1/119. Transnasal endoscopic repair of congenital defects of the skull base in children.OBJECTIVE: To examine imaging findings and methods of endoscopic treatment of congenital skull base defects in children. DESIGN: Retrospective study and case series. SETTING: Academic tertiary care center. patients: Four patients (aged 12 and 14 months and 8 and 13 years) were included from 1995 to 1997. Three presented with a nasal glioma, which was recurrent in 1 case. The fourth patient presented with bacterial meningitis due to a spontaneous cerebrospinal fluid leak. Computed tomography and magnetic resonance imaging were used to locate the defect of the skull base. INTERVENTION: Transnasal endoscopic resection of the glioma or the meningocele, with immediate repair of the skull base defects using free mucosal flaps and/or pediculized mucosal flaps and/or conchal cartilage together with fibrin glue and nasal packing during a 3-week period. RESULTS: None of the 4 patients has experienced recurrent cerebrospinal fluid leaks or postoperative meningitis. CONCLUSIONS: The transnasal endoscopic repair of congenital meningoceles is a reliable technique in select pediatric patients. Computed tomography and magnetic resonance imaging provide information that can be used to help the surgical procedure.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/119. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
3/119. Globular glioma of the tectum.Brainstem gliomas are a heterogeneous group of tumors whose prognosis and treatment depend not only on the histologic features but also on the location within the brainstem. magnetic resonance imaging allows the recognition of a distinct type of brainstem glioma of the tectal region of the midbrain, leading to aqueductal compression and hydrocephalus. The radiologic appearance of these tumors is usually rather uniform, with a characteristic nonenhancing thickening of the tectal plate. Because of its protracted course, no further treatment is necessary beyond cerebrospinal fluid diversion and close clinicoradiologic follow-up. The authors report two children with tectal plate gliomas of unusual but strikingly similar appearance. They present a clinical picture suggestive of intracranial hypertension without localizing signs. Magnetic resonance images reveal hydrocephalus related to the presence of perfectly circular lesions, hypointense on T1 and hyperintense on T2, which could be mistaken for parasitic cysts or represent dilated rostral portions of the sylvian aqueduct. After the cerebrospinal fluid diversion procedures, no further treatment was given, with one of the patients being monitored for 10 years and the other for 8 months, without tumor progression. These patients demonstrate that tectal gliomas, despite sharing a good prognosis, may have various patterns of growth, leading to unusual radiologic appearances that may pose diagnostic difficulties.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
4/119. Gliomatosis cerebri: cytologic and autopsy findings in a case involving the entire neuraxis.We describe the case of a 7-year-old girl who was clinically diagnosed as having a pontine glioma based on magnetic resonance imaging studies. Neoplastic cells were identified upon cytologic examination of cerebrospinal fluid. autopsy studies revealed an anaplastic astrocytoma (WHO grade III) diffusely infiltrating the cerebral hemispheres, brain stem, cerebellum, leptomeninges, and spinal cord to the level of the conus medullaris. The Ki-67 labeling index focally approached 30%. Although many of the neoplastic cells displayed elongated twisted nuclei reminiscent of microglia, these cells stained intensely for glial fibrillary acidic protein, supporting an astrocytic origin. Unusual features of this case of gliomatosis cerebri include involvement of the entire central neuraxis, correlation with pre-mortem lumbar puncture cytology, and a markedly elevated Ki-67 labeling index.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/119. ependymoma of the cauda equina.Myxopapillary ependymomas are benign tumours which occasionally metastasize along cerebrospinal fluid pathways. Extraneural metastases of spinal ependymomas, however, are very rare, even more so when situated in the pleura. We report the case of a 67 year old woman presenting with shortness of breath after recurrent myxopapillary ependymomas of the cauda equina. Chest X-ray showed multiple pleural lesions diagnosed as metastases of a myxopapillary ependymoma. The MIB-1 proliferation index was 3.1% for the initial spinal tumour, 14.2% for the first and 11.2% for the second recurrence while 12.0% for the pleural metastasis.- - - - - - - - - - ranking = 1.5keywords = spinal (Clic here for more details about this article) |
6/119. Spinal oligodendroglioma with gliomatosis in a child. Case report.The authors present a rare case of oligodendrogliomatosis in a child, which they believe originated from a primary spinal cord tumor. At 2.5 years of age this boy developed poor balance, neck stiffness, and a regression in developmental milestones. A computerized tomography (CT) scan of the head initially revealed ventriculomegaly and multiple cystic cerebellar lesions. In addition, magnetic resonance (MR) imaging revealed a cystic intramedullary lesion involving the cervical spinal cord. A CT scan of the head and an MR image obtained 3 years later demonstrated diffuse small cysts on the surface of the brainstem, cerebellum, medial temporal and inferior frontal cortices, subcortical white matter, and corpus callosum suggestive of leptomeningeal tumor spread. Analysis of pathological specimens obtained at surgery showed neoplastic glial cells with small, uniform nuclei and perinuclear clear zones. The cells appeared to migrate along the subpial space but no tumor cells were present in the subarachnoid space. These findings were compatible with a diagnosis of oligodendrogliomatosis cerebri. Despite having a complicated course, chemotherapy with carboplatin has provided the patient with long-term palliation and a high quality of life. This case may represent the fifth report in the literature of oligodendrogliomatosis occurring in a child but only the third occurring with a spinal primary tumor.- - - - - - - - - - ranking = 1.5keywords = spinal (Clic here for more details about this article) |
7/119. Primary diffuse leptomeningeal gliomatosis: unusual MRI with non-enhancing nodular lesions on the cerebellar surface and spinal leptomeningeal enhancement.A 28 year old man presented with a 1 month history of symptoms of intracranial hypertension. Examination showed bilateral papilloedema and meningeal signs. magnetic resonance imaging showed nodular lesions on the cerebellar and pontine surface and thickening of the thoracic spinal leptomeninges. Throughout the course of the disease, contrast enhancement was detected in the spinal leptomeninges but not intracranially. Primary diffuse leptomeningeal gliomatosis (PDLG) was diagnosed by biopsy and later confirmed on necropsy. The present case is remarkable for the nodular superficial cerebellar lesions and the absence of intracranial contrast enhancement of the leptomeninges.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
8/119. Intraspinal extradural myxopapillary ependymoma of the sacrum arising from the filum terminale externa. Case report.Extradural ependymomas of the sacrococcygeal region are very rare, with most arising from the soft tissues of the presacral area or from the regions dorsal to the sacrum. In even rarer circumstances, the tumor may arise within the sacral canal, likely as a result of ependymal cells of the extradural filum terminale. Because of bone erosion caused by extension of the tumor into the pelvis or dorsal to the sacrum, a truly intraspinal extradural ependymoma in this region has until now never been clearly demonstrated. The authors present a patient with a myxopapillary ependymoma arising from the filum terminale externa in which there was no involvement of the intradural filum or extension outside the sacral canal. A review of the literature is presented, with emphasis on the pathogenesis and clinical management of these rare tumors.- - - - - - - - - - ranking = 2.5121814822827keywords = spinal, canal (Clic here for more details about this article) |
9/119. Primary diffuse leptomeningeal gliomatosis predominantly affecting the spinal cord: case report and review of the literature.Primary leptomeningeal gliomatosis is a rare, fatal neoplastic syndrome. A 71 year old man is reported on, who after a 2 month history of back stiffness, epigastric pain, and weight loss developed visual blurring. Cranial CT and MRI studies showed no leptomeningeal enhancement. Examination of CSF 10 weeks premortem showed an increase in protein and decrease in glucose but no malignant cells. He became increasingly confused and repeated CSF examination showed inflammation and a few suspicious cells but no definitive evidence of neoplasia. He died 7 months after onset of his initial symptoms. At postmortem meningeal whitening was seen at the base of the brain and over the spinal cord. histology disclosed diffuse leptomeningeal gliomatosis (GFAP positive, cytokeratin negative) over the brain, optic nerves, and spinal cord without parenchymal involvement. No tumour was found in internal organs. The diagnosis of primary leptomeningeal gliomatosis was not evident after cranial CT and MRI and CSF examination premortem. Suspected cases need MRI scanning of the entire neuraxis and meningeal biopsy.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
10/119. Nasal glioma: a case report.Nasal gliomas are rare, benign, congenital tumors that are thought to be the result of an abnormality in embryonic development. Three types of clinical presentations have been recognized: extranasal (60%), intranasal (30%), and combined (10%). Clinically, these masses are firm, noncompressible, nonpulsatile, gray or purple lesions that obstruct the nasal cavity intranasally and cause deformity extranasally. Histologically, these tumors are made up of astrocytic neuroglial cells interlaced with fibrous and vascular connective tissue that is covered with skin or nasal respiratory mucosa. The preferred treatment is excision via a frontal craniotomy approach for those patients whose gliomas have an intracranial connection, for those patients who have cerebrospinal fluid rhinorrhea, and for those who have recurrent episodes of meningitis. Otherwise, a transfacial lateral rhinotomy is advised. In this article, we describe a case of a nasal glioma that was removed via a lateral rhinotomy.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
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