1/219. Clinical and radiographic response in three children with recurrent malignant cerebral tumors with high-dose tamoxifen.The purpose of this study was to deliver tamoxifen as antiangiogenic therapy to children with recurrent progressive malignant brain tumors. tamoxifen was administered orally in very high dosage to one child as monotherapy and to two children in combination with oral etoposide and dexamethasone. One boy was diagnosed with high-grade astrocytoma in the brain stem, one girl with anaplastic ependymoma of the fourth ventricule, and one girl with high-grade astrocytoma in the midbrain. Conventional treatment with multiple surgeries, first- and second-line chemotherapy, and external beam therapy had failed. Tumor reduction was seen in radiographic images together with clinical improvement in 2 children, and clinical and radiographic halting of tumor progression was demonstrated in the patient with anaplastic ependymoma. None of the patients developed complications from the treatment. Follow up of the patients ranged from 15 to 30 months with a mean of 17 months. These encouraging preliminary results suggest a potential for this type of therapy. More studies are needed to start clinical trials and prove that angiostatic activity may contribute to the therapeutic effect of antiestrogens in estrogen receptor-negative tumors.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
2/219. A case history of glioma progression.Low-grade diffuse astrocytomas have an intrinsic tendency for malignant progression but the factors determining the kinetics of this process are still poorly understood. We report here the case of a male patient who developed a fibrillary astrocytoma at the age of 33 years and who underwent six surgical interventions over a period of 17 years without radiotherapy or chemotherapy. The first three biopsies spanned a period of 11 years and led to the diagnosis of low-grade, diffuse astrocytoma (WHO grade II), with a growth fraction (MIB-1 labeling index) of 2.3-3.7%. The fourth to sixth biopsies showed histological features of anaplastic astrocytoma (WHO grade III), with growth fractions between 5.0 and 10.5%. The fraction of gemistocytic neoplastic astrocytes also increased, from 0.3% in the first biopsy to 17.5% in the last biopsy and preceded the increase in proliferative activity and transition to anaplastic astrocytoma. The fraction of tumor cells immunoreactive to BCL-2 increased from 0.3% to 8.2%. A p53 mutation in codon 273 (CGT-->TGT, Arg-->Cys) was identified in the first biopsy and persisted throughout the course of the disease. However, the fraction of cells with p53 protein accumulation increased significantly during progression, from 3.2% in the first biopsy to 13.7% in the last. The absence of additional genetic alterations (PTEN mutations, loss of chromosome 10 and 19q) may be responsible for the slow progression and lack of glioblastoma features even after a 17-year disease duration.- - - - - - - - - - ranking = 7keywords = progression (Clic here for more details about this article) |
3/219. Cytologic diagnosis of a solitary brain metastasis from papillary carcinoma of the thyroid. A case report.BACKGROUND: Papillary carcinoma of the thyroid metastasizes to the brain in rare instances. In published series and case reports of metastatic papillary thyroid carcinoma, diagnosis of central nervous system (CNS) metastases has been determined by histologic methods. We present a case of papillary carcinoma metastatic to brain diagnosed by cytologic methods. CASE: A 43-year-old female, initially diagnosed at age 12 with papillary carcinoma of the thyroid metastatic to regional lymph nodes and lung, presented with head aches of increasing frequency and severity. A computed tomography scan confirmed a 1-cm nodule in the right inferior frontal lobe of the brain. For clinical reasons, the patient was followed with serial imaging for five years. At age 48 there was significant progression of the CNS disease, and the patient underwent stereotactic biopsy with drainage of cyst fluid. Cytologic examination of the cyst fluid and immunocytochemical studies confirmed the typical features of papillary thyroid carcinoma, including papillary clusters of cells with finely granular chromatin, micronucleoli, nuclear grooves and an associated psammoma body. CONCLUSION: Neurocytology is a useful technique in the examination of cystic lesions of the brain and may be the sole technique for determination of diagnosis.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
4/219. Establishment of two glioma cell lines from two surgical specimens obtained at different times from the same individual.We established two glioma cell lines from two surgical specimens obtained at different times from the same patient. One (No. 9R), which was derived from the recurrent tumor (glioblastoma, grade IV), proliferated more rapidly in vitro than the other (No. 9) from the primary tumor (slightly anaplastic astrocytoma, grade II-III). No. 9R showed heterotransplantability in nude mice, whereas No. 9 did not. These findings indicate that No. 9R has a more aggressive or malignant nature than No. 9. Both cell lines showed homozygous deletion of the representative tumor suppressor p16 and p15 genes, but no p53 gene alteration. However, examination of the overall mRNA expression profile using a commercially available cDNA-spotted membrane revealed much higher expression levels of several mRNAs, at least, in No. 9R than in No. 9, although the relationship between these mRNAs and the growth potentials remained unknown. These two cell lines, derived from the same individual, with different proliferating potentials may be useful for studies on the molecular bases of glioma malignancy and progression.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
5/219. Mutations of the adenomatous polyposis coli and p53 genes in a child with Turcot's syndrome.Turcot's syndrome is a rare heritable complex that is characterized by an association between a primary neuroepithelial tumor of the central nervous system and multiple colonic polyps. The aim of this study was to analyze genetic alterations in a case of Turcot's syndrome in a 10.5-year-old boy in whom a colorectal tumor developed 3.5 years following astrocytoma. An APC germline non-sense mutation at codon 1284 leading to a truncated protein was identified, as was a somatic p53 mutation in the colorectal carcinoma in exon 7, codon 244. The latter was not identified in the primary astrocytoma. However, immunohistochemistry revealed high p53 protein expression in both tumors, suggesting an additional p53 mutation in the primary astrocytic tumor. The diverse p53 mutations observed in this unique syndrome in two different sites and stages of the disease may shed light on the multistep progression of the malignant events.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
6/219. Globular glioma of the tectum.Brainstem gliomas are a heterogeneous group of tumors whose prognosis and treatment depend not only on the histologic features but also on the location within the brainstem. magnetic resonance imaging allows the recognition of a distinct type of brainstem glioma of the tectal region of the midbrain, leading to aqueductal compression and hydrocephalus. The radiologic appearance of these tumors is usually rather uniform, with a characteristic nonenhancing thickening of the tectal plate. Because of its protracted course, no further treatment is necessary beyond cerebrospinal fluid diversion and close clinicoradiologic follow-up. The authors report two children with tectal plate gliomas of unusual but strikingly similar appearance. They present a clinical picture suggestive of intracranial hypertension without localizing signs. Magnetic resonance images reveal hydrocephalus related to the presence of perfectly circular lesions, hypointense on T1 and hyperintense on T2, which could be mistaken for parasitic cysts or represent dilated rostral portions of the sylvian aqueduct. After the cerebrospinal fluid diversion procedures, no further treatment was given, with one of the patients being monitored for 10 years and the other for 8 months, without tumor progression. These patients demonstrate that tectal gliomas, despite sharing a good prognosis, may have various patterns of growth, leading to unusual radiologic appearances that may pose diagnostic difficulties.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
7/219. Neurologic sequelae of treatment of primary CNS lymphomas.Novel efficient and aggressive treatment protocols for primary CNS lymphomas have resulted in an increasing number of long term survivors. Follow up data show that in a substantial fraction of these patients, treatment benefits are overshadowed by neurotoxic sequelae. Neurotoxicity especially affects the older age group, presenting as cognitive dysfunction, ataxia or dementia as a consequence of leukoencephalopathy and brain atrophy. The combination of radiotherapy and chemotherapy seems to be particularly hazardous, though data are too sparse to draw any definite conclusions yet. Long term follow up of patients included in clinical studies therefore should not only evaluate survival or time to tumour progression, but also serial neuropsychometric evaluation and quality of life assessment.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
8/219. Intact sensory fusion in a child with divergence paresis caused by a pontine glioma.PURPOSE: To describe a child with divergence paresis esotropia caused by a brain tumor with intact sensory and motor fusion. METHOD: Case report. RESULTS: A 9-year-old boy who had one episode of double vision was initially seen with a small, variably present esophoria at near vision, an intermittent 10 prism diopter esotropia at distance, and stereopsis of 80 arc seconds. A magnetic resonance imaging examination disclosed a 4.0 x 4.5-cm pontine glioma. CONCLUSIONS: Ophthalmologists should recognize that the presence of intact sensory and motor fusion in a child with acute, comitant esotropia of the divergence paresis type does not preclude intracranial abnormality. If immediate neuroimaging is deferred, repeated thorough ocular motility examinations are warranted to detect progression.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
9/219. Loss of tissue immunoreactive androgen receptor in prostate cancer presenting initially as an intracranial tumor.We report a case of prostate cancer that presented initially as an intracranial tumor. biopsy specimens of the prostate before endocrine treatment were nearly negative immunohistochemically for prostate-specific antigen and the androgen receptors. All metastases including those in the brain expressed neither androgen receptor nor prostate-specific antigen at the protein and mRNA levels. The tumor, which did not respond to the anti-androgen therapy, had an aggressive course. In this case, the androgen-independent growth and rapid progression might be associated with the initial loss of the antigen and androgen characteristics of the prostate. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
10/219. Histologically repeatedly confirmed gliosarcoma with long survival: review of the literature and report of a case.OBJECTIVE AND IMPORTANCE: A rare case of gliosarcoma in a 61-year-old woman is presented with a stable situation over 22 years with an excellent quality of life. CLINICAL PRESENTATION: The patient was initially symptomatic and was operated on in 1975 for a deep-seated left parietal gliosarcoma. During the following 20 years, she was clinically asymptomatic until she complained of increasing headache in 1995. Neuroradiological imaging showed a sharply demarcated lesion on MRI at the former operative site, which was operated on again. Four months later, the residual tumour did grow again. INTERVENTION: As radiation therapy could not stop tumour progression and the neurological status worsened, the patient was operated on again for a massive tumour mass in the left parieto-occipital region, filling out nearly all of the previous resection cavity. Despite radio-immunotherapy, the patient finally died 22 years after the first discovery of the tumour. CONCLUSION: The present case shows that, in rare instances, gliosarcomas may show prolonged survival, although the underlying pathogenetic mechanisms for this clinical behaviour are not understood.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
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