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胫骨尤因肉瘤鞍区及鞍上转移罕见病例

Rare Case of Sellar and Suprasellar Metastasis from Ewing's Sarcoma of Tibia.

作者信息

Gupta Vinita, Aggarwal Kirti, Nishant Prateek, Arora Rajnish Kumar

机构信息

Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Department of Neurosurgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

出版信息

J Neurosci Rural Pract. 2019 Jan-Mar;10(1):158-161. doi: 10.4103/jnrp.jnrp_139_18.

Abstract

We hereby report a case of metastatic Ewing's sarcoma presenting with rapid-onset total ophthalmoplegia, optic atrophy, and right temporal hemianopia. Comprehensive ophthalmic and neurological evaluation with targeted radioimaging revealed a tumor mass centered over the sella, compressing optic chiasma, extending to involve the left cavernous sinus and the left orbital apex. Whole-body imaging revealed the evidence of multifocal lung and mediastinal metastasis with focal lytic defect in the left femoral head. Histopathological evaluation of transnasal punch biopsy from the nasopharyngeal extension of the tumor revealed small round-cell tumor with strong CD99 positivity, supporting the diagnosis of Ewing's sarcoma. Rapid, aggressive extensions of the metastatic tumor into vital structures despite the initiation of chemoradiation of the extensive intracranial tumor led to unexpected demise of the patient. Our case is an unusual case of Ewing's sarcoma metastasis manifesting as a sellar mass and mimicking a pituitary adenoma radiologically, with a rapid progression within 2 weeks to cause massive extension of tumor into suprasellar, infrasellar, and left parasellar area, indicative of highly malignant nature of the tumor.

摘要

我们在此报告一例转移性尤因肉瘤病例,该病例表现为迅速发作的完全性眼肌麻痹、视神经萎缩和右侧颞侧偏盲。通过全面的眼科和神经科评估以及针对性的放射成像检查发现,肿瘤块位于蝶鞍上方,压迫视交叉,延伸至左侧海绵窦和左侧眶尖。全身成像显示有多发性肺和纵隔转移的证据,左侧股骨头有局灶性溶骨性缺损。对肿瘤鼻咽部延伸部位进行经鼻穿刺活检的组织病理学评估显示为小圆形细胞瘤,CD99呈强阳性,支持尤因肉瘤的诊断。尽管对广泛的颅内肿瘤进行了放化疗,但转移性肿瘤仍迅速、侵袭性地向重要结构扩展,导致患者意外死亡。我们的病例是尤因肉瘤转移的罕见病例,表现为蝶鞍区肿块,影像学上酷似垂体腺瘤,在2周内迅速进展,导致肿瘤大量扩展至鞍上、鞍下和左侧鞍旁区域,表明肿瘤具有高度恶性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1abf/6338001/e5b334afa085/JNRP-10-158-g001.jpg

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