Chordoma of the Sellar Region Mimicking Meningioma: A Literature Review and Illustrative Case

K. Guettabi, Jihad Echnin, Abdelkoudouss Laaidi, S. Hilmani, K. Ibahioin, Abdessamad Naja, A. Lakhdar
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Abstract

Background: Chordoma is a rare malignant tumor that accounts for 1-4% of primary malignant bone tumors arising primarily in the axial skeleton and primarily affects adults. It seems to have difficulty with diagnosis and treatment. Case description: A 63-year-old man with a history of diabetes treated with oral antidiabetic medication and hypertension. Presented with two years of bilateral decrease in visual acuity. Neurological examination revealed left ptosis, divergent strabismus, and higher cerebral function disorders. Brain MRI revealed a sellar and suprasellar lesion evokes a sellar meningioma, macro adenoma. Hormonal laboratory tests revealed that the prolactin level was elevated to 140ng/ml without any other hormonal disorder. The patient underwent partial surgical resection of the lesion through endoscopic endonasal. Histopathological examination revealed a chondroid chordoma. Postoperatively the patient had no endocrinological or neurological complications, but he kept the starbism and the ptosis in the left eye. Conclusion: In the context of progressive ophthalmological signs by a sellar and suprasellar lesion, the differential diagnosis should include chordoma along with meningioma, macro adenoma and metastasis. Complete and early surgical removal is the first line treatment.
鞍区似脑膜瘤脊索瘤:一个文献回顾和说明性病例
背景:脊索瘤是一种罕见的恶性肿瘤,占原发性恶性骨肿瘤的1-4%,主要发生在中轴骨骼,主要影响成年人。它似乎很难诊断和治疗。病例描述:63岁男性,有糖尿病病史,口服降糖药治疗并伴有高血压。表现为两年的双侧视力下降。神经学检查显示左上睑下垂、发散性斜视及高级脑功能障碍。脑MRI显示鞍区及鞍上病变提示鞍区脑膜瘤,大腺瘤。激素实验室检查显示催乳素水平升高至140ng/ml,无其他激素紊乱。患者经鼻内窥镜行部分病变手术切除。组织病理学检查显示为软骨样脊索瘤。术后患者无内分泌及神经系统并发症,但左眼仍有斜视及上睑下垂。结论:在鞍区及鞍上病变的进展性眼科征象中,脊索瘤合并脑膜瘤、大腺瘤及转移应予以鉴别诊断。完全和早期的手术切除是第一线的治疗。
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