表达与手术治疗的颅底脊索瘤的临床病程相关联。
回顾性分析14例经组织病理学和免疫组织化学标准（上皮膜抗原（EMA）、S100、全细胞角蛋白（panCK））诊断为颅底脊索瘤的患者的脊索瘤肿瘤样本，采用免疫组织化学法分析短尾型蛋白的表达。短尾型蛋白的表达采用4分半定量评分系统进行分级。局灶性（II级）和弥漫性染色（III级）被视为过表达。使用Kaplan-Meier生存分析比较短尾型蛋白过表达组和非过表达组患者的无复发生存率和总生存率。
在染色的肿瘤样本中，85.7%的短尾型蛋白表达呈阳性。两组各有1个样本为阴性。我们未观察到两组在染色、分级和短尾型蛋白阳性细胞百分比方面存在任何显著差异。
肿瘤样本中的短尾型蛋白表达不是脊索瘤预后的敏感指标。
