Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Central neurocytoma is a low-grade neuronal neoplasm that occurs most often within the lateral ventricles. We report the case of a 60-year-old woman who presented with gait problems, headache and memory loss. Preoperative evaluation demonstrated a heterogeneous, hypervascular and partially cystic mass in the left lateral ventricle. Histopathological examination revealed characteristic features of central neurocytoma, including immunoreactivity for synaptophysin, as well as the unusual feature of abundant pigment in the cytoplasm of tumor cells. Special stains revealed iron, consistent with hemosiderin, but found no evidence of melanin or melanosomes. Previous reports of pigmented central neurocytoma have described the presence of lipofuscin or neuromelanin. To our knowledge, the present case represents the first example of pigmented central neurocytoma secondary to hemosiderin deposition.
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PMID:Pigmented central neurocytoma. 1499 86

This case study describes the long-term course of behavioral alterations and MRI findings in a patient with a combined limbic and cerebellar paraneoplastic syndrome, which was associated with a squamous lung carcinoma. Clinical equivalents were cerebellar ataxia, as well as severe anterograde memory loss, frontal executive dysfunction and behavioral alterations. MRI revealed inflammatory changes followed by progressive atrophy affecting the cerebellum and both temporal lobes. Tumor surgery yielded only a partial and transient recovery of neurological symptoms, and paraneoplastic atrophy continued to progress despite radical excision of the carcinoma. This case of paraneoplastic encephalitis suggests that the related atrophy may present as a chronic, progressive, multifocal encephalopathy and that the associated cognitive impairments may include several cognitive domains.
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PMID:Long-term cognitive and MRI findings in a patient with paraneoplastic limbic encephalitis. 1501 90

Alzheimer's disease (AD) characteristically presents with early memory loss. Regulation of K(+) channels, calcium homeostasis, and protein kinase C (PKC) activation are molecular events that have been implicated during associative memory which are also altered or defective in AD. PKC is also involved in the processing of the amyloid precursor protein (APP), a central element in AD pathophysiology. In previous studies, we demonstrated that benzolactam (BL), a novel PKC activator, reversed K(+) channels defects and enhanced secretion of APP alpha in AD cells. In this study we present data showing that another PKC activator, bryostatin 1, at subnanomolar concentrations dramatically enhances the secretion of the alpha-secretase product sAPP alpha in fibroblasts from AD patients. We also show that BL significantly increased the amount of sAPP alpha and reduced A beta 40 in the brains of APP[V717I] transgenic mice. In a more recently developed AD double-transgenic mouse, bryostatin was effective in reducing both brain A beta 40 and A beta 42. In addition, bryostatin ameliorated the rate of premature death and improved behavioral outcomes. Collectively, these data corroborate PKC and its activation as a potentially important means of ameliorating AD pathophysiology and perhaps cognitive impairment, thus offering a promising target for drug development. Because bryostatin 1 is devoid of tumor-promoting activity and is undergoing numerous clinical studies for cancer treatment in humans, it might be readily tested in patients as a potential therapeutic agent for Alzheimer's disease.
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PMID:Therapeutic effects of PKC activators in Alzheimer's disease transgenic mice. 1526 77

Limbic encephalitis was identified as a clinicopathological entity in 1968. Up to a few years ago, 200 cases were described, most associated with lung cancer and more infrequently with other tumors. The recent identification of patients with this syndrome, idiopathic limbic encephalitis, who never develop cancer and have high titers of antibodies to voltage-gated potassium channels (VGKC) and an excellent response to immunosuppressive therapy, has extended the etiological spectrum and suggests that the syndrome may be under-recognized. The disorder, which develops in a few days or weeks, is characterized by the development of short-term memory loss, seizures, confusion and psychiatric features. The presence of symptoms beyond the limbic system is highly suggestive of a paraneoplastic origin. When limbic encephalitis is suspected, the following tests should be performed in order to demonstrate: a) involvement of the temporal lobes (EEG and brain MRI); b) presence of inflammatory abnormalities in the CSF, and c) the presence of onconeural antibodies or anti-VGKC. Once the diagnosis is confirmed by the clinical picture and MRI findings, treatment must be initiated without waiting for the antibody results because its negativity does not exclude the diagnosis. Detection of an onconeural antibody will confirm that the limbic syndrome is paraneoplastic and will help us to search for an underlying tumor and to predict possible response to the treatment. The recommended treatment is cycles of methylprednisolone (1 g/day for 3 to 5 days). Therapeutic response in the idiopathic limbic encephalitis is excellent and may be good in limbic encephalitis with anti-Ma2 or without onconeural antibodies. On the contrary, immunosuppressant treatment is not usually effective in limbic encephalitis associated to anti-Hu antibodies.
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PMID:[Limbic encephalitis: a probably under-recognized syndrome]. 1570 18

The diagnosis of Gliomatosis cerebri (GC) is known to be difficult and is still a matter of debate. We describe an in vivo case of GC associated with a pituitary tumor. A 47-year-old woman presented with short-term memory loss. A MRI revealed the presence of a pituitary enhancing tumor and a diffuse lesion involving the brain. A left pterional craniotomy with partial temporal lobectomy and removal of the pituitary lesion were performed in order to obtain diagnosis. The histological analyses showed a pituitary non-functioning tumor and a GC consisting of neoplastic oligodendrocytes and astrocytes. Both lesions showed nuclear immunoreactivity for progesterone receptors (PGr) and estrogen receptors (EGr). This result could suggest there is a common receptor substrate in these tumors. In this case hormones could constitute a common step in tumorigenesis of both lesions.
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PMID:Gliomatosis cerebri and pituitary adenoma: case report and literature review. 1613 22

Memory loss can be a symptom of paraneoplastic limbic encephalitis (PLE) a neuropsychiatric disorder associated mostly with small-cell lung cancer and anti-Hu antibodies or with testicular tumors and anti-Ma2 antibodies. We present the case of a patient with temporal coincidence of beginning cognitive decline and diagnosis of a carcinoma of the prostate in whom we diagnosed anti-Ma1/Ma2-positive PLE. The tumor had been completely resected but memory impairment further deteriorated. As the effective treatment of the cancer is considered as the most efficient treatment of a paraneoplastic neurological syndrome (PNS) a second neoplasia was suspected in the patient. By the aid of whole body positron emission tomography with 18-fluorine fluoro-2-deoxy-glucose (FDG-PET) an adenocarcinoma of the cecum could be detected. Two months after surgery anti-Ma antibodies were negative. We conclude that a second neoplasia should be considered, if effective cancer treatment does not lead to improvement or stabilisation of a PNS. Tumor search should be exhaustive and include PET when conventional imaging fails to show a malignancy.
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PMID:Second primary tumor in anti-Ma1/2-positive paraneoplastic limbic encephalitis. 1631 42

Object. The purpose of this study was to determine the efficacy of endoscopic treatment in patients with intraventricular tumors. Methods. A series of 30 patients with endoscopically treated intraventricular lesions is reported. The lesions included seven colloid cysts, six astrocytomas, three subependymomas, two ependymomas, and one each of the following: pineoblastoma, pineocytoma/pineoblastoma (intermediate type), epidermoid cyst, pineal cyst, medulloblastoma, arteriovenous hemangioma, cavernoma, choroid plexus papilloma, pituitary adenoma, craniopharyngioma, melanoma, and germinoma. Total tumor resections, partial resections, biopsies, stent implantations, septostomies, and third ventriculostomies were performed. In two cases (two subependymomas > 2 cm in diameter), piecemeal endoscopic resection was ineffective because of the very firm consistency of the tumors. Therefore the endoscopic procedure was discontinued and the tumors were removed microsurgically. In the remaining cases the procedures were completed as planned. Even in the presence of difficulties such as poor orientation or significant bleeding, there was no need to abandon the endoscopic procedure. A total of 28 strictly endoscopic interventions were performed, in which the average duration was 85 minutes (range 35-170 minutes). All colloid cysts and the epidermoid lesion were completely evacuated and the capsules were widely resected. Total extirpation of solid tumors was achieved in five cases, whereas most astrocytomas were partially resected. The hydrocephalus-related symptoms resolved in all of the 22 patients with cerebrospinal fluid pathway obstruction. There were no endoscopy-related deaths. In two cases, major bleeding occurred and was controlled endoscopically. The authors observed one case of meningitis, one of mutism, two of memory loss attributed to forniceal injury, one of transient trochlear palsy after a biopsy specimen of an aqueductal tumor was obtained, and one of transient confusion after a biopsy specimen of a germinoma was obtained. Conclusions. In the authors' preliminary experience, the endoscopic approach was found to be safe and effective. In this series, it was possible to achieve relief of noncommunicating hydrocephalus, tumor resections, and even complete tumor removals by using endoscopic techniques. Based on the results, the authors believe that endoscopic techniques should be considered in the treatment of selected intraventricular lesions.
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PMID:Neuroendoscopic approach to intraventricular lesions. 1668 59

Several types of mass lesions may occur in the third and lateral ventricles. Typically they arise from the lining of the ventricular cavity or from contiguous structures, by extension into the ventricle. The authors describe two patients, each of whom presented with a different rare lesion of the ventricular system. The first was a 53-year-old woman with a history of hypertension who sustained a blunt traumatic injury to the occipital region and subsequently developed a progressively worsening right-sided headache. Radiological examinations over the next 2 years revealed an enlarged right lateral ventricle and, ultimately, a choroid plexus cyst in its anterior and middle third, near the foramen of Monro, which is a rare location for these lesions. The cyst was removed en bloc, and follow-up examinations showed a significant improvement in her headache and a minimal differences in size between right and left ventricles. The authors also describe a 57-year-old man with hypertension, diabetes mellitus, and an old mycardial infarct, who presented to an outside institution with a progressively worsening headache, generalized malaise, and loss of olfactory sensation. Diagnostic imaging revealed a 1.5-cm oval lesion centered in the lamina terminalis region, an open craniotomy was performed, and evaluation of a biopsy sample demonstrated the mass to be a chordoid glioma of the third ventricle, a recently described glioma subtype. Two days after surgery, he suffered a left parietal stroke and an anterior mycardial infarction. After convalescing, he presented to The University of Texas M. D. Anderson Cancer Center for radiotherapy and follow up; 7 months later he was readmitted complaining of headache, short-term memory loss, and worsening confusion and disorientation. Neuroimaging revealed progression of the tumor (now 2 cm in diameter), which was removed by gross-total resection. His headache resolved immediately, and 2 months later his only complaint was of episodes of confusion. Three weeks later he died of a massive myocardial infarction. These two patients represent the sixth case of an adult with a choroid plexus cyst in the anterior lateral ventricle and the 19th case of an adult with a chordoid glioma of the third ventricle, respectively.
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PMID:Choroid plexus cyst and chordoid glioma. Report of two cases. 1672 23

Paraneoplastic limbic encephalitis is a syndrome characterised by behaviour changes, short-term memory loss and seizures. We report on a case of a 62-year-old man, ex-smoker who was admitted to the Intensive Care Unit with seizures, and who had previously experienced behaviour changes. Tests revealed nothing notable, except for the CSF examination which revealed elevated protein and MRI findings indicating temporal lobe abnormalities. Once other diagnoses had been ruled out, neoplasm screening was initiated. Conventional imaging failed to identify the tumour. Antineuronal anti-Hu antibodies were negative. Finally, fluorodeoxyglucose (FDG) whole body -PET imaging was carried out revealing a pathologically increased glucose metabolism at the pharynx-larynx level. The biopsy showed an epidermoid carcinoma of the pyriform sinus. To the best of our knowledge, this is the first reported case of an epidermoid carcinoma of the pyriform sinus associated with paraneoplastic limbic encephalitis.
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PMID:[Paraneoplastic limbic encephalitis and epidermoid carcinoma of the pyriform]. 1706 34

Adriamycin (ADR), a potent anti-tumor agent, produces reactive oxygen species (ROS) in cardiac tissue. Treatment with ADR is dose-limited by cardiotoxicity. However, the effect of ADR in the other tissues, including the brain, is unclear because ADR does not pass the blood-brain barrier. Some cancer patients receiving ADR treatment develop a transient memory loss, inability to handle complex tasks etc., often referred to by patients as chemobrain. We previously demonstrated that ADR causes CNS toxicity, in part, via systemic release of cytokines and subsequent generation of reactive oxygen and nitrogen species (RONS) in the brain. Here, we demonstrate that treatment with ADR led to an increased circulating level of tumor necrosis factor-alpha in wild-type mice and in mice deficient in the inducible form of nitric oxide (iNOSKO). However, the decline in mitochondrial respiration and mitochondrial protein nitration after ADR treatment was observed only in wild-type mice, not in the iNOSKO mice. Importantly, the activity of a major mitochondrial antioxidant enzyme, manganese superoxide dismutase (MnSOD), was reduced and the protein was nitrated. Together, these results suggest that NO is an important mediator, coupling the effect of ADR with cytokine production and subsequent activation of iNOS expression. We also identified the mitochondrion as an important target of ADR-induced NO-mediated CNS injury.
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PMID:Adriamycin-mediated nitration of manganese superoxide dismutase in the central nervous system: insight into the mechanism of chemobrain. 1722 39


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