Primary Paraspinal Malignant Peripheral Nerve Sheath Tumor

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare tumors. We experienced a case of MPNST in the cervical paraspinal space which was not associated with neurofibromatosis. The tumor located in left C6-7 foramen and compressed C7 root. The tumor was removed through the occipital triangle. We report a case of the primary cerivcal MPNST in a patient who did not have neurofibromatosis-1.     

Malignant Nerve Sheath Tumor of the Spinal Accessory Nerve: A Unique Presentation of a Rare Tumor

Background

Malignant peripheral nerve sheath tumors (MPNSTs), sarcomas originating from tissues of mesenchymal origin, are rare in patients without a history of neurofibromatosis.

Case Report

We report a case of an MPNST of the spinal accessory nerve, unassociated with neurofibromatosis, which metastasized to the brain. The tumor, originating in the intrasternomastoid segment of the spinal accessory nerve, was removed. Two years later, the patient presented with focal neurological deficits. Radiographic findings revealed a well-defined 2.2×2.2×2.2 cm, homogeneously enhancing mass in the left parieto-occipital region of the brain surrounded by significant vasogenic edema and mass effect, culminating in a 1-cm midline shift to the right. The mass was surgically removed. The patient had nearly complete recovery of vision, speech, and memory.

Conclusions

To our knowledge, this is the first documented case of an MPNST arising from an extracranial segment of the spinal accessory nerve and metastasizing to the brain.     

The Early Detection of Recurrence of Malignant Peripheral Nerve Sheath Tumor by Frequent Magnetic Resonance Imaging

Surgery has a key role in the treatment of malignant peripheral nerve sheath tumors (MPNSTs), but the resectability of paraspinal MPNSTs is only 20%. Therefore, spinal MPNSTs show frequent recurrence and poor prognosis. Local recurrence is much more common than metastasis for MPNSTs, and surgery still has a key role in the treatment of local recurrence. Therefore, it is important that recurrence must be detected before resectability is lost. However, no evidence-based follow-up protocol has been established for MPNST. The authors performed gross total resection in a 34-year-old woman presented with thoracic MPNST. Adjuvant radiotherapy and chemotherapy were not administered since these adjuvant therapies generally do not improve survival in MPNST and may cause additional neurovascular damage. Instead, the authors monitored the primary site every 3 months using magnetic resonance imaging to detect local recurrence at the earliest opportunity. The tumor recurred locally on two occasions without overt symptoms at 21 and 24 months postoperatively. These recurrences were treated successfully by gross total removal.     

Malignant peripheral nerve sheath tumor of the cauda equina with craniospinal metastasis

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.     

Malignant peripheral nerve sheath tumor of the cauda equina

Only one case of malignant peripheral nerve sheath tumor (MPNST) affecting the cauda equina region has been reported earlier. A 32-year-old male with congenital multiple subcutaneous swellings presented with low back pain, progressive paraparesis and bladder-bowel dysfunction. Magnetic resonance imaging (MRI) demonstrated a heterogenously enhancing intradural lesion at L2–L4. At operation, on opening the dura, multiple nodular, firm matted masses attached to the lumbosacral nerve roots were encountered. Peripheral lesions were partially excised. Histopathological exam revealed varied cellularity with necrosis and pleomorphic nuclei suggestive of MPNST. MRI features, pathophysiological characteristics and the literature are reviewed. Received: 2 February 2001 / Accepted in revised form: 13 June 2001     

Malignant intracerebral giant nerve sheath tumor in a 14-month-old girl with neurofibromatosis type 1: a case report

Introduction  Malignant intracerebral nerve sheath tumor (MINST) is extremely rare and the origin is still unclear. The authors present the clinical, radiological, and pathological features of a malignant intracerebral giant nerve sheath tumor. Case report  A giant tumor in the right frontotemporoparietal lobes causing a midline shift was detected in a 14-month-old girl who presented with developmental delay, vomiting, and lethargy. The physical examination was consistent with neurofibromatosis type 1 (NF-1). Subtotal resection was performed and the histopathological examination revealed the diagnosis of MINST. Discussion  There are only six cases of malignant intracerebral nerve sheath tumor in the literature. The presented case is the youngest and the occurrence of MINST in a 14-month-old girl may support the hypothesis of multipotent mesenchymal stem cell origin; however, the tumors which arise from multipotent mesenchymal stem cells may be seen in later stages of life. Another important feature of the presented case is the occurrence of MINST in NF-1. Conclusion  MINSTs are extremely rare tumors with unknown origin. The location, the degree, and the size of the tumor and the general condition of the patient are prognostic factors in MINSTs, like in other malignant tumors.     

超声评估中国健康成年人视神经鞘直径超声评估中国健康成年人视神经鞘直径

目的 确定中国正常成人视神经鞘直径（optic nerve sheath diameter,ONSD）的参考值范围,并且探索
与其相关的影响因素。
方法 以2013年1月-2014年1月健康体检的成年人作为研究对象,每位受试者的双眼视神经鞘的矢
状位和横断面均由两位医生分别测量两次。每位受试者ONSD的最终报告值是双眼视神经鞘的16个
测量值的平均值。
结果 共有230例受试者,获得3680个ONSD测量值,范围为2.65～4.30 mm。ONSD的上限值低于以往
白种人及黑人的研究结果。简单线性回归分析发现ONSD与性别、体质指数（body mass index,BMI）、
腰围、头围相关。在调整了其他可能的影响因素后发现性别（偏回归系数0.189,P<0.001）、BM（I 偏回
归系数0.032,P <0.001）是ONSD的独立影响因素。低体重组的女性ONSD最小。
结论 在确立ONSD的正常值标准时应考虑种族、性别、BMI的差别。     

Nerve ultrasound in neurofibromatosis type 1: A follow-up study

Objective

To investigate development of sonographic abnormalities and applications of high-resolution ultrasonography (HRUS) in neurofibromatosis type 1 (NF1).

超声评估中国健康成年人视神经鞘直径

目的 确定中国正常成人视神经鞘直径（optic nerve sheath diameter,ONSD）的参考值范围,并且探索 与其相关的影响因素。 方法 以2013年1月-2014年1月健康体检的成年人作为研究对象,每位受试者的双眼视神经鞘的矢 状位和横断面均由两位医生分别测量两次。每位受试者ONSD的最终报告值是双眼视神经鞘的16个 测量值的平均值。 结果 共有230例受试者,获得3680个ONSD测量值,范围为2.65～4.30 mm。ONSD的上限值低于以往 白种人及黑人的研究结果。简单线性回归分析发现ONSD与性别、体质指数（body mass index,BMI）、 腰围、头围相关。在调整了其他可能的影响因素后发现性别（偏回归系数0.189,P<0.001）、BM（I 偏回 归系数0.032,P <0.001）是ONSD的独立影响因素。低体重组的女性ONSD最小。 结论 在确立ONSD的正常值标准时应考虑种族、性别、BMI的差别。     

颅内恶性神经鞘膜瘤临床诊断及治疗

目的通过分析颅内恶性神经鞘膜肿瘤这一罕见肿瘤的临床资料，加深对这一疾病的认识．以期提高治疗效果。方法回顾性分析我院1994年1月-2006年11月手术切除并经病理证实的11例颅内恶性神经鞘膜肿瘤患者的临床资料。结果本组病例年龄为3～51岁，男女病例比例为7：4；多数有颅神经障碍，局部肿块以及原有症状再度出现是本组病人的主要临床表现；这些病变起源可分为：（1）直接起源于颅神经根的；（2）非明确神经根起源的；（3）复发并恶变的。肿瘤全切除5例．次全切除3例，部分切除3例。术后近期效果较好，3例辅以放疗。术后3例存活超过5年，存活最长的病例超过10年。结论颅内恶性神经鞘膜肿瘤多见于儿童及中年患者，多数病人病程较短．临床症状因部位不同表现相应神经功能障碍，术后近期效果较好，肿瘤全切除及放疗可提高5年生存率。     

中国高颅压患者视神经鞘直径的诊断价值

目的 确定视神经鞘直径（optic n erve s heath d iameter,ONSD）评估颅内压（intracranial p ressure,ICP) 增高的诊断标准并研究其相关影响因素。 方法 本研究为双盲横断面研究,收集2013年3月-12月可疑高ICP需要进行腰椎穿刺测量脑脊液压力 的患者为研究对象,记录患者的个体相关信息及脑脊液压力值,分为ICP正常组及增高组,比较两组 患者相关因素的差异。运用接受者操作特性曲线（receiver operating characteristic,ROC）确定高ICP的 ONSD最佳诊断值。 结果 共收录了279例患者,其中包括101例高ICP患者。比较显示,ONSD是ICP的独立预测因素（P ＜0.001）,不受性别、年龄、体质指数（body mass index,BMI）、头围、腰围、高血压和病因分型的影响。 ONSD能够有效准确地评估高ICP。运用超声检测ONSD诊断高ICP的最佳临界值是4.1 mm（敏感度95%, 特异度92%）。 结论 我国高ICP患者的ONSD的诊断值比白种人低,因此建议检测ONSD评估高ICP时应该注意种族 差异。     

Clinical features and post-surgical outcome of patients with astroblastoma

Astroblastoma is a rare tumor, and thus experience with these lesions is very limited. The prognosis and appropriate treatment is not well understood, as few individual centers have enough experience with astroblastoma to guide treatment recommendations. We performed a systematic comprehensive search of the published English language literature on patients undergoing surgery for astroblastoma to summarize what is known about these tumors, and to provide some framework for future efforts in this area. A total of 62 references met our inclusion criteria, and contained individual patient data on 116 patients with astroblastoma. Determination of overall survival rates was performed using Kaplan-Meier analysis. This analysis suggests that the distribution is bimodal, with a prominent peak in young adulthood. Astroblastomas are generally amenable to complete tumor resection, even when very large, with gross total resection (GTR) achieved in 71/85 (84%) of reported patients, including both 9 cm tumors reported. Patients undergoing GTR experienced a significant improvement in survival compared to patients who underwent subtotal resection (STR) (5-year progression-free survival: GTR 83% versus [vs.] STR 55%, log rank p = 0.011). Although patients receiving external beam radiotherapy or fractionated three-dimensional conformal radiotherapy (XRT) seemed to have lower survival rates, this was not statistically significant (5-year survival: GTR 94% vs. GTR + XRT 73%, log rank p = 0.463). Thus, we have reported the results of a summary of the literature on astroblastomas and have accurately described outcome characteristics using a data set that would be difficult to accumulate at a single center treating this tumor.     

替莫唑胺应用于恶性脑胶质瘤的研究进展

脑胶质瘤是临床最常见的肿瘤之一,约占颅内肿瘤发病率的40％～50％,由于胶质瘤呈恶性浸润性生长,且多生长在脑重要结构,如基底节、中央沟区、丘脑、脑干等部位,不仅手术难以全切,而且术后易复发。长期以来,单纯手术治疗恶性脑胶质瘤的5年存活率〈25％。但术后合理的放疗、化疗和其他综合治疗可以控制肿瘤生长和延缓复发,使一些病人病情得到较好的改善。化疗作为颅内恶性胶质瘤综合治疗手段之一,已为广大临床医生所接受。替莫唑胺作为一种新型的烷化剂,对恶性脑胶质瘤辅助治疗效果良好。本文就替莫唑胺应用于恶性脑胶质瘤的发展近况作一综述。