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

This is a descriptive study of 19 families with glial tumors. Twelve were identified prospectively from 178 consecutive, unrelated adults and children with newly diagnosed gliomas seen at a regional cancer center between 01 Jan 89 and 31 Mar 91 (6.7%). There were 45 affected members (42 confirmed); 30 males, 15 females, ages 4 months-78 years (median, 44.5 years; mean, 38.9 years). Two families had four affected members, three families had three, and the others two. All confirmed tumors were supratentorial and all, save one, contained an astrocytic element. Three additional members of two families had other brain or neuroectodermal tumors. These families were not unusually cancer prone and did not appear to have neurofibromatosis, tuberous sclerosis, or colonic polyposis. There was no consistent pattern of inheritance.
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PMID:Gliomas in families. 142 46

We report a case of association of a brain tumor with multiple intestinal polyposis (Turcot's syndrome) and offer a critical analysis of the relevant literature with a view to revising the classification of the syndrome in relation to familial multiple polyposis and Gardner's syndrome. For this purpose, we considered only cases of intestinal polyposis associated with a primary neuroepithelial tumor (medulloblastoma, glioma, or glioblastoma) as originally described by Turcot. Differences emerged, depending on the central nervous system tumor type, which suggests that this neoplastic association may be classified as two distinct syndromes.
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PMID:Association between neuroepithelial tumor and multiple intestinal polyposis (Turcot's syndrome): report of a case and critical analysis of the literature. 184 39

A nonfamilial case of Turcot syndrome (glioma-polyposis syndrome) is described. A 16-year-old male with no siblings first developed a frontal astrocytoma, and was later found to have colonic polyposis with adenocarcinoma. The family history was negative for the syndrome, but his parents were first cousins.
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PMID:Nonfamilial turcot syndrome presenting with astrocytoma--case report. 247 61

The reported clinical manifestations of Turcot syndrome were studied to determine whether these corresponded to those of Turcot's original cases. Among the patients with well-documented colonic lesions, the colonic lesions were classified into three groups. First, there was a main group in which colonic lesions had the following characteristics that coincided with those of Turcot's original cases: a low number of polyps (20-100), large polyps over 3 cm in diameter, and complication by colonic cancer during the second or third decades. In the second group, the patients had too few polyps to be diagnosed as polyposis. The third group included patients with numerous colonic polyps similar to those of familial polyposis coli. The recognition of these characteristics of colonic lesions may lead to early detection of glioma in the asymptomatic period.
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PMID:Turcot syndrome and its characteristic colonic manifestations. 400 35

A case of Turcot syndrome (glioma polyposis) is described in a 22-year-old woman. The patient initially presented with a frontoparietal glioma, and was subsequently found to have segmental colonic polyposis with adenocarcinomatous changes. Her colonic polyposis was nonfamilial.
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PMID:Turcot syndrome (glioma polyposis). Case report. 405 88

A case of Turcot's syndrome is described in an 8-year-old girl. Turcot's syndrome is a rare hereditary disease in which malignant glioma of the central nervous system is associated with colonic polyposis. The patient initially presented with a left parietal glioblastoma diagnosed by computed tomography (CT), and was subsequently found to have nonfamilial colonic polyposis.
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PMID:Turcot's syndrome: a new case in the first decade of life. 778 21

We report on a 12-year-old patient with Turcot Syndrome (Glioma polyposis). This patient's case deals with the association between a glioblastoma, anaplastic glioma (WHO Grade III) and colonic adenocarcinoma based on familial polyposis coli. Possible etiology and neurosurgical, clinically important characteristics of this rare syndrome, such as the young age of the patient and the relatively long survival time, will be discussed.
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PMID:The Turcot syndrome (glioma polyposis) and its neurosurgical significance. Case report. 812 48

We reported a case of Turcot Syndrome (glioma polyposis) in a 19-year-old woman with nonfamilial polyposis coli and adenocarcinoma of the colon, and grade 3 astrocytoma in the right parietal lobe. The patient was admitted with the complaint of general convulsion after colostomy for polyposis and adenocarcinoma of the colon. CT scans on admission showed a large parietal tumor in the right side. Total removal was performed successfully and histological examination showed astrocytoma grade 3. One year after the operation, the tumor recurred. Conservative treatment failed to improve her condition and she died one year later. Turcot Syndrome (glioma polyposis) is very rare and only 10 cases have been reported in Japan. In this report, the clinical characteristics of this syndrome were discussed.
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PMID:[A case of Turcot syndrome (glioma polyposis)]. 838 50

We investigated clinical manifestations in 124 patients with a possible Turcot's syndrome whose data were taken from documented cases. The cases were subclassified mainly on the bases of the type of familial occurrence and listed in five Tables. We searched for differences in colonic manifestation, histologic type of glioma, mode of inheritance, frequency of parental consanguinity, skin lesions and other accompanying lesions among these five groups. The differences of these clinical findings suggested that glioma-polyposis syndrome should be classified as follows; (1) cases of Turcot's syndrome who had characteristic colonic and brain manifestations, (2) cases of FAP associated with glioma, (3) suspicious cases of glioma-polyposis, and (4) cases other than glioma-polyposis syndrome.
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PMID:Turcot's syndrome and familial adenomatous polyposis associated with brain tumor: review of related literature. 840 93

The term "Turcot's syndrome" has been used to describe approximatively 55 patients with an association of colonic polyposis and primary neuroepithelial tumors of the central nervous system. The p53 tumor suppressor gene is a possible candidate underlying the syndrome because (a) mutations in the p53 gene are ubiquitous in human cancer, including colon carcinoma and gliomas, and (b) somatic or germ line mutations of the p53 tumor suppressor gene cause the Li-Fraumeni syndrome, which is characterized by the association of breast and soft tissue tumors. We determined the DNA sequence of the conserved regions of the p53 gene (exons 5 to 9) in the tumor tissues and lymphocytes of two patients with glioma-polyposis and found that mutations did occur as independent tumor-specific alterations but did not involve the germ line of these patients, suggesting that p53 may play a role in progression but not initiation of the disease.
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PMID:Turcot's syndrome of glioma and polyposis occurs in the absence of germ line mutations of exons 5 to 9 of the p53 gene. 843 70


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