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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Angiofibromas from two patients with tuberous sclerosis were studied by light and electron microscopy. Light microscopy revealed that these tumor-like nodules (which in the past have been called adenoma sebaceum) were made up of dilated capillaries, venules and arterioles embedded in connective tissue. At the ultrastructural level the arterioles embedded in connective tissue. At the ultrastructural level the endothelium of these vessels showed large numbers of microvilli on their luminal surface. The stroma contained many banded structures (so-called fibrous long spacing collagen). Myofibroblasts recently described in juvenile nasopharyngeal angiofibromas were not found in these angiofibromas of tuberous sclerosis.
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PMID:Angiofibromas in tuberous sclerosis: a light and electron microscopic study. 22 82

A progressive facial paralysis in a child of 14 years allowed the diagnosis of a hamartoma to be made. The discovery of such a benign lesion should always be followed by a search for other lesions elsewhere, i.e. to see if there is not a phacomatosis. Persistent sciatic pains which this child now has lead one to fear a second lesion, compressing a nerve root or trunk of the sciatic nerve. There is a strong possibility that this tumor of the auditory canal is only the first manifestation of a neurocristopathy.
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PMID:[Hamartoma of the internal auditory canal and cerebropontile space in a child. Trans-temporo-extradural excision methods]. 31 71

Concerning the clinical signs and symptoms, we noticed skin white macula in 87%, adenoma sebaceum in 53%, mental retardation in 75%, retinal phacoma in 54% and seizure in 92%. Numbers of nodules ranged from zero to 11, and showed no correlation with aging. Half of the cases showed slight ventricular dilatation, one quarter showed moderate dilatation and the remainder were normal. There was no definite correspondence between size of the ventricle and number of nodules. There were relatively many cases with normal mental state in the group with normal ventricles. In adult cases a large ventricle does not always mean poor prognosis in mental state. Almost all of the cases with cortical atrophy had mental retardation. In the group with moderate dilatation infantile spasms were the most frequent type of seizure. On the contrary, grand mal was most frequent in the normal group. As for the position of nodules there was no difference between the normal and dilated groups. Nodules were predominantly seen in the lateral aspect of the body, trigone of the lateral ventricle and adjacent to the foramen of Monro. Asymmetrical lateral ventricles were noticed in 18%. Incidence of laterality increased as dilatation proceeded. It was larger on the left side in 9 out of 11 cases. We have no reasonable explanation of this tendency. There was one case with a tumor which was verified by enhanced CT. There were five cases with brain anomalies.
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PMID:Computed tomography in tuberous sclerosis--with special reference to relation between clinical manifestations and CT findings. 31 69

A 13-year-old boy presented with an obstructive left lateral intraventricular mass. Pathological examination revealed a subependymal giant-cell astrocytoma. Further clinical investigation confirmed the diagnosis of tuberous sclerosis. Ultrastructural examination confirmed previously reported features of this tumor. Tumor cells containing large dense granulated bodies and dense-core vesicles, not previously described in this tumor, were identified.
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PMID:Subependymal giant-cell astrocytoma. Case report with ultrastructural study. 43 Jan 39

In a small number of cases of tuberose sclerosis, tumors develop in the cerebral subependymal region. Their exact nature has been the subject of debate. The cytology, histology and electron microscopy of a tumor which developed in a 16 year old male suffering from tuberose sclerosis are presented and the findings are discussed.
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PMID:Subependymal giant cell tumor of tuberose sclerosis. A light and ultrastructural study. 44 1

The vlue of epinephrine enhanced angiography in the preoperative distinction of isolated hamartoma (angiomyolipoma without stigma of tuberous sclerosis) from hypernephroma has been largely discounted in the past. The authors performed this procedure in 6 patients (4 with isolated hamartoma and 2 with tuberous sclerosis complex). Vasoconstriction of the tumor vessels in the isolated group suggested the benign nature of the hypervascular mass in all cases. Vasoconstriction stronger than that of normal parenchymal vessels suggested the diagnosis of isolated renal angiomyolipoma. This form of pharmacoangiography should be part of the routine preoperative workup of all solid renal masses.
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PMID:Epinephrine enhanced renal angiography in the diagnosis of hamartoma (angiomyolipoma): a reevaluation. 45 Dec 20

Two patients with tuberous sclerosis are described in whom computed tomography of the head revealed areas of diffuse diminution of density suggestive of demyelination. Computed tomography is a sensitive test for the detection of intracerebral lesions in tuberous sclerosis, and the finding of areas of diminished density in the absence of evidence for tumor or ventricular obstruction correlates with the common histologic finding of diffuse demyelination. Serial studies with computed tomography are likely to determine whether such demyelination precedes the growth of hamartomas and calcification that characterize tuberous sclerosis.
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PMID:Demyelination of the brain in tuberous sclerosis: computed tomography evidence. 48 Sep 92

The authors studied 9 girls and 5 boys, with ages ranging from 9 months to 26 years, suffering from Bournevilles tuberous sclerosis, for an average period of 7 years. The various types of epileptic attacks and their progression were studied as well as the degree of mental retardation. Only two of the children had a normal IQ. The accepted value of computer tomography examinations in this disease is confirmed. Pathological examinations were conducted in two cases, one complete (autopsy) and one partially only (histological examination of a paraventricular tumor removed by operation).
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PMID:[A study of 14 cases of Bourneville's tuberous sclerosis, including two pathological reports and seven cases investigated by computer tomography (author's transl)]. 53 86

Early clinical manifestations and radiologic features of tuberous sclerosis were studied in 18 consecutive patients encountered in the past two years, including two patients harboring an associated intraventricular tumor (giant-cell astrocytoma). Depigmented naevi rather than adenoma sebaceum, infantile spasms and intracranial calcifications were the cardinal early features in this present series, and CT scanning proved to be the single most useful diagnostic technique in the early detection of intracranial calcifications and therefore for the early diagnosis of this disorder. CT scanning was also useful in detecting the intraventricular tumors in the early stage before they became clinically manifest.
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PMID:Tuberous sclerosis: early neurologic manifestations and CT features in 18 patients. 55 37

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


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