Myelopathy presenting as an intrinsic spinal cord tumor

Two patients are presented who became progressively quadriplegic with myelographic appearances suggesting intrinsic spinal cord tumors. In both cases, laminectomy and biopsy of the spinal cord disclosed non-neoplastic conditions (neuromyelitis optica and acute necrotic myelopathy) confirmed at autopsy. Since surgery is not helpful in such cases, the problems of differentiation between such lesions and intraspinal tumors are reviewed.     

Gliomatosis of the brain and spinal cord masquerading as infective lesions

BACKGROUND

Gliomatosis of the brain or spinal cord is an infiltrating glial neoplasm that shows widespread invasion of the central nervous system with relative sparing of the underlying cytoarchitecture. Acceptance of the idea that the condition represents a distinct entity remains controversial in the absence of conclusive pathogenetic data. The clinico-pathological problems and difficulties in the ante-mortem diagnosis as well as the clinical and pathological similarities to infective lesions are evaluated.

METHODS AND RESULTS

Three cases of cerebral and spinal gliomatosis are presented that clinically mimicked infective lesions and were diagnosed and treated as such. The correct diagnosis in each case was only made at post-mortem examination. The ante-mortem diagnosis of this rare tumor remains difficult owing to poor correlation of clinical, neuroradiological, and neuropathological findings.

CONCLUSION

Gliomatosis of the brain and spinal cord may simulate infective lesions owing to difficulty in ante-mortem diagnosis because of vagueness of physical, radiological, and pathological findings. It is a diagnostic pitfall particularly in our setting where there is a high incidence of HIV/AIDS and patients often present with opportunistic infections such as mycobacterial, fungal, and/or viral infections, which show an atypical clinical picture and radiological findings. Multifocal neurologic deficit with noncontrast enhancing lesions that show diffuse contiguous involvement with overall preservation of the spinal or cerebral architecture and do not respond to infective treatment could suggest a diagnosis of gliomatosis cerebri.     

The case. Giant-cell tumor

Giant-cell tumor is a benign but locally aggressive primary bone tumor that requires surgical management. Most giant-cell tumors initially are demonstrated on radiographs as distal, subarticular, geographic osteolytic lesions. Abundant giant cells on histology are reactive secondary to a neoplastic fibroblast-like stromal cell. Giant cells are present in many neoplastic and non-neoplastic bone lesions; therefore the diagnosis of giant-cell tumors requires correlation of clinical, imaging, and pathologic data to exclude other lesions that demonstrate a similar histologic pattern. A small number of giant-cell tumors result in lung lesions, many of which have benign histology, can be treated by wedge resection, and do not affect long-term outcome. After adequate surgical curettage and the use of intraoperative surgical adjuvants, recurrence rates of approximately 10% are reported.     

Magnetic resonance imaging in acute spinal cord trauma

Magnetic resonance imaging (MRI) has proven to be an invaluable tool for evaluating neoplastic, congenital, and degenerative conditions of the spine and spinal cord. Because of various technical limitations, however, the use of MRI in acutely spinal cord-injured patients has not been fully explored. Sixty-two spinal cord-injured patients underwent MRI within the first 36 hours of injury. A variety of pathological findings were detected on the MRI scans: anatomical cord transection (7 cases), spinal cord deformity secondary to extrinsic compression (28), focal cord enlargement/swelling (21), hyperintense intramedullary lesions (17), and disc herniations (2). MRI may be a useful adjunct in the evaluation of acute spinal cord injury.     

Intramedullary mature teratoma of the cervical spinal cord at C1-2 associated with occult spinal dysraphism in an adult. Case report and review of the literature

There is a well-recognized association between dysontogenetic tumors of the spinal cord (including teratomas and enterogenous cysts) and dysraphic congenital spinal malformations. The authors present a case of an adult with an intramedullary mature teratoma (IMMT) at the level of C1-2 of the cord associated with dysraphic congenital spinal malformations. Intramedullary mature teratomas of the cervical region of the spinal cord are very rare in adults; only four such lesions have been reported, two of which involved upper cervical segments. Despite the potentially critical location of the tumor, monitored microsurgery resulted in complete removal of the tumor with an intact surrounding capsule, associated fibrous tract, and ellipse of skin with a central dimple. There was an excellent postoperative neurological outcome. The clinical features, imaging studies, treatment options, postoperative outcome, and plausible pathological correlations of IMMTs are discussed.     

Spinal cord compression: Tumors and allied non-traumatic conditions

Tumors of the spinal cord, if diagnosed and removed early in their development, can be permanently cured. Any condition which produces compression of the spinal cord may simulate tumors of the spinal cord.In order to correlate more fully the relation of clinical, surgical, and pathologic aspects of compression of the spinal cord, material gathered from cases reported at The Mayo Clinic from 1912 to 1929 has been analyzed. In view of the difficulty in nomenclature of tumors of the spinal cord, the terminology was confined to an accepted classification, and the microscopic examination was made by means of frozen sections stained with hematoxylin and eosin.Of 312 cases analyzed, 223 tumors were found which did not involve the spinal cord except by compression; these were classified as extramedullary, and were divided again into 156 intradural and 67 extradural tumors. There were also 89 tumors which involved the spinal cord and were classified as intramedullary.Included in the group of 67 cases of extradural tumors compressing the spinal cord were lesions arising from bone, intervertebral disks, extradural fat, spinal nerves, and blood vessels, as well as unsuspected metastatic malignant lesions. A preoperative diagnosis of bony extradural lesion may often be made by roentgenologic examination; likewise, it is possible to make a presumptive diagnosis of metastatic malignant lesion when the primary lesion can be determined.The intradural extramedullary series of tumors comprised the largest group, and fortunately carried the most favorable prognosis. These lesions take their origin from the fibroblastic structures of the meninges and the vessels of the meninges, or they are projected into the subarachnoid space from without the dura or from within the spinal cord. The 2 types of tumor predominating are the endothelioma, or meningeal fibroblastoma, and the neurofibroma. In this series, about half of the intradural extramedullary tumors proved to be endotheliomas, and about a third were neurofibromas.In analyzing the intramedullary lesions, 89 cases were reviewed. Tissue was available in only 62 cases. Tumors arising from the cord and comprising the group of gliomas predominated, but also benign encapsulated tumors which were capable of complete removal were found. In a number of cases in which tissue was not removed, palliative relief was obtained by means of decompression, coincidental with laminectomy.In view of the fact that laminectomy can be performed with minimal risk, exploration can be carried out in many atypical cases of compression of the spinal cord for palliative as well as for diagnostic purposes. In a very high percentage of cases of compression of the spinal cord, a differential diagnosis between inflammatory and neoplastic lesions can be ascertained preoperatively by means of roentgenologic examination, complete neurologic study including examination of the cerebrospinal fluid, and the use of lipiodol when indicated. But the exact pathologic nature of the underlying cause of compression is rather difficult to determine, and this pathologic analysis furnishes evidence of the multiplicity of lesions that can be encountered at operation.     

脊髓血管母细胞瘤的MRI特点

【摘要】〓目的〓分析探讨脊髓血管母细胞瘤的MRI表现特征。方法〓回顾性分析经手术病理证实的7例脊髓血管母细胞瘤的临床及MRI表现。全部患者均行MRI平扫及增强扫描。结果〓单发6例,多发1例,共有脊髓血管母细胞瘤病灶8个,所有病灶均位于髓内。其中,延髓1个,颈段4个,胸段2个,腰段1个。肿瘤多呈点状、结节状、香肠状或不规则形状,肿瘤在MRI T1WI呈等或稍低信号,T2WI呈稍高信号,增强扫描上下径<20 mm的病灶呈明显、均匀强化,上下径>20 mm的病灶呈明显不均匀强化,其内可见多发流空的血管影;7例患者均伴有不同程度脊髓空洞或水肿。结论〓脊髓血管母细胞在MRI中具有特征性的影像表现,MRI对诊断脊髓血管母细胞瘤具有重要价值。     

"No performance in surgery more interesting and satisfactory": Harvey Cushing and his experience with spinal cord tumors at the Johns Hopkins Hospital

Although Harvey Cushing was a neurosurgical pioneer, his work on the spine remains largely unknown. In fact, other than his own publications, Cushing's patients with pathological lesions of the spine who were treated while he was at the Johns Hopkins Hospital, including those with spinal cord tumors, have never been previously described. The authors report on 7 patients with spinal cord tumors that Cushing treated surgically between 1898 and 1911: 2 extradural, 3 intradural extramedullary, and 2 intramedullary tumors. The authors also describe 10 patients in whom Cushing performed an "exploratory laminectomy" expecting to find a tumor, but in whom no oncological pathological entity was found. Cushing's spine surgeries were limited by challenges in making the correct diagnosis, lack of surgical precedent, and difficulty in achieving adequate intraoperative hemostasis. Other than briefly mentioning 2 of the 4 adult patients in his landmark monograph on meningiomas, these cases-both those involving tumors and those in which he performed exploratory laminectomies--have never been published before. Moreover, these cases illustrate the evolution that Harvey Cushing underwent as a spine surgeon.     

Spinal angiolipoma: case report and review of the literature

Summary Spinal angiolipomas are rare lesions usually found in the epidural space of the thoracic spine. This report presents a case of and reviews the literature on this rare entity. The etiology, clinical presentation, imaging, and treatment are discussed. In 92 reported cases of spinal angiolipoma 56 occurred in women (61%), and 36 in men (39%). Mean age of occurrence is 42.9 years (range 10 days–85 years) with most patients presenting with slowly progressive symptoms of spinal cord compression. Most cases occur in the extradural compartment, and are of the non-invasive subtype. This rare clinical entity must be considered in the differential diagnosis of spinal epidural lesions. In most cases complete removal is possible, however, prognosis is good even for infiltrating lesions. Thus, one must not risk neurological damage to attain complete resection.     

A case of intraroot cauda equina cavernous angioma: clinical considerations

STUDY DESIGN: Case report and literature review. OBJECTIVE: Intradural-extramedullary cavernoma is a rare condition with only 23 cases reported in the literature. Most cases described adhere to spinal root or spinal cord. We report an example of this rare entity located within the sheets of one cauda equina nerve root. SETTING: Rome, Italy. METHODS: The authors report clinical, radiological, surgical, and pathological features of intraroot cavernoma with a literature review. RESULTS: Patient was operated. One year after surgery, he was healthy. CONCLUSION: Lumbar intradural cavernous angioma may present with sciatalgia, low-back pain, neurological deficit, or, more rarely with subarachnoid hemorrhage or hydrocephalus. They are treated successfully with surgical resection but preoperative status is a predicting factor for outcome. Sometimes the sacrifice of the spinal root is inevitable because, as in present case, the cavernous angioma is included in the nerve root. These lesions are rare and benign lesions. It is important for neurosurgeons to be aware of the existence of this entity to avoid preoperative misdiagnosis with tumor.     

Nonenhancing tumors of the spinal cord

OBJECT: Enhancement of pathological entities in the central nervous system is a common finding when the blood-brain barrier has been compromised. In the brain, the presence or absence of gadolinium enhancement is often an indicator of tumor invasiveness and/or grade. In the spinal cord, however, contrast enhancement has been shown in all tumor types, regardless of grade. In this study the authors explore the incidence of nonenhancing tumors of the spinal cord and the clinical course of patients with these lesions. METHODS: A retrospective analysis was conducted in which investigators examined the patterns of enhancement of histologically proven intramedullary spinal cord tumors that had been evaluated at the Mayo Clinic between 1998 and 2002. The tumors that did not enhance were the subject of this report. RESULTS: A total of 130 patients with intramedullary tumors were evaluated. Of those, 11 patients (9%) had tumors that did not enhance. Histologically, a majority of tumors were astrocytomas (eight low-grade and two high-grade lesions); one tumor was a subependymoma. Morphologically, most of the tumors were diffuse and none had associated cysts. Tumors spanned from two to seven levels and were located throughout the spinal cord (four cervical, three cervicothoracic, one thoracic, and three thoracolumbar). Biopsy procedures were performed in eight patients, subtotal resection was performed in two, and gross-total resection in one. After a mean follow-up period of 19 months, tumors remained stable in eight patients but progressed in three, two of whom died. CONCLUSIONS: A number of intramedullary spinal cord tumors will not enhance after addition of contrast agents. The absence of enhancement does not imply the absence of tumor.     

Schistosoma haematobium presenting as an intrinsic conus tumour

In the small and diverse group of atypical, non-neoplastic intramedullary spinal cord lesions, parasitic infections are rarely considered, especially in Caucasian patients without systemic complaints or eosinophilia. A case of atypical myelopathy caused by Schistosoma haematobium is presented. The clinical, laboratory and imaging features in the MRI era both before and after treatment are discussed.     

Pleomorphic xanthoastrocytoma of the spinal cord. Case report

The authors present clinical, radiological, and pathological features in a patient with a pleomorphic xanthoastrocytoma (PXA) of the spinal cord. To their knowledge, this is only the second report of a spinal cord PXA. In addition they provide a review of the literature on these tumors.     

Primary spinal syringomyelia. Invited submission from the joint section meeting on disorders of the spine and peripheral nerves, March 2005

In the present review the author describes the different types of syringomyelia that originate from abnormalities at the level of the spinal cord rather than at the craniovertebral junction. These include posttraumatic and postinflammatory syringomyelia, as well as syringomyelia associated with arachnoid cysts and spinal cord tumors. The diagnosis and the principles of managing these lesions are discussed, notably resection of the entity restricting cerebrospinal fluid flow. Placement of a shunt into the syrinx cavity is reserved for patients in whom other procedures have failed or who are not candidates for other procedures.     

Intraoperative ultrasound for spinal lesions

A variety of spinal pathological processes demonstrated by intraoperative ultrasound is presented. Use of spinal ultrasound proved helpful in viewing alignment, assessing spinal cord pathology, and viewing anterior to the cord in cases of spinal trauma. As an operative adjunct, ultrasound was especially helpful for viewing extradural spinal metastases and cavitary lesions of the spinal cord, such as syrinxes, cystic tumors, and hematomas. In cases of spondylotic myelopathy, intraoperative ultrasound allowed assessment of the adequacy of the decompression, the space ventral to the cord, and the size and configuration of the spinal cord.     

In vivo proton magnetic resonance spectroscopy of human spinal mass lesions

OBJECTIVE: To observe metabolic differences between spinal tumor and other diseases in human spinal mass lesions, in vivo 1H magnetic resonance spectroscopy (MRS) was attempted to obtain metabolic signals in patients with various spinal mass lesions. METHODS: 1H nuclear magnetic resonance (NMR) spectra were obtained from 14 patients before surgery using a receive-only surface coil on a 1.5 T clinical magnetic resonance imaging (MRI) unit. MRS findings were compared with the histopathologic results from biopsy. In addition, tumor spectra were compared with the spectra of other benign diseases including disc herniation, which can mimic spinal cord tumor. In vitro 1H-NMR spectra were also collected from perchloric acid extracts of some spinal tumors. RESULTS: Typical water resonance line widths were in the 6- to 10-Hz range, but the metabolic signals observed were sufficiently resolved to be assigned from comparison with the 1H spectra of brain tissue. Choline was detected in all tumor spectra (n = 6) except ependymoma, whereas it was absent in other benign diseases including disc herniation (mimicking spinal cord tumors), dermoid cyst, tuberculosis, and non-multiple sclerosis myelitis. Spectral patterns of meningiomas, schwannomas, metastasis from renal cell carcinoma, and ependymomas in the spinal cord were similar to those of central nervous system (CNS) tumors. It was not possible to observe distinctive metabolic differences between benign diseases owing to relatively larger line broadening of some signals compared with that in CNS tissue. CONCLUSIONS: It appeared that acquisition of in vivo 1H-NMR signals was possible in human spinal mass lesions on a 1.5 T clinical MRI unit. Detection of choline only in the spinal tumors may indicate that there is some potential in using in vivo 1H-MRS to distinguish spinal tumors from disc herniation mimicking spinal cord tumors, non-multiple sclerosis myelitis, and dermoid cysts. On the basis of our NMR findings, however, it was not possible to distinguish between benign diseases.     

Pediatric skull, skull base, and meningeal tumors.

Calvarial neoplastic and non-neoplastic tumors are routinely encountered by all neurosurgeons. Benign and malignant skull base and meningeal tumors are relatively rare lesions in children. Interdisciplinary approaches to those tumors more frequently encountered in the pediatric population in these locations are discussed. Unique aspects of the diagnosis, treatment, and prognosis for infants and children are discussed.     

Spinal extradural arachnoid cyst

Summary Spinal extradural arachnoid cysts are rare expanding lesions in the spinal canal. They usually present with progressive signs and symptoms caused by spinal cord compression if they enlarge. A comprehensive review about spinal extradural arachnoid cyst is made including the author’s own case of a 59-year-old woman with a 6-month history of progressive back pain radiating to both legs. Key points concerning the possible pathogenesis including symptomatology, diagnosis, and the implications for treatment are highlighted. Surgical treatment is curative and this rare clinical entity should be considered in the differential diagnosis of spinal extradural lesions.     

Intramedullary astrocytoma with granular cell differentiation

Granular cell astrocytomas are uncommon tumors of the central nervous system (CNS) of which no cases have been documented in the spinal cord. This variant of glioma should not be confused with benign granular cell tumor which, although rare, has been well characterized in the spinal cord. We describe here the clinical, pathological, and radiological features of such an astrocytoma arising within the spinal cord at the dorsal level. A 48-year-old female was seen after about 1 year of dorsal pain and gradual spastic paraparesis. Magnetic resonance imaging (MRI) studies showed a 2-cm contrast-enhanced mass in the spinal cord at T6-T7, which had the appearance of an astrocytoma. At surgery, the tumor was found to be infiltrating a posterior column with no dural attachment. It was debulked and dissected. The histological diagnosis was astrocytoma with granular cell differentiation. In addition to documenting a unique example of intramedullary granular cell astrocytoma, we review the literature to investigate differences from other tumors with granular changes described in the spinal cord.