Kagawa T, Takamura M, Moritake K, Tsutsumi A, Yamasaki T
Department of Neurosurgery, Shimane Medical University.
Noshuyo Byori. 1993;10(2):103-6.
A rare case of sellar chordoma occurring in a 67-year-old woman who survived for more than 10 years is presented. Clinical signs and symptoms masqueraded as a non-functioning pituitary adenoma with visual disturbance and hypopituitarism. Initial computed tomography (CT) showed an intrasellar mixed dense mass with suprasellar extension, accompanied by non-homogeneous contrast enhancement. Partial removal of the mass was accomplished via right fronto-temporal craniotomy. Histological examination revealed a typical chordoma with no malignancy. After postoperative irradiation, the patient was discharged with clinical improvement. Serial CT and magnetic resonance imaging 8 years after treatment disclosed a regrowth of the intrasellar lesion, which extended to the sphenoid sinus and clivus, accompanied by non-homogeneous contrast enhancement. The patient underwent subtotal removal of the recurrent tumor through a sublabial transsphenoidal approach. Histological examination confirmed the previous diagnosis. Immunohistochemical study demonstrated positive cytoplasmic expression of vimentin, epithelial membrane antigen, keratin and S-100 protein, in contrast with a lack of appearance of carcinoembryonic antigen. After reoperation, she completely recovered and has survived for more than 10 years with good quality of life.
本文报告了一例发生于一名67岁女性的鞍区脊索瘤罕见病例，该患者存活超过10年。临床症状和体征伪装成伴有视力障碍和垂体功能减退的无功能性垂体腺瘤。最初的计算机断层扫描（CT）显示鞍内混合密度肿块并向鞍上延伸，伴有不均匀强化。通过右额颞开颅手术部分切除了肿块。组织学检查显示为典型的无恶性特征的脊索瘤。术后放疗后，患者临床症状改善出院。治疗8年后的系列CT和磁共振成像显示鞍内病变复发，延伸至蝶窦和斜坡，伴有不均匀强化。患者通过经唇下经蝶窦入路接受了复发性肿瘤的次全切除。组织学检查证实了先前的诊断。免疫组织化学研究显示波形蛋白、上皮膜抗原、角蛋白和S-100蛋白在细胞质中呈阳性表达，而癌胚抗原未出现。再次手术后，她完全康复，存活超过10年，生活质量良好。
