Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 37 year-old patient with tuberous sclerosis and renal failure had renal transplantation performed, after 21 months of hemodialysis treatment and cerebral glioma removal, with good results 7 years later. In order to assess renal transplantation as a treatment for end-stage renal failure of tuberous sclerosis, we have reviewed the literature. 14 observations could be analysed: the patients were young with a mean age of 29.2 years, women in majority, with minor neurologic involvement. Because of the risks of neoplastic transformation and angiomyolipoma bleeding, bilateral nephrectomy is cautious, before or during the renal transplantation. Otherwise a close monitoring of the native kidneys by CT-scan is necessary. So the patients with tuberous sclerosis are good candidates for renal transplantation, with the same results as other patients of the same age, with no aggravation of the neurologic disorders, even when a cerebral tumor has been removed, as in our case.
 ...
PMID:[Renal transplantation in Bourneville tuberous sclerosis]. 781 64

Tumors of the central nervous system (CNS) are common causes of morbidity and mortality. These tumors can occur sporadically or in individuals with genetic disorders predisposing to cancer development. Such syndromes include neurofibromatosis type 2, neurofibromatosis type 1, Li-Fraumeni syndrome, as well as von Hippel-Lindau disease, tuberous sclerosis, and Turcot syndrome. There may also be familial syndromes resulting in glioma or meningioma alone, but these are not well understood. Development of sporadic gliomas is accompanied by a number of molecular genetic alterations, including activation of dominant oncogenes and inactivation of tumor suppressor genes. Some of these alterations may be associated with progression of gliomas to their most malignant form, glioblastoma multiforme. However, at this time molecular genetic analysis of gliomas does not provide better prognosis than histopathological staging. Recently, experimental treatments of gliomas in rodents, using gene therapy, have been reported. Results of these studies have been promising, and these techniques may represent a future direction for therapy in humans.
 ...
PMID:Genetics, prognosis and therapy of central nervous system tumors. 802 96

The clinical and pathologic features of an unusual retinal glioma that was the only clinically overt manifestation of tuberous sclerosis in a 27-year-old woman are reported. The tumor was composed predominantly of large pleomorphic cells with glassy eosinophilic cytoplasm. Immunologic staining yielded positive results for gamma enolase, but not for glial fibrillary acid protein. The histologic and immunopathologic features of this tumor were essentially identical to the subependymal giant cell astrocytoma found in tuberous sclerosis. This case illustrates the large overlap in dysplastic astrocytic and neuronal differentiation that can occur in the retina of patients with tuberous sclerosis. Giant cell astrocytoma of the retina can be mistaken for a malignant tumor histologically by persons unfamiliar with this entity.
 ...
PMID:Giant cell astrocytoma of the retina in tuberous sclerosis. 833

Overall, intraventricular supratentorial tumors are rare in childhood. Classification can be based on the separation of lesions originating in intraventricular structures, such as choroid plexuses, from glial neoplasms of the ventricular wall which tend to infiltrate the ventricular cavities. Aim of the present study is to review the most common neoplasms of this region in childhood. Choroid plexus tumors (papillomas and carcinomas) and subependymal giant cell astrocytomas are dealt with, while for the other neoplasms which are rarer or more typic of other age ranges, specific reports should be consulted. Choroid plexus papillomas affect infants and are the most frequent oncological type among congenital tumors. The malignant variant (grade III-IV) is represented by the less frequent the neoplasms. The malignant variant (grade III-IV) is represented by the less frequent choroid plexus carcinoma which is markedly invasive with respect to adjacent nervous structures and has a high tendency to form metastases even at onset. Anaplastic papillomas are intermediate forms whose correct histopathology is still debated. Most frequent glial tumors are subependymal giant-cell astrocytomas. They are benign tumors (grade I) typically albeit not constantly associated to tuberous sclerosis. In this case the differentiation from subependymal nodules plays a major role. Contrast enhanced CT is fundamental in this assessment. Anaplastic variants, though rare, are well-known.
 ...
PMID:Intraventricular supratentorial tumors in children. 867 37

Pediatric brain tumors occur with a frequency of 24 to 27 cases/year within a cohort of 1 million children. Nearly 25% of these lesions will involve the cerebral hemisphere, with the low-grade glioma representing the most common group of tumors in this location. Pilocytic and fibrillary astrocytomas are the most frequently encountered glioma, although other variants, such as the ganglioglioma, pleomorphic xanthoastrocytoma, astroblastoma, ependymoma, and oligodendroglioma, must also be considered in the differential diagnosis. The etiology of these tumors remains obscure, although may be linked to therapeutic radiotherapy, previous history of hematopoietic malignancy and maternal exposure to nitrosamine-laden foods. An associated link to a phakomatosis, e.g., neurofibromatosis, tuberous sclerosis, has also been documented to exist with astrocytomas, in particular. The goals of surgery include a complete removal, in most circumstances, with an attempt to alleviate an associated seizure disorder when intractable. This is possible in nearly every type of hemispheric glioma with the aid of intraoperative navigational systems, i.e., frameless stereotaxy, neurophysiological based stimulation mapping, and electrocorticography. In the setting where a complete removal is possible, no further therapy is warranted. For those lesions that are incompletely resected, conservative management with routine diagnostic imaging follow-up is appropriate. Reoperation is necessary if recurrence is documented and radiotherapy is utilized for those lesions that are incompletely resected following recurrence.
 ...
PMID:The impact of technical adjuncts in the surgical management of cerebral hemispheric low-grade gliomas of childhood. 883 58

The causes of glioma, the most common type of primary malignant brain tumor, are poorly understood. This study compared the personal and first-degree familial medical histories of 462 adults newly diagnosed with glioma in the San Francisco Bay Area between August 1, 1991, and March 31, 1994, with those of 443 controls who were frequency-matched on age, sex, and ethnicity. Cases and controls had equivalent personal histories of cancers other than brain cancer and most nervous system conditions, but they differed significantly regarding histories of epilepsy, seizures, or convulsions 3 or more years prior to diagnosis (odds ratio = 3.3, 95% confidence interval (CI) 1.4-7.9), chickenpox (odds ratio = 0.4, 95% CI 0.3-0.6), and shingles (odds ratio = 0.5, 95% CI 0.3-0.8). Four cases (less than 1%) and no controls had known genetic disorders (three had neurofibromatosis and one had tuberous sclerosis). Cases and controls had similar family histories of cancer and seizures. However, the odds ratio for a validated family history of primary brain tumor was 2.3 (95% CI 1.0-5.8). These results suggest that although family history of any cancer probably is not an important risk factor for adult glioma, a family history of brain tumors may play a role. Variation in exposure to or biologic response to common viral infections might play a greater role in the etiology of adult glioma than family history.
 ...
PMID:Familial and personal medical history of cancer and nervous system conditions among adults with glioma and controls. 909 74

The tuberous sclerosis 2 (TSC2) gene is thought to function as a growth suppressor in sporadic and TSC-associated hamartomas and tumors. Clusters of dysplastic glial cells are a common feature of cortical tubers and subependymal nodules in tuberous sclerosis patients. In an effort to identify TSC2 gene alterations in sporadic gliomas, we detected a novel polymorphism adjacent to the 3'splice site of intron 4. We evaluated the distribution of this variant allele in a series of 244 patients with glial tumors, including 55 gangliogliomas, 31 pilocytic astrocytomas (WHO grade I), 50 astrocytomas (WHO grades II and III), and 108 glioblastomas (WHO grade IV). The allelic distribution in the general population was estimated by examining 381 healthy blood donors. This rare allele appeared in the control population and in the patients with astrocytic gliomas with a virtually identical frequency (8.14%, and 8.20%, respectively). The frequency of the rare allele in gangliogliomas, however, was significantly higher (15.5%; p = 0.024). The fact that both gangliogliomas and cortical tubers in tuberous sclerosis contain neuronal and astrocytic elements and may resemble each other histologically suggests that the TSC2 gene may be involved in the development of these tumors. The rare allele of the TSC2 gene emerges as a candidate for a predisposing factor for the formation of sporadic gangliogliomas.
 ...
PMID:A novel splice site associated polymorphism in the tuberous sclerosis 2 (TSC2) gene may predispose to the development of sporadic gangliogliomas. 921 Aug 77

We report a 9-year-old girl with tuberous sclerosis presenting intractable adversive seizure. She had been suffering from frequent attacks of consciousness loss since the age of 6 years. Although a considerable amount of antiepileptic drugs had been administered, her epileptic attacks were not controlled, but instead rather increased. She had been suffering from adversive seizure to the right for more than 2 years. CT scan failed to show any abnormal density area. MRI showed a small lesion in the left frontal subcortical area. The electroencephalogram showed relatively mild abnormal waves in the left hemisphere. We undertook surgical removal of the lesion with epileptogenic foci because her epilepsy has not been controlled and the lesion could be a glioma. Abnormal spike waves were detected around the lesion with electrocorticogram. "Gyrectomy" technique was employed and the spike waves totally disappeared. After the surgery, no neurological deterioration was presented. She has suffered no seizure attack since the surgery even though the amount of the antiepileptic drugs has been significantly decreased. Resection of the epileptogenic foci as well as the abnormal lesion using the technique of gyrectomy is useful for the control of the intractable epilepsy, and makes the quality of life of patients much higher.
 ...
PMID:[A case of tuberous sclerosis presenting intractable adversive seizure, successfully resected with the technique of "gyrectomy"]. 933 Apr 2

Surgical specimens from 30 patients (13 males and 17 females) with intractable epilepsy with brain tumors and allied lesions were histopathologically examined: 4 of nonneurogenic origin (1 angiolipoma with cortical dysplasia and 3 cavernous hemangiomas), 2 low-grade fibrillary astrocytomas, 1 pleomorphic xanthoastrocytoma, 3 pilocytic astrocytomas with nuclear polymorphism, 1 oligoastrocytoma, 9 gangliogliomas, 3 gangliogliomatous lesions combined with tuberous sclerosis-like dysplastic changes, and 7 undetermined lesions suspected of being mixed glioma, dysembryoplastic neuroectodermal tumor (DNT), or dysplasia. They were all located supratentorially: in the temporal lobe in 21, frontal lobe in 6, and parietooccipital lobe in 3. The age of onset was under 20 years in most patients. Some kinds of dysplasias, such as focal cortical dysplasia, glioneuronal heterotopia, and clustered neurons in the hippocampus and amygdaloid nucleus, were combined in 11 cases, especially those with age of onset under 10 years. Pilocytic astrocytoma-like features were seen in 5 of the gangliogliomas and 3 of the undetermined lesions, and DNT-like features in 2 of the former and 3 of the latter. Gangliogliomas, pilocytic astrocytomas, mixed gliomas, DNTs, and dysplasias may be closely inter-related in the development of intractable epilepsies of young patients.
 ...
PMID:Brain tumors in surgical neuropathology of intractable epilepsies, with special reference to cerebral dysplasias. 987 63

A recent case of angiomyolipoma (AML) with a prominent component of polygonal epithelioid cells is described. A 27-year-old Japanese male with tuberous sclerosis presented with massive abdominal tumors increasing progressively in size. The patient died of respiratory disturbance and the autopsy revealed massive tumors in the bilateral kidneys, liver and lymph nodes, subependymal giant cell glioma of the brain and lymphangiomyomatosis of the lungs. The giant tumors were an unusual type of AML with a component of polygonal epithelioid cells, which showed a hepatocellular carcinoma-like pattern in some areas. Smooth muscle components comprising spindle cells, short or plump spindle cells and polygonal epithelioid cells frequently exhibited positive staining for HMB-45 but negative staining for epithelial cell markers. The unusual AML presented in this case was thought to be of low-grade malignancy and slow growing. It has been suggested that angiomyolipomas with diffuse areas of epithelioid cell component are potentially malignant. Immunostainings positive for HMB-45 but negative for epithelial cell markers are considered to be useful in differentiating AML with polygonal epithelioid cell component from other tumors, especially from renal cell carcinoma and hepatocellular carcinoma.
 ...
PMID:Multiple giant angiomyolipomas with a polygonal epithelioid cell component in tuberous sclerosis: an autopsy case report. 995 47


<< Previous 1 2 3 4 5 6 Next >>