Malignant spinal cord compression in cancer patients may be mimicked by a primary spinal cord tumour

Although it is quite rare, second primary neoplasms in cancer patients may present with the signs and symptoms of malignant spinal cord compression. Primary spinal cord tumours in the cancer patients may be deceptive and considered as the recurrent first cancer. Therefore, it should be precisely differentiated and appropriately managed. We report such a case of intramedullary ependymoma of the cervical spinal cord mimicking metatstatic recurrent lymphoma and causing cord compression. A 50-year-old man developed intramedullary ependymoma of the cervical spinal cord 1.5 years following chemoradiation for Waldeyer's ring lymphoma. He presented with a 2-month history of neck pain, progressive upper- and lower-extremity numbness and weakness, and bowel and bladder dysfunction. Magnetic resonance imaging revealed an intramedullary expansive lesion extending from C4 to C6 levels of the cervical spinal cord. The clinical and radiological findings were suggestive of malignant process. A comprehensive investigation failed to detect another site of disease. He underwent operation, and the tumour was subtotally resected. The patient's neurological deficits improved subsequently. The development of the intramedullary ependymoma following treating lymphoma has not been reported. We describe the clinical, radiological and pathological findings of this case and review the literature.     

伸长细胞型室管膜瘤1例报告

通过分析吉林大学第一医院1例确诊为伸长细胞型室管膜瘤患者的临床资料,整理近期国内外关于伸长细胞型室管膜瘤的相关文献。我们可以得出伸长细胞型室管膜瘤十分罕见,多来源于脑室壁和脊髓中央管表面室管膜瘤细胞,缺乏特征性影像学表现,以压迫而不是侵犯脑和脊髓组织为主要临床表现,室管膜瘤的治疗方案是手术中容易达到的部位全切肿瘤结合术后放疗治疗。伸长细胞型室管膜瘤手术后预后尚需进一步观察。     

Late Dissemination of Fourth Ventricle Ependymoma: A Case Report

Dissemination of benign ependymoma is relatively rare, especially when a local tumor is under control. The majority of the failures take place within a few years after surgery. However, we experienced a case in which multiple spinal cord dissemination was found 13 years after resection of the fourth ventricle ependymoma without a local failure. A 38-year-old man had undergone a subtotal resection of the fourth ventricle ependymoma and radiation therapy to the posterior fossa when he was 25 years old. Follow-up MR imaging repeated once every one or two years detected no recurrence until he began to complain of lumbago and numbness of the right foot 13 years after the surgery. MR imaging revealed multiple nodules along the whole spinal cord. Examination of the cerebrospinal fluid detected tumor cells with ependymal cell features. He underwent radiation therapy to the whole spine, and remained stable at 18 months after the therapy. This case alerts us to the necessity for long-term radiological follow-up including the spinal cord even in benign ependymomas, although it is still not certain for how long and how often we should do it.     

髓内星形细胞瘤与室管膜瘤的MRI诊断与鉴别诊断

目的:探讨髓内星形细胞瘤与室管膜瘤的MRI表现及鉴别诊断要点。方法:回顾性分析12例髓内星形细胞瘤与室管膜瘤患者的临床资料和MRI资料,并总结其MRI特征。结果:12例患者中,星形细胞瘤7例,室管膜瘤5例。7例星形细胞瘤中,4例位于颈段脊髓,3例位于胸段脊髓,MRI表现为浸润性生长,范围广泛,增强扫描呈不均匀强化,边界不清,趋向于散在强化。5例室管膜瘤中,3例位于颈段脊髓,2例位于圆锥和终丝段,多呈膨胀性生长,可占据整个脊髓断面,增强扫描呈较均匀性强化,边界可相对清楚,多伴有脊髓空洞和囊变,出血常见。结论:髓内室管膜瘤手术可完全切除,预后较好;星形细胞瘤较难完全切除,预后相对较差,MRI可提供对于二者的鉴别依据,具有一定的临床意义。     

Improved localization of infratentorial ependymoma by magnetic resonance imaging: Implications for radiation treatment planning

Ependymomas of the posterior fossa extend into the upper cervical spinal cord in approximately one-third of cases. Unfortunately, the posterior fossa and upper cervical cord region is often poorly seen on computed tomography (CT), making radiotherapy planning difficult. We report five cases of posterior fossa ependymoma with extension into the cervical cord where magnetic resonance imaging (MRI) demonstrated the caudal extent of tumor more clearly than CT. The extent of tumor depicted by MRI in each case correlated well with the operative findings. Higher doses of radiotherapy to the entire tumor volume are associated with improved survival in infratentorial ependymoma; however, the radiation tolerance of the cervical spinal cord is close to the dose necessary to control posterior fossa ependymoma, making limitation of radiation field volumes important. MRI may provide a method of precisely defining caudal tumor extent of posterior fossa ependymomas so that limited volume, high dose radiotherapy can be more safely administered to these patients.     

Primary myxopapillary ependymoma of the fourth ventricle with cartilaginous metaplasia: a case report and review of the literature

Myxopapillary ependymoma (MPE), which is a benign histological subtype of ependymoma, is found predominantly in the cauda equina region. It occurs rarely in the brain and mostly as a metastatic deposit from a spinal lesion. The occurrence of primary intracranial MPE is exceptional, with only 11 cases reported to date. We report an additional case of intracranial MPE, which is the third reported case in the fourth ventricle. The tumor manifested in a 50-year-old lady, who presented with features of raised intracranial pressure. A gross total resection of the tumor was achieved. Histologically, the tumor had characteristic features of MPE with focal metaplastic cartilaginous deposit. On further evaluation, there was no evidence of a primary tumor in the spinal cord. Intracranial MPE needs further evaluation by craniospinal MRI to exclude an unrecognized primary in the spinal region, which could warrant surgical attention.     

MR imaging features that distinguish spinal cavernous angioma from hemorrhagic ependymoma and serial MRI changes in cavernous angioma

Cavernous angiomas of the spinal cord exhibit imaging characteristics that may overlap with those of hemorrhagic ependymoma. In the present study, we aimed to identify specific magnetic resonance imaging (MRI) findings that could be used to differentiate cavernous angioma from hemorrhagic ependymoma, and to evaluate serial MRI changes in cases of cavernous angioma. We retrospectively evaluated MR images of spinal cord tumors collected at our hospital from 2007 to 2015. From this cohort of images, 11 pathologically confirmed cavernous angiomas and 14 pathologically confirmed hemorrhagic ependymomas were compared with respect to the size of the tumor, longitudinal location, axial location, enhancement pattern, syrinx, edema, tumor margin, signal intensity of T2WI, signal intensity of T1WI, and longitudinal spreading of the hemorrhage. Serial MR images of seven spinal cavernous angiomas were reviewed. Small size, eccentric axial location, minimal enhancement, and absence of edema were more frequently observed on images of cavernous angioma compared to those of hemorrhagic ependymoma (p?<?0.01). Serial MRI changes in cases of cavernous angioma included increased longitudinal spreading of the hemorrhage (6/7, 86?%) and emergence of high signal intensity on T1WI (1/7, 14?%). Small size, eccentric axial location, minimal enhancement, and absence of edema are significant MRI findings that may be used to distinguish Type I and Type II spinal cavernous angiomas from hemorrhagic ependymomas. Furthermore, longitudinal spreading of the hemorrhage may be observed on follow-up MRIs of cavernous angiomas.     

Spinal cord ganglioglioma presenting as hydrocephalus

Summary We report a case of a 5 year old boy with a spinal cord ganglioglioma and hydrocephalus. The insidious onset with back stiffness and hamstring tightness, the diagnosis by magnetic resonance imaging, and the treatment by total excision is described. An adequate proven explanation for the hydrocephalus is lacking. Mechanisms which have been proposed include impaired cerebrospinal fluid absorption from increased cerebrospinal fluid protein, and arachnoiditis.     

Parenchymal anaplastic ependymoma with intratumoral hemorrhage: a case report

We report an unusual case of a 56-year-old woman with a supratentorial anaplastic ependymoma localized in the parenchyma without continuity with the ventricular system and brain surface. The patient presented with vertigo, and a calcified mass was detected in the left temporal parenchyma. Five years later, she had seizure of the right extremities. Computed tomographic scanning and magnetic resonance imaging revealed an enhanced mass with an intratumoral hemorrhage adjacent to the calcified mass. Subtotal removal of the tumor was performed. The histological analysis revealed that the tumor was an anaplastic ependymoma. After focal radiation therapy (50 Gy), the outcome was favorable, although the residual lesion was still seen on the images. Ependymomas usually arise from the cells lining the ventricular system and the central canal of the spinal cord. We discuss the summary of published cases of supratentorial ectopic ependymoma since the first case in 1995.     

Spinal cord anaplastic oligodendroglioma with 1p deletion: report of a relapsing case treated with temozolomide

Primary spinal cord oligodendroglial tumor is very rare, and in only one patient with spinal cord oligodendroglioma has a deletion of chromosome 1p/19q been reported. We present the case of an 18-year-old girl, who had one-year lower back pain and one-month lower limb weakness. Magnetic resonance images of the spinal cord showed an intramedullary mass from level T8 to T10, which was then radically removed. Histology revealed an anaplastic oligodendroglioma. The patient was treated with radiotherapy postoperatively. Eight months after the treatment, follow-up magnetic resonance images disclosed an enhancing intramedullary mass at level T4–T8; recurrence of the tumor was therefore diagnosed. Maximum surgical removal of the recurrent tumor was performed, diagnosis of anaplastic oligodendroglioma was made, and a chromosome 1p deletion was determined by FISH. After treatment with temozolomide for six months, the patient had a remarkable improvement of her lower limb symptoms, and complete imaging regression of the residual tumor showed no evidence of recurrence at any other sites. The most recent MRI of brain and spinal cord showed postoperative changes without evidence of tumor recurrence of the spine and oligodendrogliomatosis along the cerebral–spinal axis. To our knowledge, this is the first report of a recurrent anaplastic oligodendroglioma with 1p deletion occurring in the spinal cord. It is also the first case of the patient with recurrent intramedullary anaplastic oligodendroglioma who had a significant clinical improvement and complete imaging remission after subtotal resection then treatment with temozolomide chemotherapy.     

脊髓血管母细胞瘤的MR诊断

目的：探讨脊髓血管母细胞瘤的MRI表现及其鉴别诊断。方法：回顾性分析12例经手术和病理证实的脊髓血管母细胞瘤患者的临床和MRI资料。所有病例均行MRI平扫及增强扫描。结果：本组12例患者均经手术及病理证实。8例发生在颈段脊髓,3例发生于颈胸段脊髓,1例发生于胸段脊髓。其中1例呈多发,同时累及小脑半球和颈髓。肿瘤实质最大1.5cm,最小3mm,3例为局部囊变,9例为邻近脊髓空洞积水,2例伴有明显的水肿。累及范围为1个椎体区段至10个椎体区段。T1WI显示病变囊影呈脑脊液样低信号,结节与脊髓相比,呈等或略低信号。其中9例结节位于脊髓背侧,3例位于脊髓中央区域。9例囊肿及空洞积水周围的脊髓未见明显水肿存在。3例邻近脊髓水肿呈T1 W略低信号,T2 W呈较高信号。1例同时伴有小脑血管母细胞瘤。T2 WI囊性病变呈脑脊液样高信号,结节呈略高信号,囊肿周围水肿区呈较高信号,其中1例结节内见血管流空信号。T1WI增强扫描12例结节病灶及伴有小脑病灶的均显著增强。结论：脊髓血管母细胞瘤在MR图像上具有一定的形态及信号特点,对于术前正确诊断具有一定意义。     

Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy

Purpose: To evaluate the effectiveness of complete resection and postoperative radiotherapy in spinal cord ependymomas. Methods and materials: We conducted a retrospective study over 20 patients (13 males and 7 females) with histologically confirmed spinal cord ependymomas between July 1985 and April 2001. Among them, 13 patients had ependymomas, 6 had myxopapillary ependymomas, and 1 had anaplastic ependymoma. All patients received radical surgery for tumor removal with 13 patients achieving complete resection and 7 incomplete resection due to technical difficulty. Among those with incomplete resection, 6 patients received postoperative radiotherapy to tumor bed and only one patient with anaplastic ependymoma received surgery alone. The total tumor dose ranged from 50 to 60 Gy. Results: Among the 20 patients, 19 patients were alive and showed local control. The median survival time of all patients was 109 months, with 104 months in the complete resection alone group and 135 months in the incomplete resection with postoperative radiotherapy group. One patient with anaplastic ependymoma and no postoperative radiotherapy developed leptomeningeal seeding 9 months after surgery. Salvage therapy of radiotherapy and chemotherapy maintained normal neurological functions. The patient expired 34 months from the initial diagnosis due to progression of leptomeningeal seeding. Conclusion: Complete resection alone in spinal cord ependymoma can achieve excellent local control and survival. Patients should receive complete resection if technically possible. Postoperative radiotherapy is not recommended for complete resection. For incomplete resection, postoperative local radiotherapy is recommended and it can also achieve excellent local control and survival. Local radiotherapy with 50-60 Gy is effective and safe. Salvage radiotherapy improves quality of life for local recurrence or leptomeningeal seeding patients.     

Lhermitte's sign as a presenting symptom of primary spinal cord tumor

Summary We describe a previously healthy 29 year-old man who developed Lhermitte's sign, a shock-like or electric sensation, transmitted down the spine, which occurred during neck flexion or rotation. Evaluation demonstrated an intrinsic, fusiform mass extending from c₅ to c₇. At operation, the mass was completely removed and found to be a low-grade ependymoma. The sensory phenomena of Lhermitte's sign were most likelycaused by tumor-induced distortion and demyelination of cervical dorsal column sensory axons. Lhermitte's sign is most prevalent in patients with multiple sclerosis, cervical spondylotic myelopathy, cisplatin neurotoxicity, cervical radiation injury, and neck trauma. Rarely, Lhermitte's sign occurs with spinal cord compression from epidural or subdural tumor. This patient is the first reported case of an intrinsic spinal cord tumor to present with Lhermitte's sign.     

Long-term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: a retrospective multicenter study by the Korea Spinal Oncology Research Group

Background

We sought to determine the surgical treatment and functional outcome and identify the predictors of survival in a retrospective cohort of patients with spinal cord ependymoma using data collected from the Korea Spinal Oncology Research Group database.

Methods

The data regarding 88 patients who had been surgically treated for histologically confirmed spinal cord intramedullary and extramedullary ependymoma from January 1989 to December 2009 were retrospectively reviewed.

Results

Histopathological examination revealed myxopapillary ependymoma in 24 patients, ependymoma in 61 patients, and anaplastic ependymoma in 3 patients. Gross total removal was achieved in 72 patients, subtotal removal in 15 patients, and partial removal in 1 patient. Twenty patients were treated with postoperative radiation. Fifty-two patients had stable or improved postoperative neurological function, while 36 experienced neurological deterioration. A permanent decrease in McCormick classification grade was seen in 17 patients. The progression-free survival rate was 87% for all patients at 5 years and 80% at 10 years. During follow-up, local recurrence/progression was seen in 13 patients. Diffuse meningeal spread developed in 2 anaplastic ependymoma patients. Postoperative radiotherapy after incomplete resection did not significantly correlate with longer times to recurrence. Multivariate analysis revealed histology and surgical extent of resection as independent predictors of longer progression-free survival.

Conclusions

Gross total removal alone is a good treatment strategy for spinal ependymomas. Early diagnosis and surgery, before severe paralysis, are important to obtain good functional outcomes. Subtotal resection with radiation therapy for intramedullary lesions appears to offer no advantages over gross total removal.     

脊髓髓内室管膜瘤的显微外科治疗——附173例临床总结

背景与目的：随着神经影像技术，显微外科技术和术中监测手段的发展，大多数脊髓髓内肿瘤的早期诊断和治疗已成为现实。本研究旨在探讨显微外科手术治疗脊髓室管膜瘤的经验。方法：回顾性研究北京天坛医院神经外科脊柱脊髓组从2000年1月至2005年4月经显微外科手术治疗的髓内室管膜瘤173例，并分析其预后影响因素。结果：肿瘤全切除163例（94．2％），近全切除8例（4．6％），大部分切除2例（1．2％），出院3-6个月门诊随访好转142例（76．3％），余随访不全。结论：积极的显微外科治疗，争取全切是髓内室管膜瘤治疗的最佳选择，术前神经功能状况、肿瘤的性质与部位、手术技巧与切除程度等都是影响预后的重要因素。     

经小脑延髓裂治疗四脑室室管膜瘤

目的：总结第四脑室室管膜瘤的显微外科治疗经验。方法：回顾性分析2000年1月至2010年1月我院收治的50例第四脑室室管膜瘤患者的临床资料。结果：肿瘤全切41例,次全切除5例,大部切除4例,无手术死亡病例,术后发生并发症4例：小脑缄默症2例,脑积水1例,脑神经功能障碍1例。术后随访平均6.5年,其中恢复良好42例,改善5例,恶化3例。结论：应用显微外科技术行经小脑延髓裂入路治疗第四脑室肿瘤,手术成功率高。熟悉第四脑室的显微解剖,注意保护脑干功能是手术成功的关键。     

Spinal Cord and Intradural-Extraparenchymal Spinal Tumors: Current Best Care Practices and Strategies

The management of patients with intradural spinal tumors differs in many respects from approaches taken for patients with intracranial tumors. Intramedullary lesions are often completely surrounded by normal spinal cord, displacing vital functional tracts eccentrically. Extramedullary lesions can drastically compress the spinal cord and nerve roots, reducing normal tissue to a ribbon-like consistency. The small amount of normal tissue relative to tumor has implications for surgery and postoperative adjuvant therapy. In addition, operative intervention must take spinal stability into consideration. In this report, we describe the current best care practices and strategies for patients with a diagnosis of spinal astrocytoma, ependymoma, hemangioblastoma, schwannoma, and meningioma. Treatment of patients with intradural tumors of the spinal cord and adjoining structures has changed over the past 20 years. Advances in many disciplines including neuroradiology, neurosurgery, neurooncology, and neuropathology have contributed to expediting diagnosis and improving outcomes.     

Platelet-derived growth factor receptor (PDGFR) expression in primary spinal cord gliomas

Abnormal signaling through the platelet-derived growth factor receptor (PDGFR) has been proposed as a possible mechanism of spinal cord glioma initiation and progression. However, the extent of PDGFR expression in human spinal cord gliomas remains unknown. In this study we perform immunohistochemical analysis of PDGFRα expression in a series of 33 primary intramedullary spinal cord gliomas of different types and grades. PDGFRα was seen to be expressed in a significant subset of these tumors across all major glioma types including ependymoma, oligodendroglioma, pilocytic astrocytoma, astrocytoma, and glioblastoma. These results support the hypothesis that growth factor signaling through the PDGFR may be important for the development of at least a subset of human spinal cord gliomas. Further studies investigating the prognostic significance of PDGFR expression as well as the role of PDGF signaling on the development of intramedullary spinal cord gliomas are warranted.     

Tanycytic ependymoma of the spinal cord with anaplastic cytological features

In a 43-year-old man, an intramedullary spinal cord tumor spreading from the level of the T2 to T5 vertebrae was subtotally resected. The tumor predominantly consisted of a fascicular proliferation of spindle cells having bland nuclei and bipolar, long cytoplasmic processes, and a few perivascular pseudo-rosettes were found. Although there were no true ependymal rosettes, intracytoplasmic dot-like immunoreactivity for epithelial membrane antigen (EMA) was found in a few cells. In some areas, a dense and diffuse proliferation of anaplastic, short-spindled cells having hyperchromatic nuclei and scant cytoplasm was noted, and the Ki-67 labeling index was remarkably higher (18.2%) in these areas. Neither microvascular proliferation nor necrosis was observed. In the boundary region, these two areas showed gradual transition from one to the other. The patient has remained free from recurrence for 10 months postoperatively. This is the first documentation of tanycytic ependymoma in which tumor cells showed anaplastic cytological features.     

Perineural Spread of Malignant Mesothelioma Resulting in an Intradural Spinal Cord Mass: Case Report

Spinal cord involvement by perineural spread of malignant mesothelioma is rare. We report a case of malignant mesothelioma that spread locally to invade the bony spine with both extradural and intradural perineural spread into the spinal canal that resulted in spinal cord compression. A 61-year-old man with a history of malignant mesothelioma presented with progressive leg weakness and right-sided arm weakness. Magnetic resonance imaging showed an enhancing lesion in the apex of the right lung with extension through the C7–T1 foramina with right hemicord enhancement. The patient underwent a C7–T1 laminectomy and right-sided C7–T1 and T1–T2 foraminotomies for neural decompression and biopsy of the lesion. Intraoperatively, tumor extended epidurally, and intradural perineural tumor spread along the C8 and T1 nerve roots into the spinal cord. Because it adhered to the spinal cord, no dissectible plane could be identified that would allow for safe total removal of the tumor. The epidural portion of the tumor, the adjacent involved bone, and the T1 nerve root were resected. Pathologic examination revealed malignant mesothelioma with bony invasion and perineural spread along the T1 nerve root. After decompression of the spinal cord, the patient had moderate improvement of his hand and leg function. Perineural spread of malignant mesothelioma resulting in spinal cord compression is an unusual clinical presentation. Intimate involvement of the spinal cord may prohibit aggressive tumor resection.