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)

Cystic mass lesions within the sella turcica are common, and they include cystic pituitary adenomas, craniopharyngiomas, Rathke's cleft cysts, arachnoid cysts, and other entities. Until recently, such lesions were typically removed by a microsurgical transsphenoidal route. Given the increased use of the endoscope in transsphenoidal surgery, we evaluated the potential benefits of this tool in the treatment of such lesions. Between January 1997 and March 2005, 76 consecutive patients with sellar-suprasellar cystic lesions treated in three Neurosurgical Divisions underwent transsphenoidal removal in which the endoscope was used at least during the sellar step of the procedure (endoscope-assisted or fully endoscopic). The series consisted of 26 pituitary macroadenomas, 20 Rathke's cleft cysts, 18 craniopharyngiomas, 10 arachnoid cysts, one craniopharyngioma associated with an adrenocorticotropic hormone-secreting adenoma, and one chordoid glioma. Rigid 4-mm endoscopes (0 degrees , 30 degrees , and/or 45 degrees ) were used, and the advantages and limits of the endoscope during the sellar step of the procedure were recorded. Endoscopic exploration after lesion evacuation was generally easier and of greatest efficacy when the residual cystic cavity was larger as opposed to smaller. The use of angled endoscopes was optimal in larger residual cavities. Early descent of the suprasellar cistern, bleeding inside the residual cyst cavity, and a small sella were the most common causes preventing thorough exploration of the residual cavity after its evacuation. In no cases did the endoscope cause injury during the sellar cavity exploration. Endoscopic exploration of the sellar cavity during transsphenoidal surgery offers both general and specific advantages in the treatment of a variety of different cystic sellar lesions. Its routine use during transsphenoidal surgery for such lesions is recommended to achieve maximal and safe tumor removal.
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PMID:The role of the endoscope in the transsphenoidal management of cystic lesions of the sellar region. 1792 53

Radiation induced gliomas are uncommon. Occurrence of glioma following radiotherapy for craniopharyngiomas is extremely uncommon and only eight case reports have been so far published. We present our experience with one similar case of temporal gliomas occurring twelve years following radiotherapy for a sub totally excised craniopharyngioma. Although the exact mechanism of gliomas formation is unclear, their occurrence following conventional radiotherapy is a distinct possibility and signifies a poor prognosis.
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PMID:Malignant astrocytoma following radiotherapy for craniopharyngioma. 1799 22

Acute hyponatremia, following neurosurgery, results from inappropriate antidiuretic hormone secretion (SIADH) or cerebral salt wasting (CSW). CSW is due to abnormally high atrial or brain natriuretic peptides (ANP, BNP), which block all stimulators of zona glomerulosa steroidogenesis, resulting in mineralocorticoid deficiency. A 3 year-old girl presented CSW at day 4, after resection of craniopharyngioma and hypophysectomy. Hyponatremia, hyperkalemia and high natriuresis occurred on day 8, with low renin and aldosterone and elevated BNP 120.3 ng/ml (undetectable before surgery). Fludrocortisone 100 microg/day controlled natriuresis and restored electrolytes within 24 hours. A 5 year-old boy presented CSW at day 6 after partial resection of optic glioma. Fludocortisone 100 microg/day restored electrolytes within 8 hours. ANP was elevated, 60.6 ng/l, aldosterone and renin were low. Fludrocortisone supplementation should be considered in CSW, as excessive natriuresis is controlled, and electrolytes are easily restored, avoiding life-threatening complications of this complex disorder.
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PMID:Mineralocorticoid deficiency in post-operative cerebral salt wasting. 1805 34

To study the hypothesis that a delay in the diagnosis of paediatric brain tumours results in decreased survival outcome probability, we compared the prediagnostic period of 315 brain tumour patients (median age 6.7 years, range, 0 to 16 years) with progression-free and overall survival. The median prediagnostic symptomatic interval was 60 days (range, 0 to 3,480 days), with a median parental delay of 14 days (range, 0 to 1,835 days) and a median doctor's delay of 14 days (range, 0 to 3,480 days). The prediagnostic symptomatic interval correlated significantly with the patient age, tumour histology, tumour location and year of diagnosis, but not with gender. We then grouped the patients according to histology (low-grade glioma [n=77], medulloblastoma [n=57], high-grade glioma [n=40], craniopharyngioma [n=27], ependymoma [n=20] and germ cell tumours [n=18]). Contrary to common belief, long prediagnostic symptomatic interval or long doctor's delay did not result in decreased survival outcome probability in any of these groups. The effect of tumour biology on survival seems to be dominant and overwhelms any possible opposing effect on survival of a delay in diagnosis.
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PMID:Does diagnostic delay result in decreased survival in paediatric brain tumours? 1853 35

Intracranial tumors are the most common solid tumors in children. The infratentorial compartment will be the primary site for 60% to 70% of these tumors, including astrocytomas, medulloblastomas, and ependymomas. Several technological advancements have increased our knowledge of the cell biology of pediatric brain tumors, facilitated earlier diagnosis, and improved neurosurgical resections while minimizing neurological deficits. These in turn have not only improved the survival of children with brain tumors but also their quality of life. Current management strategies in most cases rely on surgery coupled with adjuvant therapies, including radiation therapy and chemotherapy. The vulnerability of the immature brain to adjuvant therapies creates many challenges for the treating physician. We review current diagnostic and therapeutic approaches and outcome for children harboring the most common pediatric brain tumors: astrocytomas (low-grade and high-grade glioma), ependymoma, medulloblastoma, and craniopharyngioma. The emphasis will be on the neurosurgical management of children with these tumors.
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PMID:Initial management of childhood brain tumors: neurosurgical considerations. 1895 80

The aim of this study was to collect, describe and analyze the radiation-induced gliomas in craniopharyngioma patients reported in the literature up to date. Review of the relevant literature was performed. One personal illustrative case was added. Reports of 15 patients, including the presented illustrative case, were evaluated. The average age of the patients at the time of irradiation was 12.5 years. All patients underwent conventional fractionated radiotherapy with mean total radiation dose of 55Gy and an average latency period of 10.8 years. Glioma localization varied with the highest frequency of the temporal lobe involvement. All but one patient had high-grade gliomas on the histological exam. Although exceptionally rare, the radiation-induced gliomas in craniopharyngioma patients are potentially possible, long-term complications with devastating consequences in typically younger patients with long life-expectancy. The radiation-induced iatrogenic injury on one hand should provoke the research and elaboration of safer and at least, equally efficient alternative treatment modalities and on the other hand ought to prompt the investigation of the patients' risk factors predisposing the oncogenesis after irradiation.
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PMID:Radiation-induced gliomas following radiotherapy for craniopharyngiomas: a case report and review of the literature. 1944 44

We report the case of a 31-year-old male who presented to the ER with a 1-week history of progressively worsening, throbbing, left retro-orbital headache, ptosis, and subjective worsening of short-term memory function. Initial review of systems and laboratory data were noncontributory. Non-contrasted CT demonstrated a large hyperdense mass centered in the suprasellar cistern without evidence of dissecting extra-axial hemorrhage. Though the initial appearance mimicked a basilar tip aneurysm or another primary extra-axial suprasellar pathology such as a hemorrhagic or proteinaceous craniopharyngioma, germinoma, or optic glioma, a second smaller, clearly intra-axial, hyperdense lesion was observed in the left periventricular forceps major white matter. Consideration for multiple cavernomas versus hypervascular metastatic disease such as renal malignancy, thyroid malignancy, or melanoma was raised. CTA confirmed normal intracranial vasculature. Subsequent MRI images showed an acutely hemorrhagic mass centered at the left paramedian hypothalamus and tuber cinereum with numerous secondary foci, demonstrating mature hemorrhagic elements and confirming the diagnosis of multiple cavernomas.
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PMID:Exophytic hypothalamic cavernous malformation mimicking an extra-axial suprasellar mass. 2130 32

We present a rare case of optochiasmatic cavernous angioma (CA) that progressed despite radiation therapy. A 31-year-old female patient presented with sudden loss of left visual acuity and right homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed a suprasellar mass and findings compatible with a craniopharyngioma or an optic glioma with bleeding. An open biopsy was conducted using the transcranial approach, and histological examination revealed gliosis. During the one-year follow-up period, imaging suggested intratumoral bleeding and the mass continued to grow. We recommended re-operation, but the patient refused due to fear of surgery. Consequently, the patient received fractionated radiation therapy (3,000 cGy) to the parasellar area. Despite the radiotherapy, the mass continued to grow for the following 6 years. The final MRI before definitive treatment revealed a multilobulated, multistage hematoma with calcification in the parasellar area, extending into the third ventricle and midbrain. The patient ultimately underwent reoperation due to the growth of the tumor. The mass was completely removed with transcranial surgery, and the pathologic findings indicated a cavernous angioma (CA) without evidence of glioma. As shown in our case, patients may suffer intratumoral hemorrhage after biopsy and radiotherapy. This case places the value of biopsy and radiotherapy for a remnant lesion into question. It also shows that reaching the correct diagnosis is critical, and complete surgical removal is the treatment of choice.
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PMID:Optochiasmatic cavernous angioma with rapid progression after biopsy despite radiation therapy. 2151 2

Increasing use of neuroimaging in children has led to more incidental findings of CNS mass lesions, the management of which is uncertain. The authors' aims in this study are to describe these mass lesions and their evolution, as well as to discuss the management options and determine the prevalence of incidental CNS mass lesions at their pediatric clinic. A retrospective study was undertaken in children with primary CNS tumors who were younger than 18 years old and were admitted to the University Children's Hospital of Zurich, Switzerland, between January 1995 and December 2010. In 19 (5.7%) of 335 patients with newly diagnosed CNS tumors, the diagnosis of a CNS mass lesion was an incidental finding. Reasons for obtaining neuroimages in these 19 patients were head trauma (in 6 patients); research protocols (in 3); nasal/orbital malformations (in 2); endocrinological and psychiatric evaluations (in 2); and vertebral bone anomaly without neurological signs, absence seizures, congenital ataxia, recurrent vomiting, developmental delay, and "check-up" at the explicit request of the parents (in 1 patient each). Seven patients underwent immediate surgery for low-grade glioma (4 patients) and craniopharyngioma, ependymoma, and choroid plexus papilloma (1 patient each); and 12 were treated conservatively or were observed. Ten of 12 conservatively treated patients remained stable (median follow-up time 1.8 years) and the other 2 underwent delayed surgery because of tumor progression (medulloblastoma in one patient and fibrillary astrocytoma in the other). Clinicians are increasingly challenged by the discovery of incidental CNS mass lesions. A subgroup of such lesions (with typical imaging patterns such as tectal glioma and dysembryoplastic neuroepithelial tumor) can be monitored conservatively, clinically, and radiographically. Future prospective studies are needed to define optimal management strategies based on larger collections of natural histories, as well as to assess the true prevalence of incidental CNS mass lesions.
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PMID:Incidental findings of mass lesions on neuroimages in children. 2213 79

Visual field deficits can be a consequence of brain tumor location or treatment. The prevalence of unrecognized visual field deficits in children diagnosed with brain tumors is not known. All children at a single tertiary care pediatric children's hospital diagnosed with a primary brain tumor were tested for visual field deficits by a child neurologist and neuro-ophthalmologist over 16 months. Children with reproducible visual field deficits on two separate occasions were included in the analysis. Patients with optic glioma, craniopharyngioma, or previously known visual field deficits were excluded. Fourteen of 92 (15.2%) children (average 8.9 years, 8 girls) had undiagnosed visual field deficits. Average time between diagnosis of tumor and unrecognized visual field deficit was 3.7 years (range 0-13 years). Unrecognized visual field deficits were not associated with age (P = 0.27) or gender (P = 0.38). Visual field deficits were attributed to direct tumor infiltration (n = 8), postoperative complications (n = 5) and post-radiation edema (n = 1). Deficits included bitemporal hemianopsia (n = 2), homonymous hemianopsia (n = 9), quadrantanopsia (n = 2), and concentric visual field loss (n = 1.) Tumor location included temporal lobe (n = 9), parietal lobe (n = 2), posterior fossa (n = 2), hypothalamic-chiasmatic (n = 2) and multifocal areas (n = 4). Children with temporal lobe tumors were more likely to have unrecognized visual field deficits (P = 0.004). In all 14 patients, visual field deficits were determined by examination only and were not reported by either the patient or caregiver regardless of age. The prevalence of unrecognized visual field deficits in children with brain tumors can be surprisingly high. Serial neuro-ophthalmologic evaluation of children with brain tumors is often required to diagnose a visual field deficit since patient or caregiver reporting may be limited.
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PMID:Unrecognized visual field deficits in children with primary central nervous system brain tumors. 2213 48


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