1/75. pulmonary edema after resection of a fourth ventricle tumor: possible evidence for a medulla-mediated mechanism.A well-recognized fact is that some patients may have development of pulmonary edema in association with disorders of the central nervous system. The origin of this phenomenon, known as neurogenic pulmonary edema, is unclear but may result, in part, from select pulmonary venoconstriction modulated by autonomic outflow from the medulla oblongata. We describe a 21-year-old man who had development of pulmonary edema in association with surgical resection of a brain tumor that was close to the medulla. Other than the possibility of medullary dysfunction, which could have occurred after surgical manipulation, no other risk factor for pulmonary edema was identified. Of note, the patient's blood pressure remained normal throughout the perioperative period, and no fluid overload or primary cardiac dysfunction was evident. This case supports the theory that the medulla is an important anatomic site of origin for neurogenic pulmonary edema and that alterations in medullary function can induce pulmonary edema in humans, independent of systemic hypertension.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/75. Heterotopic nasopharyngeal brain tissue associated with cleft palate.OBJECTIVE: The occurrence of extracranial brain tissue is rare. Most of the literature describes cases in which it is located around the nose and throat and has been classified as nasal glioma. Even more unusual is heterotopic brain tissue in the nasopharynx. We were able to find only 17 previously reported cases. Of these 17 cases, 6 had heterotopic brain tissue located in a cleft palate. This report comments on the identification and treatment of heterotopic brain tissue associated with cleft palate without connection to the central nervous system. Our case subject is a 10-month-old girl diagnosed with heterotopic nasophranygeal brain tissue associated with cleft palate. RESULTS: Excision and palatoplasty were performed conjunctively with excellent results. CONCLUSIONS: Simultaneous excision of heterotopic nasopharyngeal brain tissue and palatoplasty of the cleft palate is an excellent option for treatment of these cases.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/75. Chordoid glioma of the third ventricle: immunohistochemical and molecular genetic characterization of a novel tumor entity.Chordoid glioma of the third ventricle was recently reported as a novel tumor entity of the central nervous system with characteristic clinical and histopathological features (Brat et al., J Neuropathol Exp Neurol 57: 283-290, 1998). Here, we report on a histopathological, immunohistochemical and molecular genetic analysis of five cases of this rare neoplasm. All tumors were immunohistochemically investigated for the expression of various differentiation antigens, the proliferation marker Ki-67, and a panel of selected proto-oncogene and tumor suppressor gene products. These studies revealed a strong expression of GFAP, vimentin, and CD34. In addition, most tumors contained small fractions of neoplastic cells immunoreactive for epithelial membrane antigen, S-100 protein, or cytokeratins. The percentage of Ki-67 positive cells was generally low (<5%). All tumors showed immunoreactivity for the epidermal growth factor receptor and schwannomin/merlin. There was no nuclear accumulation of the p53, p21 (Waf-1) and Mdm2 proteins. To examine genomic alterations associated with the development of chordoid gliomas, we screened 4 tumors by comparative genomic hybridization (CGH) analysis. No chromosomal imbalances were detected. More focussed molecular genetic analyses revealed neither aberrations of the TP53 and CDKN2A tumor suppressor genes nor amplification of the EGFR, CDK4, and MDM2 proto-oncogenes. Our data strongly support the hypothesis that chordoid glioma of the third ventricle constitutes a novel tumor entity characterized by distinct morphological and immunohistochemical features, as well as a lack of chromosomal and genetic alterations commonly found in other types of gliomas or in meningiomas.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/75. Endoscopic biopsy for tumors of the third ventricle.Twelve patients underwent endoscopic biopsy of a tumor involving the third ventricle. Nine patients had no significant medical history while 3 had a history of cancer. Unique characteristics of each case dictated the optimal surgical technique. Endoscopic tumor biopsy was combined with additional procedures in 9 cases; shunt insertion (3), shunt insertion with endoscopic septostomy (5), and transcallosal craniotomy (1). diagnosis was established in 11 patients (92%); 6 primary brain tumors, 3 metastatic central nervous system tumors, 1 metastatic systemic cancer, and 1 region of post-treatment gliosis. One case was aborted due to poor visualization. Therapy was directly influenced by endoscopic biopsy in 11/12 cases (92%) and craniotomy for tumor resection was avoided in 10/12 patients (83%). Of the 5 patients who underwent endoscopic septostomy, 4 required no subsequent procedures for hydrocephalus. There were no complications, and hospital stay averaged 1.78 days for patients who underwent successful endoscopic biopsy. Tumors of the third ventricle are amenable to endoscopic biopsy with excellent diagnostic yield and low morbidity. The procedure must be tailored depending upon the tumor location within the third ventricle, the degree of ventriculomegaly, and the need to perform a septostomy. Singularly or combined with other endoscopic procedures, patients can be spared multiple and more invasive surgical procedures.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
5/75. Chordoid glioma: a novel tumor of the third ventricle.Chordoid glioma of the third ventricle is a recently characterized primary neoplasm of the central nervous system. We present a case and discuss the pathologic and radiologic features. We are aware of only 16 other cases documented in the world literature. This radiologic-pathologic correlation alerts pathologists and radiologists to recognize chordoid glioma as a distinct clinicopathologic entity restricted to the third ventricular area of adult patients.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/75. Midline nasal mass in infancy: a nasal glioma case report.Congenital midline nasal masses are rare anomalies that occur in about one in 20,000-40,000 live births. The most common are dermoid/epidermoid tumors, nasal cerebral heterotopias (nasal gliomas), and nasal encephaloceles; some have an actual or potential central nervous system connection. Nasal gliomas are CNS masses of neurogenic origin which have lost their intracranial connections and present as an obvious external or intranasal mass at birth without associated surgical symptoms. Careful evaluation is required to confirm the diagnosis and appropriate management. The interpretation of CT and MR images can be difficult but is useful in differentiating nasal gliomas from other congenital nasal masses. The presence of a fibrous stalk may be associated with cranial defects and CSF leak. Excisional biopsy allows histopathologic diagnosis and is the definitive treatment. They are benign lesions, and recurrences are rare, so conservative cosmetic surgical techniques should be chosen for gliomas where there is no proven intracranial extension. The authors report an illustrative nasal glioma case in a one-year-old male infant with extranasal and intranasal components, and discuss the therapeutic options.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/75. Rhabdoid glioblastoma.Rhabdoid phenotypic change has been described in a number of different neoplasms from diverse organ sites. These tumors share common light and electron-microscopic features, display a polyphenotypic immunohistochemical profile and often show cytogenetic abnormalities of chromosome 22. In the central nervous system (CNS), most rhabdoid tumors occur in the posterior fossa of very young children and are associated with a primitive neuroectodermal tumor (PNET) component and are designated atypical teratoid/rhabdoid tumors. Infrequently, other rhabdoid tumors of the CNS have been described, including rhabdoid meningiomas and malignant rhabdoid tumors of uncertain histogenesis. Several examples of conventional gliomas displaying significant areas with rhabdoid morphology were also presented in an abstract by Kepes and Moral [1991], although never published in final manuscript form. We now detail the case of an 18-year-old male with an aggressive, supratentorial CNS rhabdoid tumor that was associated with an epithelioid glioblastoma and apparently arose from areas of low-grade glioma. The rhabdoid tumor component was present in the original tumor but became more predominant with each of 3 successive resections. No areas of PNET were identified. Electron microscopy and immunohistochemistry showed features classic for rhabdoid tumors and cytogenetic studies demonstrated multiple tumor clones with monosomy 22. This case documents progressive rhabdoid transformation of a glioma, expands the spectrum of CNS tumor types that can display a rhabdoid phenotype and highlights the diagnostic and therapeutic challenges with this type of tumor.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
8/75. Nasal glioma or nasal glial heterotopia? Morphological, immunohistochemical and ultrastructural study of two cases.The term nasal glioma has been used to describe a congenital benign tumor of the nasal region containing neural tissue. The nature of these lesions remains open to controversy, because of the different locations of the heterotopic neural tissue involved, the deficient development of the bony structures and the persistence or not of the structural relations with the central nervous system. More recent terms define these lesions as ectopic nervous tissue. A clinical, morphological, ultrastructural and immunohistochemical study is made of two cases of nasal glioma, one associated with agenesis of the corpus callosum. In this case, the mother had been treated with clomiphene. In such cases, morphological and immunohistochemical findings support that "nasal glioma" remain valid as a descriptive term defining a congenital benign tumor composed of heterotopic neural tissue within the nasal region and covered by skin, that may recur following incomplete surgical resection.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/75. Primary cerebral sarcoid granuloma: the importance of definitive diagnosis in the high-risk patient population.sarcoidosis of the central nervous system has been variously reported in 5-15% of all sarcoid patients. However, presentation of sarcoidosis as an isolated 'intracranial tumor' is rare. A 35-year-old African-American woman presented with intractable headaches. neuroimaging revealed a tumor that was suggestive of a glioma or meningioma or metastasis. The symptoms did not respond to steroids, and an open biopsy of the lesion revealed non-caseating granuloma. A thorough work-up for systemic sarcoidosis was negative. The patient remains symptom-free at a 2-year follow-up. Primary sarcoid granuloma of the brain is rare. Once systemic disease has been excluded, early tissue diagnosis is crucial. This is particularly relevant for patients in the high-risk population before considering empirical radiosurgery.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/75. Melanin as a component of cerebral gliomas: the melanotic cerebral ependymoma.Even though melanin is found in certain central nervous system neurons and leptomeningeal melanocytes, gliomas rarely possess melanin. A few rare lethal melanotic medulloblastomas found typically in the cerebellar vermis of children have been described, but neuroglial cells have not been observed to contain this pigment. Since glial elements and the melanotic pigmented layer of the retina are derived from the same ciliated epithelium of the embryonic neural tube, recognition of melanin in a cerebral glioma was predictable. Indeed, melanin was observed in a cystic cerebral ependymal glioma from a 30-year-old woman, supporting the potential of glial cells to produce melanin.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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