颅底脊索瘤的病理与临床研究

Clinical and pathological study of chordoma in the skull base

目的 研究脊索瘤的组织病理及临床诊断和治疗。方法 观察 2 0例脊索瘤的临床表现 ,影像所见及组织病理特征 ;对 7例脊索瘤作免疫组化标记抗原染色 ;对 2例脊索瘤作电镜观察 ,19例手术治疗 ,其中 6例鼻侧及腭间进路中以内镜辅助下完成手术 ,11例加放射治疗 ,随访观察 3～ 5年。结果 ①根据症状和体征、影像所见、原发部位、扩展范围和手术所见 2 0例临床分型 :蝶鞍型 5例 ,斜坡型 9例 ,枕颞型 2例 ,广泛型 4例 ,其中 1例广泛型有多发性颅神经损害 ,未手术死亡 ,尸解诊断为脊索瘤肺转移 ,肿瘤压迫脑干生命中枢致死 ;3,5年生存率分别为 6 5 %和 35 % ;②脊索瘤的病理检查见特征性的液滴细胞 ;③免疫组化标记蛋白染色中上皮性抗原的阳性率比其他间叶肿瘤上皮性抗原阳性率高 ;透射电镜显示细胞内有多量扩张的内质网及胞浆微丝。结论 脊索瘤是具有上皮性及间叶性抗原分化特性的低度恶性肿瘤 ,以液滴细胞为组织病理学特征 ,免疫组化染色有助于鉴别诊断。完全切除颅底脊索瘤在技术上是不可能的 ,术后易复发 ,可发生转移。

Objective To study the clinical and pathological characteristics of chordoma in the skull base Method The clinical manifestation, radiological finding and pathological characteristics in 20 patients with skull base chordoma were reviewed Among them, 7 were immunohistochemical stained for keratin, epithelial membrane antigen, vimentin, glial fibrillary acidic protein and desmen, 2 were studied by electron microscope 19 patients received surgical resection(5 cases were assisted by nasal endoscope) Eleven patients received radiation therapy All 19 patients were followed up for 3 5 years after treatment Results (1) Clinical type were divided into four types in this group according to symptoms, sign, imaging features, operation finding, the original places and the expansive direction of the tumor. Seller type 5 cases, clival type 9 cases, occipito temproral type 2 cases and extent type 4 cases. One case with multiple cranial never palsies died due to the pressure on brain stem by the neoplasm, was autopsied prove lungs metastasis. 5 years survival rates were 65% and 35% respectively. (2) Vacuolated physaliferous cell is the pathology characteristic of chordoma. (3) Positive rate of epithelial marker antigens are higher than other mesenchymal neoplasms. Distended rough endoplasmic reticulum and filament were found within the cytoplasm under transmission electron microscope. Conclusion Chordoma is a kind of low malignancy which express epithelial and mesenchymal characteristics. Vacuolated physaliferous cell is the characteristic Pathologic appearance. Immunohistiochemical stain is helpful in differential diagnosis. Complete excision is still technically impossible. Chordoma maintain high recurrence rate after surgery, with metastasis. Combined modality therapy include surgical operation and radiation could prolonged survival period.