Patibandla Mohana Rao, Uppin Shantveer G, Thotakura Amit Kumar, Panigrahi Manas K, Challa Sundaram
Nizam's Institute of Medical Sciences, Department of Neurosurgery, Hyderabad, India.
Turk Neurosurg. 2013;23(1):98-103. doi: 10.5137/1019-5149.JTN.4131-11.1.
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.
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。