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)

The four male patients with multiple hamartoma syndrome (Cowden's disease) in this report, have most of the previously reported findings associated with this syndrome and several important unreported findings that include multiple cutaneous trichilemmomas, cafe-au-lait spots, cutaneous squamous cell carcinoma, pathologic fracture, craniomegaly, probable malignant lung tumor, retinal glioma, drusens of the optic disk and retina, pseudotumor cerebri, mediastinal mass, and multiple small papillomatous lesions of the esophagus, stomach, and duodenum.
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PMID:Multiple hamartoma syndrome (Cowden's disease). 64 96

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

A 2 and a half year-old boy with neurofibromatosis developed unilateral proptosis, decreased visual acuity, and optic disk edema. After the discovery and removal of an optic nerve glioma, the patient had ten years of excellent health until he began having headaches, nausea, and vomiting. He had papilledema in his remaining eye. At exploration, a cerebellar astrocytoma and a neuroglial hamartoma were removed. The occurrence of a glioma of right anterior visual pathway associated with other primary intracranial lesions in patients with neurofibromatosis was not previously reported.
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PMID:Optic nerve glioma and cerebellar astrocytoma in a patient with von Recklinghausen's neurofibromatosis. 80 54

The consultants agreed that the differential diagnosis should focus on congenital masses, including an encephalocele, glioma, dermoid, hamartoma, hemangioma, rhabdomyosarcoma, neurofibroma, and nasolacrimal duct cyst. There was some disagreement as to which is the best way to evaluate the mass, ranging from an MRI (Dr. Reilly), to CT scan (Dr. Cotton), to both MRI and CT (Dr. Koopman). Blood tests to evaluate pituitary function could be indicated if there was a sphenoid defect (Dr. Reilly). None of the experts would biopsy this lesion. All would proceed with a definitive resection. One surgeon would defer surgery for several months and then perform the resection via a biocoronal craniotomy (Dr. Reilly). A combined anterior craniotomy and external ethmoidectomy would be planned by another (Dr. Koopman). The third consultant would combine an anterior craniotomy with a mid-face degloving, external rhinoplasty, or lateral rhinotomy approach (Dr. Cotton). Routine perioperative antibiotics would only be used by two of the surgeons (Drs. Reilly and Koopman). If a CSF leak were encountered there are several options. A small lesion could be allowed to close on its own (Dr. Reilly). If the leak occurred while the bicoronal incision was still open or if the leak were large, it could be repaired from above (Drs. Reilly and Koopman). One surgeon would proceed with a repair from above even if the leak were encountered during the intranasal approach (Dr. Cotton). Only one surgeon would restrict postoperative activity with intubation and sedation or paralysis (Dr. Koopman). Regarding follow-up, no one was concerned about the final pathology report.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nasal mass in a pediatric patient. 139 78

Multiplanar capability and superior tissue contrast differentiation render magnetic resonance (MR) imaging the preferred method for examining patients with pituitary axis dysfunction or visual field deficits. In a review of 131 sellar or juxtasellar abnormalities, 76% were common lesions with distinctive features that helped establish their diagnosis: macroadenoma (n = 51), microadenoma (n = 20), meningioma (n = 14), craniopharyngioma (n = 10), and aneurysm (n = 5). On T1-weighted images, microadenomas were usually hypointense relative to normal pituitary gland, and macroadenomas and meningiomas were isointense relative to gray matter. Both microadenomas and meningiomas were more conspicuous immediately after contrast material administration. Craniopharyngiomas were the most heterogeneous of all the sellar lesions due to their cystic and solid components. MR images of aneurysms showed flow void and heterogeneous increased signal intensity in areas of slower turbulent flow. Other characteristics such as extrasellar versus intrasellar location, nature of contrast material enhancement, the presence of cystic components, and clinical findings permitted differentiation among less common lesions, including granulomatous disease, metastases, chiasmatic glioma, arachnoid cyst, hypothalamic glioma, schwannoma, germinoma, epidermoid, Rathke cyst, chordoma, chondrosarcoma, colloid cyst, and hamartoma.
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PMID:MR imaging of the sellar and juxtasellar regions. 194 11

Magnetic resonance imaging has been used increasingly in the staging and evaluation of neoplasia of the brain and leptomeninges. In the classification of gliomas, the MR accuracy rate approaches that of pathologic diagnosis. Contrast-enhanced MR imaging has improved specificity in evaluating brain tumors in children and is now the preferred modality for evaluating leptomeningeal metastases of the brain and spine. MR imaging in children has also increased the specificity of histologic diagnosis in hypothalamic hamartoma and juvenile pilocytic astrocytoma. Gadolinium enhancement is most useful in patients older than 35 years of age who have focal neurologic complaints and certain disease histories. The expense of gadolinium contrast material is the major drawback to its routine use. In patients with seizure disorder, MR imaging is more sensitive than CT for detecting abnormalities such as mesial temporal sclerosis, tumors, and vascular malformations. Gadolinium enhancement may be useful in differentiating tumors from mesial temporal sclerosis. Recent reports on the use of MR spectroscopy for evaluating brain metabolism and tumors demonstrate that differences in metabolites exist. A correlation was found in epidermoid tumors between high signal on T1-weighted images and high lipid content, and several studies have shown a positive correlation between glioma grade and glycolytic activity as determined on 18F-2-fluoro-2-deoxy-D-glucose positron emission tomography.
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PMID:Imaging of central nervous system tumors. 202 6

A case of supratentorial hemangioblastoma combined with two hemangiomas of the liver without manifestation of von Hippel-Lindau disease is presented. The cerebral cystic hemangioblastoma was localized in the right parietal lobe and contained a calcified area but no visible mural solid nodules. The CT and MRT differential diagnosis (arachnoidal cyst, glioma, Echinococcosis, hamartoma and metastasis) is discussed, and the literature on the subject is reviewed.
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PMID:[A supratentorial hemangioblastoma]. 219 91

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

Light and electron microscopic studies in a case of nasal glioma revealed a tumor composed nearly entirely of astrocytes. No ganglion cells or neurons were present and the mass was not surrounded by a capsule reminiscent for meningeal tissue. The vasculature resembled dermal patterns rather than typical central nervous system. No bone defect or connection to endocerebral tissue could be detected in the present case. Nasal glioma represents an ectopic focus of astrocytes, comparable to a hamartoma, rather than a true tumor or a herniation of brain tissue.
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PMID:Nasal glioma. 709 50

The present review is based on the retrospective study of 124 children with precocious puberty, 92 girls and 32 boys. In girls, the analysis of the clinical initial presentation has shown that premature pubarche (n = 18), premature adrenarche (n = 2) or isolated menstruations (n = 3) must be ruled out, as these symptoms can remain isolated for more than a year. However, in most cases the presence of growth acceleration and vaginal estrogenisation was of major diagnostic value. Bone maturation, although generally accelerated, can be normal in recently developed puberty. Precocious puberty may proceed by steps, with complete disappearance of physical signs in the intervals. Organic causes were found in 31% of the girls, and 44% of the boys with some characteristic features as rapid progression, and elevated LH response to LRF stimulation. Main causes were glioma of the optic chiasma (n = 11), 3rd ventricule invasive tumors, hamartoma (n = 8). The latter should be looked for by a non invasive procedure as the CT scan. In girls, precocious puberty with very high circulating estrogen levels was observed as part of a McCune-Albright syndrome. As the effect of precocious puberty on the final adult height is variable, the evaluation of therapeutic results remains uncertain. Medroxyprogesterone as well cyproterone acetate have not been fully efficient in controlling bone maturation. More recently, and still controversial, the treatment with long acting LRF analogues might provide a more satisfactory statural prognosis.
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PMID:[Precocious puberty. Comment on the diagnostic conditions and etiological aspects]. 718 38


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