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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixty-two patients with tuberous sclerosis were studied by skull radiography. In addition, 16 had CT scanning, 26 had air studies, and 5 had cerebral angiography. Both subependymal and parenchymal tubers were shown on CT; all had a higher density than the brain and none were enhanced by contrast material. CT showed tubers in 13 of 16 patients, including 4 of 6 patients with glioma and hydrocephalus. Air studies showed subependymal tubers in 12 of 26 patients. Four other patients had intraventricular gliomas but no tubers. Skull radiographs showed spotty calcification, compatible with tuberous sclerosis, in 30 cases. Four patients had calcification compatible with tumor. Angiograms were nonspecific, showing intraventricular masses but no tubers.
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PMID:Tuberous sclerosis. Comparison of computed tomography and conventional neuroradiology. 64 65

A 6-year-old boy had a peripapillary lesion diagnosed as retinal astrocytic hamartoma, which was not associated with tuberous sclerosis, neurofibromatosis, or intraocular extension of a glioma. The patient was observed for nine years, during which time the lesion grew significantly. Because of this growth and the evidence of proximal optic nerve involvement on computed tomography, radiation therapy was administered with a resultant marked reduction in visual acuity. The eye was subsequently enucleated. The enucleated eye was studied by both light and transmission electron microscopy. The specimen had a retinal astrocytic hamartoma with sparse vascularization and only superficial optic nerve involvement. Additionally, it revealed optic nerve drusen in varying stages of development, significant accumulations of subretinal macrophages, and an almost total loss of retinal outer segments with the preservation of the retinal pigment epithelium.
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PMID:Astrocytic hamartoma of the retina not associated with tuberous sclerosis. 71 27

The familial occurrence of brain tumors are exceedingly rare except in cases with phacomatosis. We encountered pituitary adenomas in two sisters of a family, so far presenting no evidence of multiple endocrine adenomatosis (MEA). Case 1, K. O. a 26-year-old woman was admitted to our Hospital on September 10, 1970 with visual acuity and field disturbance, irregular menstruation and acromegaly. Neurological examination: Her visual acuity was Vd 0.6 and Vs 0.3, visual field was bitemporal hemianopsia, and ther was papilledema bilaterally. She had left exophthalmos and left abducens palsy. Roentgenogram of the skull, brain scanning, cerebral angiogram, pneumoencephalogram suggested the presence of a pituitary tumor. On Sep. 17, 1970, through a left frontotemporal craniotomy the tumor was removed subtotally. The pathological diagnosis was pituitary adenoma (chromophobe). Case 2, M. T. a 31-year-old woman, sister of case 1, was admitted to the Hospital on September 19, 1973, with mild headache, left visual field disturbance and amenorrhea. She had a child, and a past history of pulmonary tbc. Neurological examination: Her visual acuity was Vd 1.2 and Vs 0.03, and visual field of the right eye was temporal lower quandrant anopsia. There was optic nerve atrophy in the left eye. Plain X-ray craniogram, brain scanning, cerebral angiogram and pheumoencephalogram suggested the presence of a pituitary tumor. On Sep. 28, 1973, a right frontal craniotomy was performed. The tumor tissue with capsule was removed subtotally. The pathological diagnosis was pituitary adenoma (mixed type). In the literatures about familial brain tumors with histological diagnosis, glioma and glioblastoma are common, meningioma is relatively rare. Pituitary adenoma with no evidence of MEA is exceedingly rare. The two sisters presented in this paper, have no evidence of hyperparathyroidism, pancreas adenoma and peptic ulcer. So, we consider, at present, these cases should not be field in MEA.
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PMID:[Familial occurrence of pituitary adenoma (author's transl)]. 94 79

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

The pathological findings, including immunohistochemical and electron microscopical findings, in three infants who died unexpectedly of cardiac tumor or cardiomyopathy are reported. The first was a 13-month-old boy with tuberous sclerosis and multiple rhabdomyomas of the heart, who presented with a postpartal cardiac murmur and moderate cardiomegaly. The further history was unknown. The rhabdomyoma nodules were composed of spider cells containing small amounts of desmin and myosin as well as isolated myofibrils. Microscopically small glioma nodules contained high amounts of GFAP. The second case, a boy 4 months of age, died of a large benign fibrous histiocytoma of the heart after an uneventful history. Tumor cells contained alpha-1-anti-chymotrypsin and lysozyme. The third case, a girl 2 months of age, died unexpectedly of histiocytoid cardiomyopathy. The affected cells contained fat droplets, glycogen granules, many leptomer myofibrils and small amounts of myosin and desmin.
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PMID:Unexpected infant death attributable to cardiac tumor or cardiomyopathy. Immunohistochemical and electron microscopical findings in three cases. 216 15

The most common ocular and orbital tumors presenting in infancy, childhood and adolescence are presented and discussed in this review. It has been prepared specifically for the clinical pediatrician and focuses on the clinical recognition of ophthalmic neoplasms, their diagnostic evaluation employing the use of advanced imaging techniques, biopsy when indicated and extent of disease workup. In addition, current treatment modalities are discussed. Ocular tumors addressed include: retinoblastoma, capillary hemangioma, lymphangioma, dermoid and epidermoid cysts, teratoma, glioma, astrocytic hamartoma, neurofibroma, rhabdomyosarcoma and fibrous tumors. Two aggressive and potentially fatal tumors, rhabdomyosarcoma and retinoblastoma, are presented in detail. In addition, the ocular tumors associated with the phakomatoses (von Hippel-Lindau, tuberous sclerosis and neurofibromatosis) are reviewed.
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PMID:Ophthalmic neoplasms in infancy and childhood. 219 81

The authors report a rare case of tuberous sclerosis associated with pineal region mixed glioma. A 38-year-old woman with tuberous sclerosis, who had a past history of left nephrectomy and tumorectomy of the right kidney for bilateral renal angiomyolipomas, was admitted because of headache and ataxic gait. CT scan and cerebral angiography suggested a pineal region tumor. Suboccipital craniectomy and partial removal of the tumor was performed via infratentorial supracerebellar approach. Microscopic examination revealed mixed oligodendroglioma and astrocytoma. It is well known that tuberous sclerosis is occasionally associated with brain tumors. However, with few exceptions, these have been subependymal giant cell astrocytomas in the lateral ventricles. They seem to originate from subependymal nodules of hamartomatous nature. As far as we are aware, association of pineal region tumor with tuberous sclerosis as in this case has not been reported.
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PMID:[A case of tuberous sclerosis associated with a pineal region tumor]. 356 82

Gliomas of the CNS associated with tuberous sclerosis have been well documented; malignant degeneration to glioblastoma multiforme, however, is rare. We studied a 17-year-old boy with stigmata of tuberous sclerosis and a cerebral glioblastoma multiforme. The rarity of this occurrence suggests that neoplasms arising from hamartomas may behave differently than those CNS tumors that arise apparently de novo.
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PMID:Glioblastoma multiforme with tuberous sclerosis. Report of a case. 625 67

We studied two families in each of which three or more individuals were affected by brain tumors. In the first family, which had no evidence of neurofibromatosis or tuberous sclerosis, a man, his sister, and her son developed histologically proven gliomas; the man's great uncle was historically reported to have died from a brain tumor, but the exact nature of the tumor was not known. In this family two of the tumors were low grade astrocytomas of the cerebrum, whereas the third was a mixed glioma of the cerebellum. Karyotypic analysis of this tumor showed no marker chromosomes. A second family had a history of an unusual concentration of brain tumors. In one patient the tumor was a histologically verified glioma. Four other patients had historically reported brain tumors, the descriptions of which suggested gliomas. Both families showed involvement of individuals in adjacent generations, although in both instances there were skipped generations. Twins, siblings, or parents and children are the kindred groups affected in most other reported families with multiple brain tumors. The mode of inheritance of brain tumors in these two families and recent literature on the conditions associated with familial brain tumors are discussed.
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PMID:Familial brain tumors: studies of two families and review of recent literature. 629 52

Hereditary tumor syndromes of the nervous system are a varied group of conditions that include neurofibromatosis 1, neurofibromatosis 2, tuberous sclerosis, and von Hippel-Lindau disease, as well as the retinoblastoma susceptibility, Li-Fraumeni, familial glioma, Turcot, Gorlin, Cowden and multiple endocrine neoplasia syndromes. For many of these conditions, the responsible genes have been localized or identified. Such studies have elucidated the genetic basis of both hereditary cancer predisposition and sporadic nervous system tumors. The first four hereditary tumors syndromes have been extensively studied and are discussed in detail in the four subsequent articles. The other syndromes have also been subject to both pathological and molecular genetic inquiries. In this introductory overview, we discuss the features common to the hereditary tumor syndromes of the nervous system, and review some of the rarer conditions.
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PMID:Hereditary tumor syndromes of the nervous system: overview and rare syndromes. 767 Jun 55


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