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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty patients with histologically confirmed posterior fossa ependymomas operated on between January 1976 and December 1988 were reviewed. The median age was 44 years (range, 1-69 yr). There were 7 children (aged 5 yr or younger) and 23 adults (aged 16 yr or older). There were 18 female patients and 12 male patients. Headache, nausea and vomiting, and disequilibrium were the most frequent symptoms. The most common findings were ataxia and nystagmus. Gross total resection was performed in 8 patients (27%), subtotal resection in 21 patients (70%), and biopsy in only 1 patient (3%). Tumors were low grade in 73% and high grade in 27%. Twenty-seven patients underwent posterior fossa radiotherapy (median dose, 5400 cGy). Fourteen patients also underwent spinal irradiation (median dose, 3520 cGy). Age was the only significant prognostic factor identified (P less than 0.01). The 5-year survival rates were 76% for adults and 14% for children. All 14 patients who died had recurrent or residual tumor at the primary site. This review suggests that in patients with primary posterior fossa ependymomas the following is true: 1) the young patient (5 yr old or younger) has a poor prognosis; 2) there was a trend toward a better 5-year survival rate with a gross total resection; 3) if recurrence occurs, it will be at the primary intracranial site; and 4) symptomatic spinal seeding does not occur frequently.
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PMID:Posterior fossa ependymomas: report of 30 cases and review of the literature. 187 43

A retrospective study was conducted on all 23 patients who underwent operation for residual or recurrent acoustic neuromas during the 10-year period January 1976 through December 1985. The most common symptoms at the time of reoperation were ataxia (16 patients), facial paresthesias (13 patients), and headaches (9 patients). Primary procedures had consisted of suboccipital posterior fossa approaches in 22 patients and a middle fossa approach in one. Reoperation for recurrent or residual tumor consisted of a retrosigmoid approach in 17 patients and a translabyrinthine or translabyrinthine/retrosigmoid approach in six patients. This study confirms that residual or recurrent acoustic tumors are not common. It also suggests that long-term follow-up, for at least 7 to 8 years, is indicated.
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PMID:Residual and recurrent acoustic neuromas. 365 63

The morbidity associated with gross total removal of pediatric posterior fossa tumors is well recognized although it is rarely isolated from other factors that comprise the management morbidity for these tumors. This study reviews (1) the operative and postoperative complications in 105 patients and (2) the neurological morbidity in a subset of 91 patients undergoing gross total removal of their tumor between 1982 and 1992. Gross total removal was achieved in 102 patients with a single procedure. Two patients with residual tumor underwent early repeat craniotomy for excision and 1 is being followed without repeat resection. Intra- and postoperative complications occurred in 33 patients and included hematoma requiring craniotomy (3), gastrointestinal hemorrhage (2), hydrocephalus requiring shunt placement (9), wound problems (4), and pseudomeningocele formation requiring additional treatment (5). Delayed onset hydrocephalus requiring shunting occurred in 2 patients and spinal deformity in 4 patients. Worsening of preoperative deficit (new cranial nerve palsies, worsening ataxia, bulbar dysfunction including apnea, mutism and seizures) occurred in 41% of patients operated on for primitive neuroectodermal tumors (PNET) (14/34), 53% of ependymomas (10/19), and 30% of astrocytomas (15/50). No patient who had a choroid plexus tumor was worsened by the procedure. Complete recovery of new postoperative deficits occurred in 14% of PNET (2/14), 50% of ependymoma (5/10) and 47% of astrocytoma (7/15), most often within 6 months of the procedure. Residual neurological morbidity, due to persistence of preoperative symptoms or due to deficits that occurred as the result of the surgical procedure, was assessed in a subgroup of 91 patients followed for an average of 48 months (2-147 months). This assessment did not include morbidity due to adjuvant therapy. Sixty-two percent of patients continued to exhibit abnormal cerebellar or bulbar signs. Forty-three percent of the total population exhibited limitation in function due to residual deficit. Only 38% of patients were both functionally normal and had a normal neurological examination at last follow-up.
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PMID:The surgical and natural morbidity of aggressive resection for posterior fossa tumors in childhood. 814 78

Encouraged by an experience with endoscopic transsphenoidal pituitary surgery, an endoscopic transsphenoidal technique was applied in a patient with a large chordoma in the posterior fossa. The patient was a 40-year-old man with a two-year history of progressive ataxia, a memory disorder and emotional instability. A magnetic resonance (MR) scan of the brain revealed a midline posterior fossa mass measuring 4 cm in diameter located between the clivus and the brainstem. The basilar artery and its bifurcation were encased by the tumor and the brainstem was also distorted by the tumor. Obstructive hydrocephalus was treated previously with a ventriculoperitoneal shunt and fractionated external beam radiation treatment was given without histological diagnosis at another hospital. Subtotal resection of the tumor was achieved utilizing an endoscopic transphenoidal technique through the patient's nostril. The portion of the tumor located behind the basilar artery was not resected in order to protect the brainstem perforating arteries. The patient showed dramatic improvement of his symptoms postoperatively. Residual tumor located behind the basilar artery was treated by stereotactic gamma-knife surgery. This is the first reported case of a large posterior fossa chordoma being treated by an endoscopic transsphenoidal technique.
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PMID:Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. 920 75

Chromosome 11q deletions are common in various malignancies, including ovarian cancer. However, the clinical significance of these genetic lesions as well as their more precise chromosomal location is largely unknown. Here we have examined epithelial ovarian cancer material from 49 patients for loss of heterozygosity (LOH) using nine microsatellite markers on 11q22.3-q25 and evaluated the effect of observed deletions with regard to different clinicopathological variables. LOH was detected in 61% of the patients. Interestingly, LOH for the D11S1340 marker locus at 11q23. 3 seemed to be associated with significantly reduced survival times (P = 0.005) and serous tumor histology (P = 0.036). LOH for D11S912 at the more distal 11q24-q25 location correlated with a higher tumor stage (P = 0.003), serous tumor histology (P = 0.015), and finding of residual tumor (P = 0.047), but not directly with survival times (P = 0.320). The majority of the analyzed tumors simultaneously displayed deletions at two distinct 11q regions, A and B, which are proximal and distal to D11S1347/NCAM (11q23.2-q23.3), respectively. Only LOH for two markers (D11S1340 and D11S912) of the B region seemed to be directly associated with a more aggressive disease course. Therefore, it appears that deletions of the ataxia telangectasia gene of the A region would not be crucial for determining the outcome of ovarian cancer. Our present results indicate that a survival factor gene in ovarian cancer would be located close to D11S1340 at 11q23.3. This corresponds well to our earlier observation in breast cancer, suggesting the involvement of a shared survival factor gene in both diseases.
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PMID:Chromosome 11q22.3-q25 LOH in ovarian cancer: association with a more aggressive disease course and involved subregions. 982 75

The authors report their encouraging experience using an endoscopic technique for transsphenoidal surgery in a patient with a large chordoma in the posterior fossa. The patient was a 40-year-old man with a 2-year history of progressive ataxia, a memory disorder, and emotional instability. A magnetic resonance (MR) image of the brain revealed a midline posterior fossa mass measuring 4 cm in diameter located between the clivus and the brainstem. The basilar artery and its bifurcation were encased by the tumor, which also distorted the brainstem. The patient had been treated at another hospital for obstructive hydrocephalus with a ventriculoperitoneal shunt and he received fractionated external-beam radiation treatment, although no histological diagnosis was ever made. The authors achieved a subtotal resection of the tumor through the patient's nostril using an endoscopic transsphenoidal technique. The portion of the tumor located behind the basilar artery was not resected to protect the brainstem perforating arteries. The patient showed dramatic improvement of his symptoms postoperatively. Residual tumor located behind the basilar artery was treated by stereotactic gamma knife surgery. This is the first reported case of a large posterior fossa chordoma treated by an endoscopic transsphenoidal technique.
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PMID:Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. Case report. 1509 97

A 63-year-old woman presented with a metastatic focus of papillary thyroid carcinoma in the cerebellopontine angle manifesting as lateral gazing nystagmus and slurred speech. Computed tomography demonstrated massive hemorrhage in the left cerebellar hemisphere. She was treated conservatively. Her symptoms resolved completely, but she experienced progressive deterioration in auditory acuity and ataxia over the next 6 months. Magnetic resonance imaging with gadolinium demonstrated an enhanced mass in the left cerebellopontine angle, and she was scheduled for elective resection of the tumor. Left retrosigmoid craniotomy was performed, and the tumor was subtotally removed except for a small amount at the junction of the trigeminal nerve and the pons. Histological examination confirmed a diagnosis of metastatic papillary thyroid carcinoma. The patient then underwent adjuvant gamma knife radiosurgery. Her clinical course was unremarkable, and her hypoacusis and ataxia resolved completely. Postoperative gallium scintigraphy revealed no residual tumor and no other systemic metastases. Hemorrhagic cerebellar metastasis from papillary thyroid carcinoma is extremely rare, but early recognition of metastatic cerebellar tumor should prompt immediate treatment to avoid the development of hearing loss, ataxia, and tonsillar herniation.
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PMID:Cerebellar hemorrhage secondary to cerebellopontine angle metastasis from thyroid papillary carcinoma. 2361 14

Intracranial hemangioblastomas are benign but hypervascular tumors, most commonly located in the cerebellum, which are difficult to resect without significant operative blood loss. While preoperative embolization may decrease the amount of operative bleeding, the vascular supply of cerebellar hemangioblastomas frequently precludes safe embolization by an endovascular route due to the risk of thromboembolic vertebrobasilar infarction. Direct puncture embolization overcomes many of the limitations of endovascular embolization but its safety and feasibility for intracranial tumors is unknown. We report a 48-year-old man who was diagnosed with a large cerebellar mass after presenting with headaches and gait ataxia. Based on diagnostic angiography, which demonstrated a highly vascular tumor supplied by the posterior inferior cerebellar and posterior meningeal arteries, we decided to embolize the tumor by a direct transcranial puncture approach. After trephinating the skull in a standard fashion, a catheter-needle construct, composed of an Echelon 10 microcatheter (ev3 Endovascular, Plymouth, MN, USA) placed into a 21-gauge spinal needle, was inserted into the tumor under biplanar angiographic guidance. Using continuous angiographic monitoring, 9cc of Onyx 34 (ev3 Endovascular) was injected through the catheter, resulting in 75% tumor devascularization without evidence of complications. The patient was taken directly to surgery where a gross total resection of the hemangioblastoma was achieved with an acceptable operative blood loss. At his 2 year follow-up, the patient was neurologically intact without neuroimaging evidence of residual tumor. We describe, to our knowledge, the first case of direct transcranial puncture for preoperative embolization of a cerebellar hemangioblastoma.
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PMID:Direct transcranial puncture for Onyx embolization of a cerebellar hemangioblastoma. 2437 May 4

An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues. The likelihood of regional or distant metastases is remote. The optimal treatment is resection with negative margins. Patients with positive margins, gross residual disease, or unresectable tumor are treated with radiotherapy or radiosurgery. Late recurrences are common, so long follow-up is necessary to assess efficacy. The likelihood of cure depends on tumor extent and is probably in the range of 50-75%.
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PMID:Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone. 2992 43

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 67-year-old woman was diagnosed 17 months ago with stage IIIB non-small-cell lung cancer (NSCLC; adenocarcinoma), without a targetable mutation, for which she received chemotherapy and thoracic radiotherapy. She developed new-onset gait ataxia, nausea, morning emesis, and headaches. A brain magnetic resonance imaging (MRI) scan demonstrated a 3.2-cm left cerebellar enhancing metastatic lesion, with surrounding vasogenic edema and mass effect, and three additional enhancing cortical, parenchymal lesions, each < 1.2 cm in maximum diameter, and none with significant mass effect ( Fig 1A ). A restaging positron emission tomography scan revealed no new sites of metastatic disease and no obvious intrathoracic progression. She underwent craniotomy, with piecemeal resection of the cerebellar metastatic lesion that proved to be recurrent lung cancer ( Fig 1B ). Her medical history was significant for mild hypertension and diet-controlled type II diabetes. She had smoked one pack a day for 32 years and had quit 3 years earlier. Postoperatively, her neurologic symptoms resolved, and physical and neurologic examinations were unrevealing; she had an Eastern Cooperative Oncology Group performance status (PS) of 0 and a Mini-Mental State Examination (MMSE) score of 29 of 30. A 3-week postoperative MRI scan confirmed the presence of 1-cm right frontal, 1.2-cm right parietal, and 1.2-cm left temporal enhancing lesions with minimal enhancement and no evidence of mass effect or edema. In addition, there was an ill-defined residual tumor bed, estimated to be approximately 3.7 cm in maximum diameter ( Fig 1C ). The patient was no longer receiving any steroids and was not receiving any anticonvulsants. Because of the residual three brain metastatic lesions, as well as the consideration of a high risk of surgical failure, she was referred for radiotherapy.
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PMID:Postoperative Management of Resected Brain Metastases: When Can Radiotherapy Be Deferred? 3029 92


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