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首页> 美国卫生研究院文献>Radiology Case Reports >Intraosseous malignant peripheral nerve sheath tumor of the sacrum in a patient with neurofibromatosis type I
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Intraosseous malignant peripheral nerve sheath tumor of the sacrum in a patient with neurofibromatosis type I

机译：I型神经纤维瘤病患者the骨骨内恶性周围神经鞘瘤

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Malignant intraosseous peripheral nerve sheath tumor is a very rare malignancy most commonly seen in patients with neurofibromatosis type 1. This tumor almost exclusively occurs in the maxillofacial region, with manifestation of this tumor in other regions of the skeleton infrequently reported. We describe a 23-year-old female with previously undiagnosed neurofibromatosis type 1 presenting with lower extremity weakness, paresthesias, and bowel/bladder symptoms. The patient had an aneurysmal lytic bone lesion centered in the upper sacrum with invasion of the L5 vertebral body. On MRI, the lesion was homogeneously isointense to muscle on T1, heterogeneously hyperintense to muscle on T2, and demonstrated homogeneously avid contrast enhancement. Multiple additional small lesions with similar imaging characteristics were identified in the paraspinal soft tissues. Low grade malignant peripheral nerve sheath tumor of the sacrum was diagnosed on biopsy. The patient was treated with sacral resection and radiation therapy for local disease control.
机译：恶性骨内周围神经鞘瘤是一种非常罕见的恶性肿瘤，最常见于1型神经纤维瘤病患者。这种肿瘤几乎只发生在颌面部区域，很少在骨骼的其他区域出现。我们描述了一个23岁的女性，以前未诊断为1型神经纤维瘤病，表现为下肢无力，感觉异常和肠/膀胱症状。该患者的动脉瘤溶解性骨病变集中在骨上，伴有L5椎体的侵犯。在MRI上，病变在T1上对肌肉均等强度，在T2上对肌肉均质不均匀，并且显示出均一的对比度增强。在椎旁副软组织中发现了多个具有相似成像特征的其他小病变。活检诊断为the骨低度恶性周围神经鞘瘤。该患者接受了re骨切除和放射治疗以控制局部疾病。

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