鞍脊索瘤模仿无功能垂体腺瘤1例,存活超过10年。

T Kagawa, M Takamura, K Moritake, A Tsutsumi, T Yamasaki
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引用次数: 0

摘要

一个罕见的病例鞍脊索瘤发生在一个67岁的妇女谁存活超过10年提出。临床体征和症状伪装成无功能的垂体腺瘤,伴有视觉障碍和垂体功能减退。最初的计算机断层扫描(CT)显示鞍内混合致密肿块伴鞍上延伸,并伴有不均匀的对比增强。通过右侧额颞开颅完成部分肿块切除。组织学检查显示为典型脊索瘤,无恶性。术后放疗后,患者临床好转出院。治疗后8年的连续CT和磁共振成像显示鞍内病变再生,扩展到蝶窦和斜坡,并伴有不均匀的对比增强。患者经唇下蝶窦入路行复发肿瘤次全切除。组织学检查证实了先前的诊断。免疫组化研究显示细胞浆中波形蛋白、上皮膜抗原、角蛋白和S-100蛋白表达阳性,而癌胚抗原未见表达。再次手术后,她完全康复,并存活了10多年,生活质量良好。
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A case of sellar chordoma mimicking a non-functioning pituitary adenoma with survival of more than 10 years.

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.

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