首页> 外文期刊>Journal of clinical neuroscience: official journal of the Neurosurgical Society of Australasia >Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis
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Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis

机译:马尾恶性周围神经鞘瘤伴颅骨棘突转移

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Intradural spinal malignant peripheral nerve sheath tumors (MPNST) are extremely rare, with only 20 adult patients reported to our knowledge, and only four primary tumors arising from the cauda equina. A 49-year-old man presented with back pain, constipation, and lower extremity weakness and was found to have a large intradural lesion involving the cauda equina. Imaging of the rest of his neuraxis revealed additional small left temporal lobe, cervical, and thoracic lesions. The patient underwent laminectomy for tumor debulking and biopsy, as gross total resection was not possible due to envelopment of the cauda equina. Histopathology revealed a MPNST with high cellularity, elevated proliferative indices, and nerve fascicle invasion. After the debulking, the patient reported improvement in his symptoms. However, 6 weeks later, the patient began having severe headaches, and his left temporal lobe lesion was found to have increased significantly in size, requiring craniotomy for palliative resection. The authors report the first adult patient with sporadic spinal MPNST with craniospinal metastasis to our knowledge. Imaging of the entire neuraxis is recommended for initial workup of these lesions, which are capable of intradural spread. The best treatment approach is unclear, but total surgical resection should be attempted, barring infiltration and engulfment of the nerve roots or widespread dissemination. (C) 2014 Elsevier Ltd. All rights reserved.
机译:硬膜内脊髓恶性周围神经鞘瘤(MPNST)极为罕见,据我们所知仅报道了20名成年患者,并且只有4例由马尾引起的原发性肿瘤。一名49岁的男性出现背部疼痛,便秘和下肢无力,并且发现其硬膜内病变累及马尾神经。其余神经影像学检查显示,左颞颞叶,颈椎和胸廓有其他小病变。该患者接受了椎板切除术,以切除肿瘤并进行活检,因为马尾神经包绕术无法进行大体全切除。组织病理学显示MPNST具有高细胞性,增高的增殖指数和神经束侵袭。减轻体重后,患者报告症状有所改善。然而,在6周后,患者开始出现严重头痛,并且发现左颞叶病变的大小明显增加,需要开颅手术进行姑息性切除。根据我们的知识,作者报告了第一例散发性脊柱MPNST合并颅脑脊髓转移的成年患者。建议对这些病灶进行初次检查以对整个神经轴成像,这些病灶可以在硬膜内扩散。最好的治疗方法尚不清楚,但应尝试进行全面手术切除,以免神经根浸润和吞噬或广泛散布。 (C)2014 Elsevier Ltd.保留所有权利。

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