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婴儿海绵窦原发性尤因肉瘤:一例报告并文献复习

Primary Ewings sarcoma of cavernous sinus in an infant: a case report and review of literature.

作者信息

Patibandla Mohana Rao, Uppin Shantveer G, Thotakura Amit Kumar, Panigrahi Manas K, Challa Sundaram

机构信息

Nizam's Institute of Medical Sciences, Department of Neurosurgery, Hyderabad, India.

出版信息

Turk Neurosurg. 2013;23(1):98-103. doi: 10.5137/1019-5149.JTN.4131-11.1.

Abstract

Ewing's / peripheral primitive neuroectodermal tumor (pPNET) of the cavernous sinus are extremely uncommon. We present clinical, radiological, pathological findings of Ewing's / pPNET involving the cavernous sinus in an eleven-month-old infant presenting with proptosis and 6th nerve palsy. MRI Contrast scans showed a well-defined, homogenously enhancing mass involving the right cavernous sinus location. Histopathology and immunohistochemical features were consistent with Ewing's/pPNET. The patient succumbed to disease in spite of starting chemotherapy. The clinical, radiological and pathological findings of previously reported cases of Ewing's sarcoma / pPNET involving the cavernous sinus are summarized. The histological differential diagnosis of primary intracranial Ewing's sarcoma / pPNET are discussed.

摘要

海绵窦的尤因肉瘤/外周原始神经外胚层肿瘤(pPNET)极为罕见。我们报告了一名11个月大、表现为眼球突出和第六脑神经麻痹的婴儿,其海绵窦受累的尤因肉瘤/pPNET的临床、放射学和病理学表现。MRI增强扫描显示一个边界清晰、均匀强化的肿块,位于右侧海绵窦区。组织病理学和免疫组化特征与尤因肉瘤/pPNET相符。尽管开始了化疗,患者仍死于该疾病。总结了先前报道的累及海绵窦的尤因肉瘤/pPNET病例的临床、放射学和病理学表现。讨论了原发性颅内尤因肉瘤/pPNET的组织学鉴别诊断。

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