Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant

Primary spinal cord primitive neuroectodermal tumor (PNET) is a rare entity. In all, 13 cases have been reported in the literature, including 3 with intracranial seeding. A 3-month-old girl with involvement of the spinal cord below the mid-thoracic level is described. The brain MRI revealed findings indicative of seeding along the intracranial subarachnoid space. Biopsy, duraplasty and removal of laminotomy flap were done. In spite of a good response to the first cycle of postoperative 8-drugs-in-a-day chemotherapy, further treatment was refused. She died 21 days after the onset of leg weakness, which reveals the rapid progression of untreated cases. To our knowledge, this is the first case of spinal cord PNET with parenchymal involvement that has been described in an infant.     

Intraspinal Ewing’s sarcoma/primitive neuroectodermal tumors

Intraspinal Ewing’s sarcoma (ES) and primitive neuroectodermal tumors (PNET) are very rare, and the characteristics and prognoses of the disease remain unclear. We present an illustrative patient with an intradural, extramedullary PNET arising within the cervical spinal canal, with clinical and radiological manifestations of leptomeningeal spread, and review the reports of a further 77 patients with intraspinal ES/PNET. Cox regression analyses showed that tumor location (extradural, intradural) (p = 0.002, RR = 4.217, 95% confidence interval [CI] 1.668-10.664) and spinal segment location (cervical, thoracic, lumbar, or sacral) (p = 0.017, RR = 2.040, 95% CI 1.133-3.673) were independent factors in the prognosis of intraspinal ES/PNET. We concluded that a peripheral PNET may originate within the spinal canal and exhibit leptomeningeal spread similar to that seen in central PNET, and that a patient with an intradural ES/PNET high in the spinal canal is more likely to have a poor prognosis.     

原始性神经外胚瘤及其在中枢神经系统的表现

目的 分析原始性神经外胚瘤(PNET)在全身各系统特别是在中枢神经系统的临床表现及其治疗.方法 结合有关文献,回顾解放军总医院近10年来通过手术治疗的89例PNET,对不同系统发生的PNET进行比较,并总结了同期90例髓母细胞瘤的病例作为对比.结果 89例PNET中,中枢神经系统21例占24%(大脑半球9例,椎管内8例,脑干4例);其他系统68例(76%).21例发生于中枢神经系统的PNET病人共接受手术27次,肿瘤全切18次,次全切9次.15例成功随访的病人有12例复发,占80%;转移4例,占27%;随访病人中已死亡10例.中枢神经系统的PNET平均发病年龄22.0岁,其他系统的PNET平均发病年龄29.2岁,髓母细胞瘤平均发病年龄13.9岁.结论 发生在全身各系统的PNET临床表现相似,病理形态和免疫标记物相同,复发率和转移率很高,手术全切结合化疗有一定疗效.中枢神经系统的PNET与其他系统的PNET虽为同一种疾病,但也有其自身特点.     

Primary spinal primitive neuroectodermal tumour: report of two cases mimicking neurofibroma and review of the literature

Primary spinal primitive neuroectodermal tumours (PNETs) are a rare entity. Most of them occur in children and young adults. To date, 47 cases of primary spinal PNET have been reported in the literature. We present two cases of primary spinal PNET. In both cases, the tumours were thoracic extradural ones with intrathoracic extension through intervertebral foramina resembling neurofibroma. These tumours are highly aggressive with rapid growth as evidenced by the short history in both of our cases. Both cases underwent gross total removal of the intraspinal and thoracic components. Postoperatively, both patients underwent cranio-spinal radiotherapy.A review of the literature shows that the overall prognosis of PNETs of the spinal cord is very poor even with adequate surgery, radiotherapy and chemotherapy. One patient died after 4 months and the other one is still alive 8 months after surgery, radiotherapy and chemotherapy.     

颅内硬脑膜动静脉瘘引起严重脊髓症状(3例报告)

目的 :结合 3例硬脑膜动静脉瘘 (IDAVF)伴脊髓表面引流的影像学、临床表现、病程特点 ,探讨引起脊髓症状的原因。方法 :分析 3例IDAVF伴脊髓表面引流的影像学特点、临床表现、病程发展 ,结合文献加以分析和讨论。结果 :临床症状主要表现为颅内症状者 1例 ,脊髓症状者 2例。结论 :IDAVF伴脊髓表面引流的临床表现与脊髓根静脉的引流出路有关。硬脑膜动静脉瘘伴脊髓症状的诊断较困难 ,特别是仅有脊髓症状者。年轻人中慢性脊髓病变 ,急性加重时应注意颅内血管造影可做出诊断     

Oligodendroglioma of the “widespread” type in the spinal cord

The authors present clinical, radiological, and pathological features of the “widespread” type of primary spinal oligodendroglioma arising in a 12-year-old boy. The initial symptoms were gait disturbance, hypesthesia of the lower trunk, and scoliosis. Gadolinium-enhanced magnetic resonance imaging revealed an intramedullary tumor with syringomyelia. The tumor spread rapidly in a “widespread” pattern within the spinal cord between C7 and T12. One year after surgery the patient had made a poor neurological recovery despite the two-stage tumor resection. Only five cases of widespread tumors, including our case, have been described with discussion of their clinical manifestations. Received: 14 July 1997 Revised: 27 February 1998     

Spontaneous spinal cord herniation: a rare cause of the Brown-Sequard syndrome

Recent reports have shown that spontaneous spinal cord herniation in the thoracic segment of the spine may be one of the causes of the progressive spinal cord lesion. Although it was described for the first time by Wortzman in 1974, it was only in early 1990s that a growing number of publications on single cases of the condition started to be observed. In the relevant literature collected by us we found reports on 53 patients altogether. In a number of cases herniation was diagnosed only intraoperatively in spite of complex radiological diagnostics. The analysis of relevant literature shows that spontaneous or idiopathic spinal cord herniation has a very typical clinical picture and most cases share a number of features, such as the clinical condition, age and sex of the patient as well as the level and location of the entity and its radiological picture. We conclude that spontaneous herniation of the spinal cord may be not as rare as previously thought and that it should be always taken into account in differential diagnostics of progressive myelopathy in the thoracic segment in middle-aged patients, especially females.     

Human tumor necrosis factor-alpha augments experimental allergic encephalomyelitis in rats

The effect of human recombinant tumor necrosis factor-alpha (TNF) on experimental allergic encephalomyelitis (EAE) was studied in Lewis rats. TNF was injected intraperitoneally at a daily dose of 1 x 10(3) or 2 x 10(4) U for 8 consecutive days from one day after sensitization with guinea-pig spinal cord in complete Freund's adjuvant. All rats in the control group developed clinical signs of EAE but recovered within 8 days after the onset. Injections of 2 x 10(4) U/day of TNF resulted in a significant prolongation of clinical EAE: clinical signs were sustained for up to 15 days after onset. Histologically, rats receiving 2 x 10(4) U/day of TNF had more severe cellular infiltrations in the spinal cord than controls. The augmentation of EAE was not found in rats receiving 1 x 10(3) U/day of TNF or TNF that had been neutralized with anti-TNF monoclonal antibody.     

副肿瘤性脊髓病的临床和影像特点

目的报道副肿瘤性脊髓病的临床表现、影像和血清学特征。方法检索宣武医院2010年1月至2019年6月期间的副肿瘤性脊髓病住院患者,系统分析其临床表现、血清学和影像学数据。结果10例患者中符合确定的副肿瘤综合征7例,可能的副肿瘤综合征3例。患者中亚急性起病3例,慢性起病7例,脊髓病起病后确诊时间平均为10.1个月。10例患者中7例表现为单纯副肿瘤性脊髓病,2例伴有周围神经病,1例伴有僵人综合征。10例患者中9例患者行腰穿检查脑脊液,其中8例有异常,5例淋巴细胞增多,5例蛋白升高,6例寡克隆区带阳性;7例患者神经肿瘤抗体阳性,分别为抗amphiphysin抗体1例、抗CRMP-5抗体1例、抗Hu抗体3例、抗Yo抗体1例、抗Ri抗体1例;7例患者病理证实为恶性肿瘤。10例患者中,脊髓磁共振异常5例,其中长脊髓病变4例(其中累及椎体节段14个1例,6个1例,3个2例),短脊髓病变1例,病灶均对称性累及传导束或者脊髓灰质。10例患者中9例接受治疗,其中单纯手术治疗3例,单纯糖皮质激素治疗1例,手术+糖皮质激素治疗1例,糖皮质激素+肿瘤化疗治疗2例,手术+糖皮质激素+肿瘤化疗治疗3例,患者临床症状均无明显改善。结论副肿瘤性脊髓病罕见,多表现为慢性进行性脊髓病且多先于肿瘤出现;多数患者脑脊液表现异常,影像学可表现为脊髓长节段病灶。该病治疗上以肿瘤治疗为主,免疫治疗为辅。     

Intracranial hypertension as an initial manifestation of spinal neuroectodermal tumor

A 19-year-old girl had headaches, blurred vision and vomiting for 2 weeks. Neurological examination revealed only bilateral papilloedema and left abducens palsy. Neuroimaging of the brain was normal. Cerebrospinal fluid study showed intracranial hypertension (IH), hypoglycorrhachia, hyperproteinorrhachia, and a negative cytology study. Eight months after the onset, paraparesis occurred. Spinal magnetic resonance imaging showed intramedullary masses at the cervical and thoracic cords with extensive seeding. Biopsy of the mass showed primitive neuroectodermal tumor (PNET). IH rarely occurs in patients with spinal cord neoplasms. Its incidence is low and the condition is always associated with signs of myelopathy. We report a patient whose initial manifestation of spinal PNET was IH only. Spinal tumor should be considered in IH patients whose intracranial examinations are negative.     

多节段脊髓室管膜瘤的显微外科治疗

目的 探讨多节段脊髓室管膜瘤的手术技术和手术治疗疗效。方法 本文回顾分析了2003年至2005年治疗的10例多节段脊髓室管膜瘤的临床资料。结果 颈髓室管膜瘤6例，胸髓室管膜瘤3例，1例术后复发肿瘤累及胸、腰髓并累及椎旁。平均累及脊髓9．4个节段。双下肢截瘫4例，高位截瘫6例，括约肌功能障碍5例，呼吸困难4例。MRI可见脊髓空洞形成8例。手术采用椎板复位9例，C-D钉棒系统脊柱后路内固定1例。术后患者肌力恢复7例，无改变3例。呼吸困难，应用呼吸机辅助呼吸1例。术后复查MRI无肿瘤残留9例，1例部分椎旁肿瘤残留。结论 采用显微神经外科技术可以达到镜下多节段室管膜瘤全切，患者术后症状大多数可以明显改善。多节段椎板切开术后应考虑椎板复位或脊柱内固定，以保持脊柱的稳定性。     

Inflammatory myelopathy presenting as a cystic intramedullary spinal cord lesion

A case of subacute progressive spinal tetraparesis had myelographic evidence of cervical spinal cord swelling and a delayed metrizamide computed tomographic myelogram (MCTM) suggested cavitation within the swollen spinal cord. Surgical exploration of the cervical cord revealed inflammatory changes only. No syrinx or intramedullary tumour was found. The accumulation of metrizamide within the spinal cord, as demonstrated by MCTM, did not represent a syrinx or a cystic tumour, but more likely an area of inflammation. Because inflammatory myelopathy may simulate an intramedullary tumor or syrinx, careful analysis of all clinical and radiological information is necessary to help make a correct diagnosis.     

Spinal cord sarcoidosis: a diagnostic dilemma.

Sarcoidosis is a chronic disorder of unknown etiology characterized by the development of non-caseating granulomas with derangement of the normal tissue architecture. Compromise of the spinal cord is one of the rarest neurologic manifestations of the disease, which may be clinically and radiologically indistinguishable from a spinal cord malignant tumor. However, neurosarcoidosis can be treated with steroids. This study reviews the clinical, radiological, and pathological features of the sarcoid compromise of the spinal cord, emphasizing the difficulties commonly encountered in making a diagnosis.     

Primary intramedullary primitive neuroectodermal tumor (PNET) – case report and review of the literature

Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.     

显微手术治疗颈段髓内肿瘤10例

目的:报道应用显微外科手术切除颈段髓内肿瘤的临床疗效。方法:对10例颈段髓内肿瘤施行显微手术治疗。采用McCormick临床神经功能分级方法及MRI检查,对患者术前、术后神经功能改变,以及肿瘤切除情况进行评估分析。结果:肿瘤全切除7例,次全切除2例,部分切除1例。术后临床神经功能改善6例,保留术前神经功能3例,术后神经功能变差1例;术后无死亡病例及手术致残者。结论:显微外科能提高手术切除颈段髓内肿瘤的临床疗效,改善患者症状。     

脊髓海绵状血管瘤

目的 研究分析脊髓海绵状血管瘤(CA)的临床和放射学特点及治疗效果。方法 回顾分析19年间收治的19例脊髓CA的临床资料。结果 平均病程4.2年,临床主要表现为脊髓压迫症。MR T_1W可见低、高和混杂信号,T_2W高信号多见,个别瘤周可见低信号环。19例脊髓CA全切15例,次全切或部分全切3例,仅作活检1例。17例平均随访5.7年,临床改善及稳定者15例。结论 MR是诊断脊髓CA的敏感方法。手术治疗效果较好,影响病人预后的最重要因素是术前功能状况及瘫痪持续时间。     

Posterior cervical decompression and fusion for circumferential spondylotic cervical stenosis: review of 50 consecutive cases.

PURPOSE OF STUDY: This study presents a clinical and radiological evaluation of 50 consecutive patients with symptomatic spondylotic cervical myelopathy and circumferential spinal cord compression who were managed with a single stage wide posterior laminectomy and lateral mass instrumented fusion. METHODS USED: 50 consecutive patients (33 male, 17 female) over a 4 year period presenting with symptomatic cervical myelopathy due to circumferential cervical spondylotic spinal stenosis were evaluated and operated upon by a single surgeon and followed in a prospective fashion. All patients underwent pre- and postoperative clinical, radiological and MRI evaluation. SUMMARY OF FINDINGS: No deaths occurred and no instrumentation-related neural or vascular injuries were noted. No patient required reoperation for ventral compression and in all cases CSF was visible anterior to the cord on postoperative MRI scanning, with relief of the circumferential compression. Most patients improved by at least 1 Nurick grade. Three patients (6%) had single level screw pullouts which did not affect clinical outcome, and required no intervention. Slight worsening of kyphosis occurred in 4% of cases but as group there was no measured difference in sagittal balance (P=0.10). Oswestry Neck Disability Scores improved from 25.7+/-3.6 to 16.6+/-7.1 (P<0.05). One patient required a foraminotomy/posterior discectomy 12 months postoperatively at an adjacent level. CONCLUSIONS: This study demonstrates that multisegmental spondylotic circumferential cervical stenosis causing symptomatic myelopathy can be managed by single stage decompression and fusion via a posterior approach with very low morbidity and excellent clinical and radiological outcome. The incidence of adjacent segment disease is lower than for anterior interbody fusions with a 1%/year incidence at follow up to date.     

脊髓髓内肿瘤显微外科治疗

目的 总结并探讨脊髓髓内肿瘤显微外科手术的治疗经验。方法 统计近3年来显微外科手术治疗18例髓内肿瘤的临床资料、手术方式及术后转归。结果 手术全切17例,大部分切除1例;病理学诊断室管膜瘤8例,星形细胞瘤6例,血管母细胞瘤3例,血管畸形1例;术后运动及感觉障碍改善14例,不明显3例,加重1例;随访共16例,未见1例复发。结论 髓内肿瘤一旦诊断应早期手术治疗。脊髓髓内肿瘤应强调显微手术镜下操作,这不仅使病变与正常脊髓更易辨清,同时使操作更为细致轻柔。仔细辨认肿瘤与脊髓界限,对全切肿瘤和保护脊髓是有帮助的。对于肿瘤与脊髓有较清楚界限时应争取显微镜下全切,但当肿瘤侵蚀已至软膜下区时,此时不能盲目追求全切,以避免永久性功能障碍。     

显微手术切除加椎管重建治疗髓内室管膜瘤七例

目的探讨经显微手术切除加椎管重建治疗髓内室管膜瘤的临床疗效和预后。方法总结我科7例采用显微镜下切除肿瘤,复位椎板复合体行椎管重建治疗髓内室管膜瘤临床资料。结果 6例肿瘤手术完全切除,症状好转4例,随访未见复发及脊柱生理弯曲变形。结论微手术切除加椎管重建治疗髓内室管膜瘤效果令人满意,且能够防止脊柱生理弯曲变形。     

Lumbosacral dysraphia and "long spinal cord" of delayed appearance (9 cases)

Urinary disorders, deformities of the feet, and sensory of trophic disturbances appearing during, or increasing in severity at adolescence, were observed in 9 patients. In seven cases there appeared to be an abnormality of the teguments of the lumbosacral region, and in all cases, as shown by straight radiological examination of a spina bifida of L5 or S1, a sacral dehiscence and wide sacral canal, and a subjacent nervous tissue abnormality was suspected. Radiculography showed the presence of a "long spinal cord" terminating in the sacrum by a tumor mass. Examination of this mass in the seven operated cases demonstrated that the tumor was an intradural lipoma with a wedge-shaped penetration into the end of the medulla, fixing the cord in the sacrococcygial region, in the absence of the filum terminale and cauda equina. The etiology of this "long spinal cord" is discussed, and it appears to be the consequence of a dysraphia, which is also the cause of the lipoma. Mechanical factors appear later and produce neurological signs.