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一种不寻常的双凸硬膜外病变:急性表现的颅外尤文肉瘤。

An unusual biconvex epidural lesion: acutely presenting extraosseous intracranial Ewing's sarcoma.

作者信息

Jan Kalimullah, Khan Eraj Khursheed, Khan Inamullah

机构信息

Department of Neurosurgery, Aga Khan University Hospital, Karachi, Pakistan.

Present Address: Department of Medicine (Neurology), Changi General Hospital Singapore, Simei, Singapore.

出版信息

Chin Neurosurg J. 2018 Nov 14;4:31. doi: 10.1186/s41016-018-0139-2. eCollection 2018.

Abstract

BACKGROUND

Ewing's sarcoma family of tumors consists of small round cell neoplasms, inclusive of primitive neuroectodermal tumor (PNET), Askin's tumor, and PNET of the bone. Extraosseous Ewing's sarcoma occurs commonly at bones of lower extremities and paravertebral region of the spine. It rarely presents as a primary intracranial lesion. When intracranial, it can be misdiagnosed as central PNET (e.g., medulloblastoma, pinealoblastoma, or supratentorial PNET), other intracranial lesions, or even as an epidural hematoma.

CASE PRESENTATION

We report the case of a 20-year-old patient who presented to the emergency department with complaints of drowsiness, headache, and fever for 1 day. On initial computed tomography (CT) scan of the brain, a right temporal biconvex epidural lesion involving squamous-temporal bone with periosteal reaction was noted.The patient underwent urgent craniotomy, and a tumor was found and excised. Biopsy report confirmed Ewing's sarcoma.

CONCLUSION

Ewing's sarcoma is a rare intracranial malignancy with only a few cases reported in literature. In a young patient with a biconvex epidural lesion, in the absence of trauma or ongoing infection, the possibility of Ewing's sarcoma should be considered as well.

摘要

背景

尤因肉瘤家族性肿瘤由小圆细胞肿瘤组成,包括原始神经外胚层肿瘤(PNET)、阿金氏瘤和骨PNET。骨外尤因肉瘤常见于下肢骨骼和脊柱椎旁区域。它很少表现为原发性颅内病变。当发生在颅内时,它可能被误诊为中枢性PNET(如髓母细胞瘤、松果体母细胞瘤或幕上PNET)、其他颅内病变,甚至硬膜外血肿。

病例报告

我们报告一例20岁患者,因嗜睡、头痛和发热1天就诊于急诊科。在初次脑部计算机断层扫描(CT)时,发现右侧颞部双凸硬膜外病变累及鳞状颞骨并伴有骨膜反应。患者接受了紧急开颅手术,发现并切除了肿瘤。活检报告证实为尤因肉瘤。

结论

尤因肉瘤是一种罕见的颅内恶性肿瘤,文献中仅报道了少数病例。在年轻患者中,若出现双凸硬膜外病变,且无外伤或正在进行的感染,也应考虑尤因肉瘤的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5764/7398318/34156149867a/41016_2018_139_Fig1_HTML.jpg

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