Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirteen patients with schwannomas of the jugular foramen were operated on at the Cleveland Clinic between 1974 and 1983. The authors' experience in managing these rare tumors is presented. Three major growth patterns of jugular foramen schwannoma were seen, and it is postulated that the position of the tumor depends on its point of origin from the nerves as they pass through the pars nervosa of the jugular foramen. The more distal lesions will expand inferiorly out of the base of the skull, and the more proximal lesions will enlarge into the posterior fossa. Tumors in the mid region will tend to expand primarily into bone. The schwannoma was primarily intracranial in six patients. In five patients the tumor expanded the bone at the base of the skull, with only a small intracranial component, and in two patients the tumor was primarily extracranial, with a small extension into the bone or posterior fossa. The presentation of the patients varied according to the tumor growth pattern. Deafness, vertigo, and ataxia were present in all patients with a major intracranial component, and in most of these there were only minimal deficits of the jugular foramen nerves. By contrast, lower cranial nerve involvement, including hoarseness and weakness of the trapezius and sternocleidomastoid muscles, occurred in patients in whom the tumor was primarily, within the bone or extracranial. Three of the five patients with the major component of the schwannoma within the bone also had deafness. Symptomatic history was longest in those with tumor mainly involving the bone at the base of the skull, and shortest in patients with entirely extracranial tumor. Surgical resection was accomplished with a joint neurosurgical-otological approach, usually combining a posterior fossa exploration with either a translabyrinthine transcochlear or infralabyrinthine procedure. The exact nature of the operation depended upon the presence of intracranial tumor and on the extent of bone or extracranial involvement. Total excision was performed in all cases. There was no operative mortality, and surgery resulted in loss of function of the ninth, 10th, and 11th cranial nerves in most patients. The major postoperative morbidity consisted of swallowing difficulties and sputum aspiration.
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PMID:Jugular foramen schwannomas. 671 39

Antiangiogenic therapy is a promising new strategy to inhibit tumor growth and formation of metastases. Vascular endothelial growth factor (VEGF) and its receptors, VEGF-receptor 1 (VEGF-R1; FLT-1) and VEGF-R2 (KDR), have been shown to play a major role in tumor angiogenesis. PTK787/ZK 222584, a specific inhibitor of both VEGF-receptor tyrosine kinases, was investigated for its antitumoral and antiangiogenic activity in a murine renal cell carcinoma model. After intrarenal application of the renal carcinoma cells, mice develop a primary tumor and metastases to the lung and to the abdominal lymph nodes. Daily oral therapy with PTK787/ZK 222584 at a dose of 50 mg/kg resulted in a significant decrease of 61 and 67% in primary tumors after 14 and 21 days, respectively. The occurrence of lung metastases was significantly inhibited at both time points (98% reduction and 78% reduction, respectively). After 14 days, no lymph node metastases developed in the PTK787/ZK 222584-treated group, whereas after 21 days of treatment, the lymph node metastases were reduced by 87%. Vessel density in tumor tissues, detected by immunohistochemistry with an anti-CD31 antibody, was significantly decreased by PTK787/ZK 222584. Using color Doppler imaging ultrasound, significant changes in blood flow in the tumor feeding renal artery were found under treatment with PTK787/ZK 222584. Blood flow changes correlated with changes in vessel density but not with tumor volume. The compound was well tolerated in all in vivo experiments and had no significant effects on body weight or general well-being of the animals. This was in contrast to the animals treated with the antiangiogenic agent TNP-470. s.c. therapy with 30 mg/kg TNP-470 every other day had to be discontinued after 13 days because of animal weight loss (>20%) and ataxia. These results demonstrate that PTK787/ZK 222584 is a potent inhibitor of tumor growth, metastases formation, and tumor vascularization in murine renal cell carcinoma. Furthermore, we have been able to demonstrate that color Doppler imaging ultrasound can be used to measure blood flow to a tumor and that flow correlates with vessel density. Thus, this may be a valuable noninvasive method for monitoring the effects of antiangiogenic agents such as PTK787/ZK 222584 on tumor vasculature.
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PMID:Effects of PTK787/ZK 222584, a specific inhibitor of vascular endothelial growth factor receptor tyrosine kinases, on primary tumor, metastasis, vessel density, and blood flow in a murine renal cell carcinoma model. 1098 92

Medical decision-making is based on benefit-to-cost analysis. Optimally, treatment obtains a high degree of benefit while minimizing the physical, social, and financial costs. The goals of the treatment of acoustic schwannomas are prohibiting tumor growth and alleviation of symptoms caused by damage to local structures. These symptoms-tinnitus, ataxia, and hearing loss--secondary to eighth nerve dysfunction, as well as symptoms arising from damage to adjacent structures such as the facial nerve, trigeminal nerve, or pons, can be caused by tumor growth or treatment. Determination of optimal therapy must also take into account an understanding of the natural history of the disease, because acoustic schwannomas are slow-growing benign tumors that when left untreated, usually enlarge over time and cause problems.
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PMID:Stereotactic radiation techniques in the treatment of acoustic schwannomas. 1754 96

Bajiaolian (Dysosma pleianthum), a species in the Mayapple family (Podophyllum pelatum), has been widely used as a traditional Chinese herbal medication for the remedies of snake bite, tumor growth, post-partum recovery, and acne. It has also been used in western medicine, especially topically for various skin lesions. Both oral ingestion and dermal application may result in severe toxicity. The clinical presentations reported after Bajiaolian poisoning include nausea, vomiting, diarrhea, abdominal cramps, tachycardia, orthostatic hypotension, paralytic ileus, urinary retention, hepatorenal dysfunction, leukocytosis followed by leukopenia, thrombocytopenia, prolonged areflexia, prolonged paraethesia and sensory ataxia, dizziness, fever, memory impairment, hallucinations, paranoia, convulsion, fainting, and coma. There are no previous reports in the literature about the cessation of nail growth as a clinical presentation following Bajiaolian poisoning. We present a case of nail growth that was halted for more than seven years after a single case of Bajiaolian poisoning.
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PMID:Cessation of nail growth following Bajiaolian intoxication. 1785 56

Medical decision-making is based on benefit-to-cost analysis. Optimally, treatment obtains a high degree of benefit while minimizing the physical, social, and financial costs. The goals of the treatment of acoustic schwannomas are prohibiting tumor growth and alleviation of symptoms caused by damage to local structures. These symptoms-tinnitus, ataxia, and hearing loss-secondary to eighth nerve dysfunction, as well as symptoms arising from damage to adjacent structures such as the facial nerve, trigeminal nerve, or pons, can be caused by tumor growth or treatment. Determination of optimal therapy must also take into account an understanding of the natural history of the disease, because acoustic schwannomas are slow-growing benign tumors that when left untreated, usually enlarge over time and cause problems.
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PMID:Stereotactic radiation techniques in the treatment of acoustic schwannomas. 2007. 1853 45

BACKGROUND: The simultaneous occurrence of cerebellopontine angle (CPA) meningioma and vestibular schwannoma (VS) in the absence of neurofibromatosis type 2 or history of irradiation is very rare. We report a case with coexistent CPA meningioma and VS, which were radiologically not distinguishable in preoperative imaging. CASE DESCRIPTION: A 46-year-old female presented with acute hearing loss, tinnitus and gait ataxia. Otorhinolaryngological diagnostic workup and imaging studies showed an intra- and extrameatal homogenous contrast enhancing lesion. The neuroradiological diagnosis was VS. The patient was operated via the retrosigmoid approach. INTRAOPERATIVELY TWO DISTINCT TUMORS WERE FOUND: a small, mainly intrameatally located VS and a larger meningioma originating from the dura of the petrous bone. Both tumors were completely microsurgically removed. The patient experienced no new neurological deficit after surgery; particularly facial nerve function was completely preserved. Histopathological examination revealed a fibromatous meningioma and a VS, respectively. CONCLUSIONS: The coincidental occurrence of CPA meningioma and VS is very rare. Careful interpretation of imaging studies before surgery is crucial. Even such rare cases should be kept in mind when discussing the therapeutic options with the patient. More studies are needed for a better understanding of mechanisms leading to multiple tumor growth.
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PMID:A Rare Case of Radiologically Not Distinguishable Coexistent Meningioma and Vestibular Schwannoma in the Cerebellopontine Angle - Case Report and Literature Review. 2104 38

The aim of the present study was to discuss the clinical features of intracranial vestibular schwannomas and to evaluate the symptoms and signs as well as their correlation with tumor extension. The records of 1,009 patients who were treated in Shanghai Huashan Hospital were reviewed retrospectively. According to the Samii classification of 1997, the patients were grouped into a T3 and a T4 group based on the radiological findings. We focused our analysis on the incidence of subjective disturbances versus objective morbidity, and symptomatology versus tumor size and extension. Of the 1,009 cases, 424 patients (42.0%) were defined as T3 while 585 patients (58%) were defined as T4. The most frequent clinical symptoms were hearing loss (85.8%), facial numbness (48.9%), ataxia (44.6%), tinnitus (40.1%), deafness (26.3%) and facial paralysis (21.1%). The ratios of gender, vertigo and facial paralysis were significantly different between the T3 and T4 groups (P<0.05); however, none of the clinical symptoms had a positive likelihood ratio (PLR) greater than 10 for T4 prediction. The most frequent cranial nerve disturbance was associated with the cochlear nerve (92.6%) and trigeminal nerve (53.5%). Disturbance of the facial nerve was more severe in T3 than T4 patients (P<0.05). Hearing deficit, facial paresthesia, ataxia and tinnitus are key symptoms of huge vestibular schwannomas. Cochlear, trigeminal and facial nerves were the most commonly affected cranial nerves in cases of large tumors. Gender and tumor size were associated with tumor extension. Although the predictive value was limited, the symptoms of vertigo, facial paralysis and hearing loss may be indicators of tumor growth.
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PMID:Clinical features of intracranial vestibular schwannomas. 2325 94

We present the case of a 30-year-old male patient with an almost complete destruction of the calvarial bone through an anaplastic meningioma diagnosed in line with dizziness. Neuroimaging revealed an extensive growing, contrast enhancing lesion expanding at the supra- and infratentorial convexity, infiltrating and destroying large parts of the skull, and infiltrating the skin. Due to progressive ataxia and dysarthria with proven tumor growth in the posterior fossa in the continuing course, parts of the tumor were resected. A surgical procedure with the aim of complete tumor resection in a curative manner was not possible. Six months after the first operation, due to a new tumor progression, most extensive tumor resection was performed. Due to the aggressive and destructive growth with a high rate of recurrence and tendency of metastases, anaplastic meningiomas can be termed as malignant tumors. The extrinsic growth masks the tumor until they reach a size, which makes these tumors almost unresectable. In the best case scenarios, the five-year survival is about 50%. With the presented case, we would like to show the aggressive behavior of anaplastic meningiomas in a very illustrative way. Chemotherapy, radiotherapy, and surgery reach their limits in this tumor entity.
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PMID:Extensive growth of an anaplastic meningioma. 2428 34

Quercetin, a natural polyphenolic flavonoid compound, can inhibit the growth of several malignant cancers. However, the mechanism still remains unclear. Our previous findings have suggested that quercetin can significantly inhibit HepG2 cell proliferation and induce cell apoptosis in vitro. It can also affect cell cycle distribution and significantly decrease cyclin D1 expression. In this study, we investigated the anti-cancer effect of quercetin on HepG2 tumor-bearing nude mice and its effect on cyclin D1 expression in the tumor tissue. First, the nude murine tumor model was established by subcutaneous inoculation of HepG2 cells, then quercetin was administered intraperitoneally, and the mice injected with saline solution were used as controls. The daily behavior of the tumor-bearing mice was observed and differences in tumor growth and survival rate were monitored. The expression of cyclin D1 in isolated tumor sections was evaluated by immunohistochemistry. We found that HepG2 tumor became palpable in the mice one-week post-inoculation. Tumors in the control group grew rapidly and the daily behavior of the mice changed significantly, including listlessness, poor feeding and ataxia. The mice in quercetin-treated group showed delayed tumor growth, no significant changes in daily behavior, and the survival rate was significantly improved. Finally, we observed increased tumor necrosis and a lighter cyclin D1 staining with reduced staining areas. Our findings thus suggest that quercetin can significantly inhibit HepG2 cell proliferation, and this effect may be achieved through the regulation of cyclin D1 expression.
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PMID:Investigation of the anti-cancer effect of quercetin on HepG2 cells in vivo. 2826 20

Neurofibromatosis type II (NF2) is a disease that lacks effective therapies. NF2 is characterized by bilateral vestibular schwannomas (VSs) that cause progressive and debilitating hearing loss, leading to social isolation and increased rates of depression. A major limitation in NF2 basic and translational research is the lack of animal models that allow the full spectrum of research into the biology and molecular mechanisms of NF2 tumor progression, as well as the effects on neurological function. In this protocol, we describe how to inject schwannoma cells into the mouse brain cerebellopontine angle (CPA) region. We also describe how to apply state-of-the-art intravital imaging and hearing assessment techniques to study tumor growth and hearing loss. In addition, ataxia, angiogenesis, and tumor-stroma interaction assays can be applied, and the model can be used to test the efficacy of novel therapeutic approaches. By studying the disease from every angle, this model offers the potential to unravel the basic biological underpinnings of NF2 and to develop novel therapeutics to control this devastating disease. Our protocol can be adapted to study other diseases within the CPA, including meningiomas, lipomas, vascular malformations, hemangiomas, epidermoid cysts, cerebellar astrocytomas, and metastatic lesions. The entire surgical procedure takes ~45 min per mouse and allows for subsequent longitudinal imaging, as well as neurological and hearing assessment, for up to 2 months.
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PMID:A cerebellopontine angle mouse model for the investigation of tumor biology, hearing, and neurological function in NF2-related vestibular schwannoma. 3061 50


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