Yamashita Y, Kumabe T, Kobayashi T, Abiko H, Seki H, Yoshimoto T
Department of Neurosurgery, Ohara Medical Center Hospital.
No Shinkei Geka. 1997 Jun;25(6):567-71.
Primary cranial Ewing's sarcoma is rare. We describe an exceptionally rare case of primary Ewing's sarcoma of the occipital bone, presenting as spontaneous acute epidural hematoma. A 19-year-old female was admitted to our hospital complaining of sudden onset of severe headache. There were no neurological deficit and no abnormal laboratory findings. Computerized tomographic (CT) scan revealed a lentiform shaped high density lesion at the left occipital epidural space. Magnetic resonance imaging (MRI) showed the lesion as iso to low intensity on T1-weighted image (T1WI) and mixed signal intensity on T2-weighted image (T2WI). There were no pathological findings at the adjacent brain. Cerebral angiography demonstrated mass effect. Right occipital craniotomy was performed. We found the tumor arising from the occipital bone and located at the epidural space. The tumor was resected totally. Histological examination revealed the tumor as Ewing's sarcoma with intratumoral hemorrhage. The postoperative course was uneventful. Radiation therapy (50Gy) was given. Follow-up examination six years after the treatment found no evidence of tumor recurrence or distant metastasis. It should be born in mind that primary Ewing's sarcoma of the skull can cause spontaneous acute epidural.
原发性颅骨尤因肉瘤罕见。我们描述了一例极为罕见的枕骨原发性尤因肉瘤，表现为自发性急性硬膜外血肿。一名19岁女性因突发剧烈头痛入院。无神经功能缺损，实验室检查无异常。计算机断层扫描（CT）显示左枕部硬膜外间隙有一透镜状高密度病变。磁共振成像（MRI）显示该病变在T1加权像（T1WI）上呈等至低信号强度，在T2加权像（T2WI）上呈混合信号强度。相邻脑组织无病理改变。脑血管造影显示有占位效应。行右枕部开颅手术。我们发现肿瘤起源于枕骨，位于硬膜外间隙。肿瘤被完全切除。组织学检查显示肿瘤为尤因肉瘤伴瘤内出血。术后病程顺利。给予放射治疗（50Gy）。治疗后六年的随访检查未发现肿瘤复发或远处转移的证据。应牢记颅骨原发性尤因肉瘤可导致自发性急性硬膜外血肿。
