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腮腺涎腺导管癌脊髓内转移,酷似放射性孤立综合征患者的横贯性脊髓炎。

Intramedullary spinal cord metastasis from salivary ductal carcinoma of the parotid gland mimicking transverse myelitis in a patient with radiologically isolated syndrome.

作者信息

Ding Dale, Fullard Michelle, Jarrell Heather S, Jones David E

机构信息

University of Virginia, Department of Neurological Surgery, P.O. Box 800212, Charlottesville, VA 22908, United States.

University of Virginia, Department of Neurology, P.O. Box 800394, Charlottesville, VA 22908, United States.

出版信息

J Neurol Sci. 2014 Jan 15;336(1-2):265-8. doi: 10.1016/j.jns.2013.10.025. Epub 2013 Oct 27.

DOI:10.1016/j.jns.2013.10.025
PMID:24199731
Abstract

Intramedullary spinal cord metastases (ISCMs) are rare lesions but their presence should not be underestimated in a cancer patient with rapidly progressive neurological compromise. Due to similar timing of clinical progression and imaging characteristics, these lesions may be misdiagnosed as transverse myelitis, an inflammatory disorder of the spinal cord that may be idiopathic or secondary to other diseases including infections, connective tissue disorders, nutritional deficiencies, and demyelinating disorders. We present a case of a 44 year-old male with a history of parotid gland metastatic salivary ductal carcinoma (SDC) and incidental demyelinating white matter lesions on brain magnetic resonance imaging (MRI) diagnosed as radiologically isolated syndrome with a CSF that was positive for oligoclonal bands. The patient initially presented with mid-thoracic dermatomal numbness, bilateral lower extremity weakness, and neurogenic bladder. MRI spine demonstrated an enhancing T5-7 intramedullary lesion initially diagnosed as transverse myelitis. After progressing to complete motor and sensory loss below T6 despite high-dose intravenous steroids and plasmapheresis, surgical biopsy was undertaken. Intraoperative findings revealed an intramedullary tumor for which a subtotal resection was performed. Pathology was consistent with a metastatic deposit from the patient's primary parotid SDC. The patient underwent postoperative chemotherapy but expired due to systemic disease progression seven months following surgery without neurological improvement. This is the first reported case of ISCM from a primary SDC. The median survival is 6 months for patients with ISCMs treated surgically. The goals of surgery are spinal cord decompression, functional preservation, and tissue diagnosis.

摘要

脊髓髓内转移瘤(ISCMs)是罕见的病变,但在患有快速进展性神经功能损害的癌症患者中,其存在不应被低估。由于临床进展和影像学特征的时间相似,这些病变可能被误诊为横贯性脊髓炎,这是一种脊髓的炎症性疾病,可能是特发性的,也可能继发于其他疾病,包括感染、结缔组织疾病、营养缺乏和脱髓鞘疾病。我们报告一例44岁男性,有腮腺转移性涎腺导管癌(SDC)病史,脑磁共振成像(MRI)上偶然发现脱髓鞘性白质病变,诊断为放射学孤立综合征,脑脊液寡克隆带阳性。患者最初表现为胸中段皮节麻木、双侧下肢无力和神经源性膀胱。脊柱MRI显示T5 - 7节段髓内病变强化,最初诊断为横贯性脊髓炎。尽管给予大剂量静脉类固醇和血浆置换治疗,但病变进展至T6以下完全运动和感觉丧失,遂进行手术活检。术中发现为髓内肿瘤,行次全切除。病理结果与患者原发性腮腺SDC的转移灶一致。患者术后接受化疗,但术后7个月因全身疾病进展死亡,神经功能无改善。这是首例原发性SDC导致的ISCM报道。接受手术治疗的ISCM患者的中位生存期为6个月。手术的目标是脊髓减压、功能保留和组织诊断。

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