Leptomeningeal spread of intramedullary spinal cord tumors. Report of three cases

Three patients with intramedullary spinal cord tumors and secondary leptomeningeal spread of their tumors are presented. Two patients had astrocytomas and one had a ganglioglioma. Two tumors were located in the cervical spinal cord and one within the thoracic spinal cord. Review of the past and recent literature shows leptomeningeal dissemination of spinal cord tumors to be relatively rare, but it should be suspected and investigated in any patient whose condition deteriorates following removal of a spinal cord neoplasm.     

Spinal metastases of cerebral glioblastoma: the value of computed tomographic metrizamide myelography in the diagnosis

Spinal metastases from cerebral glioblastoma via the cerebrospinal pathway are rarely detected when the primary tumors are under apparent control. The authors report two adult patients with cerebral glioblastoma who developed spinal symptoms referable to spinal seeding without neurological and computed tomographic findings of the recurrence of the primary tumors. Computed tomographic metrizamide myelography clearly revealed minute deposits of perispinal metastatic tumors that could not be detected by conventional myelography. Even perispinal mass lesions so minute that they are revealed only by computed tomographic metrizamide myelography can invade the spinal cord and cause clinical symptoms.     

Intramedullary spinal cord tumors

Opinion statement The three most common types of intramedullary spinal cord tumors are low-grade astrocytomas, ependymomas, and high-grade astrocytomas. Surgical extirpation is the necessary and sufficient primary treatment for most intramedullary spinal cord tumors. Radiation therapy may also have a role in the management of persistent, recurrent, or progressive low-grade astrocytomas and ependymomas. The current treatment of spinal cord high-grade astrocytomas, which includes surgical debulking, radiation therapy, and possibly chemotherapy, is clearly inadequate. Chemotherapy may have a potential role for certain progressive spinal cord tumors, but the role is undefined at present. Recent reports have described the use of stereotactic radiosurgery for extramedullary spinal tumors, and stereotactic radiosurgery may someday be useful in the management of intramedullary spinal cord tumors. Rehabilitation programs are an important component of the multidisciplinary care of patients with spinal cord tumors. Finally, more work, especially the inclusion of adults and children with intramedullary spinal cord tumors into prospective clinical trials, is needed to improve the therapy of intramedullary spinal cord tumors and rehabilitation after diagnosis of a spinal cord tumor.     

Multiple spinal neurinomas without von Recklinghausen's disease: report of three cases

Multiple spinal tumors are relatively rare and account for from only 1.2 to 9.5% of all spinal tumors. Half of the multiple spinal tumors are accompanied with von Recklinghausen's disease. Since March 1983, we have encountered 31 spinal tumors including 11 neurinomas, three of which were multiple and showed no manifestations of von Recklinghausen's disease. The incidence of multiple spinal tumors was 9.7% in our series. Altogether, 40 cases of multiple spinal neurinomas without von Recklinghausen's disease have been reported including our three cases. According to those 40 cases, much neurinomas occur 5 times more frequently in men than in women, and they are found predominantly in the lumbosacral region. In the present paper, we describe three cases of multiple spinal neurinomas, and compare the results of several neuroradiological examinations. Myelography was the most reliable method to determine whether or not the tumors were multiple. However, in cases where myelography was completely blocked out, other neuroradiological methods such as metrizamide-CT scan or MRI with Gd-DTPA intravenous injection were able to bring us information concerning multiplicity.     

Treatment of spinal cord tumors

Opinion statement  Tumors associated with the spinal cord can have devastating effects on patient function and quality of life. Most of these tumors are from metastatic disease, usually to the epidural space. Less frequently, the tumors are intrinsic to the spinal cord itself (ie, primary tumor). Regardless of the etiology, spinal cord tumors often present first with progressive local or radicular pain, or both. Other symptoms include weakness, sensory changes, or sphincter dysfunction. The timeliness of diagnosis of spinal cord tumors and promptness of treatment are important, as they directly affect outcome. Dexamethasone, a corticosteroid, is used as a temporizing measure to improve or stabilize neurologic function until definitive treatment. For nonambulatory patients with epidural metastatic tumors, surgery followed by radiation therapy maximizes neurologic function and modestly lengthens survival. However, palliative radiotherapy alone is recommended for those with neurologic deficits lasting longer than 48 hours, survival prognosis less than 3 months, inability to tolerate surgery, multiple areas of compression, or radiosensitive tumors. An ambulatory patient with a stable spine should be considered for radiation treatment only. The role of chemotherapy for epidural metastatic tumors is not well established. For intramedullary metastases, the role of surgery and chemo therapy remains controversial and radiation is the mainstay. For low-grade or benign primary spinal cord tumors, resective surgery is of benefit and can be curative. For high-grade tumors, the benefit of resection is less clear, and radiotherapy and/or chemotherapy may be helpful. The use of chemotherapy for primary spinal cord tumors has rarely been assessed. Agents reported in the literature for treatment of spinal cord gliomas include temozolomide, irinotecan, cisplatin, and carboplatin. A multidisciplinary approach is often required to maximize the therapeutic and functional outcome of patients with metastatic and primary spinal cord tumors.     

Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2.

Benign spinal nerve sheath tumors (neurofibromas and schwannomas) often occur on dorsal nerve roots sporadically or in neurofibromatosis types 1 and 2. These are histologically benign tumors, and distinction between them is frequently not made by clinicians. To determine if there is a correlation between the histological pattern of benign spinal nerve sheath tumors and the type of neurofibromatosis, the clinical and pathological features of these tumors (86 surgical specimens and five autopsies) in 68 patients were reviewed. The patients were classified into one of four categories: neurofibromatosis type 1, neurofibromatosis type 2, uncertain, or sporadic. The diagnostic criteria used for neurofibromatosis types 1 and 2 were established by the National Institutes of Health. Patients who did not fulfill criteria for either neurofibromatosis type 1 or 2 but who had multiple nervous system tumors or other stigmata of neurofibromatosis were designated "uncertain." Spinal nerve sheath tumors were considered sporadic in 42 cases (40 schwannomas and two neurofibromas). In the 14 patients with neurofibromatosis type 1, all spinal nerve sheath tumors were neurofibromas. In six of the seven patients with neurofibromatosis type 2, all spinal nerve sheath tumors were schwannomas. One patient with neurofibromatosis type 2 had a spinal nerve sheath schwannoma and a tumor with features of both tumor types. The authors conclude that spinal nerve sheath tumors in patients with neurofibromatosis type 1 are neurofibromas. In contrast, spinal nerve sheath tumors occurring in neurofibromatosis type 2 or sporadically are most frequently schwannomas. The distinct histological features of these tumors may reflect different pathogenetic mechanisms even though they arise at identical sites in neurofibromatosis types 1 and 2.     

An Overview of Decision Making in the Management of Metastatic Spinal Tumors

IntroductionSpinal metastases are the most commonly encountered spinal tumors. With increasing life expectancy and better systemic treatment options, the incidence of patients seeking treatment for spinal metastasis is rising. Radical resections and conventional low-dose radiotherapy have given way to modern ‘separation’ surgeries and stereotactic body radiotherapy which entails lesser morbidity and improved local control. This article provides an overview of the decision making and currently available treatment options for metastatic spinal tumors.MethodsA MEDLINE literature search was made for studies in English language reporting on human subjects, describing results of various treatment options that are a part of multidisciplinary management of metastatic spinal tumors. The highest-quality evidence available in the literature was reviewed.DiscussionTreatment of patients with metastatic spinal tumors is largely palliative, with radiotherapy and selective surgery being the mainstays of management. Multidisciplinary management that incorporates factors like patient performance status, expected survival and systemic burden of disease and employs well-validated decision-making frameworks for guiding treatment holds the key to an effective palliative treatment strategy. Effective pain management, achieving local control, adequate neurological decompression in the setting of epidural cord compression and surgical stabilization for mechanical stabilization are the main goals of treatmentConclusionThe management of metastatic spinal tumors has been rapidly evolving; currently, limited decompression and stabilization followed by postoperative SBRT for local tumor control are associated with less morbidity and may be referred to as the current standard of care in these patients.     

Endoscope-assisted posterior approach for the resection of ventral intradural spinal cord tumors: report of two cases.

We report our experience with the removal of ventral intradural spinal tumors through a posterior approach with the assistance of an endoscope. The endoscope allowed us to remove the tumor with minimal retraction of the spinal cord and to inspect for involvement of structures that were difficult to visualize with the microscope. Moreover, it obviated the use of a more involved anterior approach. This is the second report of the utilization of the endoscope for removal of intradural spinal cord tumors. Our data are discussed in the context of prior reported morphometric studies involving the spinal cord and expand the indications for the endoscope.     

Lipoblastomatosis of the neck causing hemiparesis: a case report and review of the literature

BACKGROUND: Lipoblastoma and lipoblastomatosis are rare pediatric adipose tumors that sometimes affect the neck or spinal cord. This case is the third report of lipoblastoma extending into the spinal canal, the first report of intradural tumor extension, and the first report of hemiparesis resulting from lipoblastoma compressing the spinal cord. METHODS: A 13-month-old boy was seen by a pediatrician for a firm, supraclavicular neck mass on the left side. After being evaluated by CT and MRI scanning, the tumor was partially resected. RESULTS: Postoperative microscopic examination of the tumor showed adipose cells with mature nuclei and well-formed fat vacuoles interspersed with fibrovascular septa, a finding consistent with maturing lipoblastoma. CONCLUSIONS: Although lipoblastomatous tumors are treated with complete surgical resection when possible, location of these tumors in the neck may dictate partial resection to avoid intraoperative injury to the spinal cord.     

Tumors of the spine

Spine tumors comprise a small percentage of reasons for back pain and other symptoms originating in the spine.The majority of the tumors involving the spinal column are metastases of visceral organ cancers which are mostly seen in older patients.Primary musculoskeletal system sarcomas involving the spinal column are rare.Benign tumors and tumor-like lesions of the musculoskeletal system are mostly seen in young patients and often cause instability and canal compromise.Optimal diagnosis and treatment of spine tumors require a multidisciplinary approach and thorough knowledge of both spine surgery and musculoskeletal tumor surgery.Either primary or metastatic tumors involving the spine are demanding problems in terms of diagnosis and treatment.Spinal instability and neurological compromise are the main and critical problems in patients with tumors of the spinal column.In the past,only a few treatment options aiming short-term control were available for treatment of primary and metastatic spine tumors.Spine surgeons adapted their approach for spine tumors according to orthopaedic oncologic principles in the last 20 years.Advances in imaging,surgical techniques and implant technology resulted in better diagnosis and surgical treatment options,especially for primary tumors.Also,modern chemotherapy drugs and regimens with new radiotherapy and radiosurgery options caused moderate to long-term local and systemic control for even primary sarcomas involving the spinal column.     

Spinal epidural tumors without destructive bone change: clinical and radiological studies

CT in 61 patients having spinal epidural tumors were reviewed. Of these patients twelve had an epidural mass lacking of destructive bone change. Histological examination of these 12 cases revealed that usual carcinomatous tumors are relatively less common but sarcomatous tumors and malignant hematologic neoplasms are more likely to form an epidural mass without bone destruction. The tumors preponderance thoracic spine and extended usually more than three spinal levels along spinal axis. In all but one tumor masses were found in the intervertebral foramen and the paraspinal region in addition to the spinal canal. Contrast enhanced CT was particularly effective to delineate tumor location and extension. Ring enhancement of the dural sac which constricted by epidural masses was considered pathognomonic of epidural tumors.     

Two cases of von Recklinghausen's disease with multiple brain and spinal tumors]

The authors reported two cases of von Recklinghausen's disease with multiple brain tumors and multiple spinal tumors. The first case, a 21-year-old man who had a past history of optic gliomas was admitted because of gait disturbance. Computed tomography (CT) and magnetic resonance imaging (MRI) showed calcification of the basal ganglia, bilateral C-P angle tumors, cystic cerebellar tumor and arachnoid cyst in the quadrigeminal cistern. Myelography and MRI revealed multiple spinal tumors. Surgical management was performed and cerebellar tumor was histologically confirmed to be a pilocytic astrocytoma. Spinal tumors were also astrocytomas. The second patient, a 56-year-old woman suffered from right iliac pain, right hemiparesis and motor aphasia. CT revealed two round tumors in the left cerebral hemisphere and bilateral C-P angle tumors. Myelography and MRI demonstrated multiple intradural-extramedullary spinal tumors. Histologically, supratentorial tumors were transitional meningiomas and spinal tumors were neurinomas. It is well known that von Recklinghausen's disease is often associated with brain or spinal tumors. But, in the literature, only 22 cases of von Recklinghausen's disease combined with multiple brain tumors with different histological types and multiple spinal tumors have been reviewed. With our two cases, the average age of these 24 cases was 28.6 years old, nine cases were male and 15 cases were female. All patients had C-P angle tumors and 23 cases were combined with intracranial meningiomas. In this paper, the clinical features and diagnostic aspects were discussed.     

脊椎肿瘤的外科治疗

目的：探讨采用不同经路切除脊椎肿瘤与改进的人工椎体和自体植骨术治疗脊椎不同节段肿瘤的疗效。方法：对13例脊椎肿瘤采取切除瘤椎、人工椎体置换和自体植骨术;肿瘤包括颈椎4例,胸椎3例,腰椎6例。术前颈脊髓瘫痪1例,不完全瘫痪4例,肿瘤的术后病理学诊断有骨巨细胞瘤3例,骨囊肿、Ewing瘤、骨神经鞘瘤、软骨瘤、骨成纤维细胞瘤、骨纤维细胞瘤、骨嗜酸性肉芽肿、骨转移瘤、骨肉瘤和骨血管瘤各1例。结果：13例安全度过围手术期,无脊髓神经症状加重。除1例3个月恢复良好后失访外,余12例获平均4．3年随访,瘫痪5例完全恢复,12例均恢复家务和工作,X线片显示假体无松动、移位,植骨融合。结论：应根据肿瘤的脊柱节段和类型,选择手术治疗方式,采用人工椎体置换和自体植骨术,对完全切除脊椎肿瘤和重建脊椎稳定性是十分有益的。     

Surgical Strategies for Cervical Spinal Neurinomas

Cervical spinal neurinomas are benign tumors that arise from nerve roots. Based on their location, these tumors can also take the form of a dumbbell-shaped mass. Treatment strategies for these tumors have raised several controversial issues such as appropriate surgical indications and selection of surgical approaches for cervical dumbbell-shaped spinal neurinomas. In this report, we review previous literature and retrospectively analyze cervical spinal neurinoma cases that have been treated at our hospital. Surgical indications and approaches based on tumor location and severity are discussed in detail. Thus, with advances in neuroimaging and neurophysiological monitoring, we conclude that appropriate surgical approaches and intraoperative surgical manipulations should be chosen on a case-by-case basis.     

MRI在脊柱肿瘤结核鉴别诊断中的价值

[目的]评价磁共振成像（MRI）在脊柱肿瘤、结核鉴别诊断中的价值。[方法]回顾分析经病理证实的63例脊柱病变（31例结核，32例肿瘤）MRI资料。[结果]32例肿瘤椎间盘均未受累，其中10例仅累及椎体，20例椎体、附件均受累，2例仅累及附件。31例结核软组织均受累，附件均未见受累，其中29例椎间隙狭窄或消失。无论结核，还是肿瘤，T1WI以低信号为主，T2WI以高信号为主。[结论]MRI有助于脊柱肿瘤、结核鉴别诊断，椎间盘未受累为肿瘤特点，椎间盘、软组织受累及附件完整为脊柱结核的特点。     

Intramedullary dermoid tumor diagnosed with the assistance of magnetic resonance imaging

Intramedullary dermoid tumors are unusual. Traditional methods of diagnosing spinal tumors have included clinical suspicion, plain roentgenography, myelography, and computed tomography. A case of intramedullary tumor provisionally diagnosed preoperatively by traditional methods and specifically as dermoid tumor with the assistance of magnetic resonance imaging is presented. Diagnosis and treatment of spinal dermoid tumors with an emphasis on magnetic resonance imaging is discussed.     

Intradural spinal tumors in adults—update on management and outcome

Neurosurgical Review - Among spinal tumors that occur intradurally, meningiomas, nerve sheath tumors, ependymomas, and astrocytomas are the most common. While a spinal MRI is the state of the art...     

Multiple meningiomas of the spinal canal and posterior fossa--case report

The majority of multiple meningiomas are associated with von Recklinghausen's disease, and those that are not, i.e., true multiple meningiomas, particularly with simultaneous development in the posterior fossa and spinal canal, are very rare. We recently encountered a case of true multiple meningiomas in which the lesions were found simultaneously in the left posterior fossa and the upper thoracic spinal canal. The spinal meningioma consisted mainly of meningotheliomatous components, and that in the posterior fossa of fibroblastic components. Both tumors were surgically removed. The cause of the multiplicity in cases of true multiple meningiomas is unknown. Incidental multiplicity is a consideration when the number of tumors is relatively small. Spinal meningiomas are often undetected in the absence of neurological symptoms. However, even if von Recklinghausen's disease is not present, and particularly when more than two meningiomas have been discovered, the patient must be carefully examined for other tumors.     

脊髓髓内肿瘤的显微外科治疗及临床观察

目的　总结脊髓髓内肿瘤的显微手术经验 ,并对其并发症的处理及疗效进行评价。方法　对 48例髓内肿瘤采用显微外科手术。术中取侧卧位或半坐位 ,正中切口 ,尽量做到全切 ,并观察其术后的并发症及处理。结果　本组室管膜瘤、星形细胞瘤、脂肪瘤和血管网状细胞瘤分别占髓内肿瘤的 3 7.5 %、2 9.2 %、2 0 .8%、10 .4% ,其全切率分别为 88.9%、2 8.6%、10 .0 %、60 .0 % ;1例转移瘤全切。术后早期有感觉缺失 ,主要并发症有感染。 48例全部随访 ,平均 3 0 .4个月 ,大部分病人术后 1～ 3个月功能好转或无继续恶化 ,47例存活 ,1例死亡。结论　脊髓髓内肿瘤以良性及低恶性度肿瘤多见 ,手术效果较好 ,应尽早手术     

The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients

171 patients with intramedullary spinal cord tumors were operated on, of which 25 patients (15%), mostly children, developed symptomatic hydrocephalus. Twenty patients (12%) had malignant tumors, with 13 of the 20 cases (63%) complicated by increased intracranial pressure and ventriculomegaly. Of the remaining 151 patients with benign tumors (89%), only 12 (8%) developed symptomatic hydrocephalus. In an effort to understand the relationship between hydrocephalus and intramedullary spinal cord tumor, the authors analyze the level and histology of the neoplasm, as well as its association with spinal cysts. A review of the neurosurgical literature reveals that 34 similar cases of hydrocephalus associated with intramedullary spinal cord tumors have been reported to date. The authors note that the presence of hydrocephalus in patients with malignant intramedullary astrocytomas is associated with a shorter rate of survival than in those patients with high-grade lesions but without hydrocephalus, apparently due to rapid tumor progression. The ventriculomegaly seen with benign spinal cord gliomas has no statistically significant effect upon long-term prognosis.