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Journal of oral and maxillofacial surgery: official journal of the American Association of Oral and Maxillofacial Surgeons 07/2011; 70(2):367-72. · 1.58 Impact Factor
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ABSTRACT: Hemangioblastomas, highly vascular tumors, occur sporadically or associated with von Hippel-Lindau (VHL) disease. Diverse mutations in the VHL gene inactivate the VHL protein and constitute the molecular etiology of the disease. Changes in VHL gene were analyzed in patients with multiplex ligation-dependent probe amplification and single-strand conformation polymorphism analyses. We report here that other angiogenesis-related changes in vitronectin were identified with 2D electrophoresis of plasma samples and restriction fragment length polymorphisms. Our findings revealed that most patients (80.0%) with a familial VHL deletion carried the threonine (T) allele at vitronectin codon 381. Adults simultaneously carrying a VHL defect and the T allele were 5.0-fold more likely to be affected by VHL disease than were methionine/methionine (M/M) homozygotes carrying a VHL defect. Patients with sporadic hemangioblastoma, C-terminally truncated VHL protein or a large deletion in the VHL gene, and the T allele were 18.0-fold more likely to develop recurrent disease. Taken together, individuals with mutated VHL are more likely to be affected by familial or recurrent sporadic hemangioblastoma when carrying the M/T or T/T genotype at codon 381 of vitronectin.
Journal of Molecular Medicine 04/2009; 87(6):613-22. · 4.67 Impact Factor
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ABSTRACT: To investigate the prognostic significance of the volumetrically assessed extent of resection on time to tumor progression (TTP), overall survival (OS), and tumor recurrence patterns, the authors retrospectively analyzed preoperative and postoperative tumor volumes in 102 adult patients from the time of the initial resection of a hemispheric anaplastic astrocytoma (AA).
The quantification of tumor volumes was based on a previously described method involving computerized analysis of magnetic resonance (MR) images. Analysis of contrast-enhancing tumor volumes on T1-weighted MR images was conducted for 67 patients who had contrast-enhancing tumors. Measurements of T2 hyperintensity were obtained for all 102 patients in the study. The presence or absence of preresection enhancement, actual volume of this enhancement, and the percentage of preoperative enhancement as it relates to the total T2 tumor volume did not have a statistically significant relationship to TTP or OS. In addition to age, the volume of residual disease measured on T2-weighted MR images was the most significant predictor of TTP (p < 0.001), and residual contrast-enhancing tumor volume was the most significant predictor of OS (p = 0.003) on multivariate analysis. In contrast to low-grade gliomas, there was no statistically significant relationship between the extent of resection and histological characteristics at the time of recurrence, that is, tumor Grade III compared with Grade IV.
Data from this retrospective analysis of a histologically uniform group of hemispheric AAs treated in the MR imaging era suggest that residual tumor volumes, as documented on postoperative imaging studies, may be a prognostic factor for TTP and OS for this patient population.
Journal of Neurosurgery 07/2006; 105(1):34-40. · 2.96 Impact Factor
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ABSTRACT: Intramedullary hemangioblastomas are relatively rare intraspinal tumors. Total removal of these tumors without causing significant neurological deficit remains a great challenge. This study analyzed the preoperative characteristics, management, and outcome in patients with successful total removal of spinal intramedullary hemangioblastomas.
Data from the medical records of patients with intraspinal hemangioblastomas treated from 1993 to 2003 were reviewed. The neurological function of these patients was graded and the preoperative data were recorded and correlated with clinical outcome.
Ten patients with spinal intramedullary hemangioblastomas underwent microsurgical resection by the same neurosurgeon during the 10-year study period. They included 3 men and 7 women with age ranging from 20 to 49 years, with a mean age of 33 years. Five of the patients met the diagnostic criteria for von Hippel-Lindau disease. Preoperative neurological function was grade I in 6 patients, grade II in 3 patients and grade III in 1 patient. Immediate postoperative neurological function was worse in 3 cases, but all of these patients recovered their preoperative function within 3 weeks. At 3 months' follow-up, 9 patients had achieved functional grade I, and 1 patient with preoperative grade III function had improved to grade II.
Detailed preoperative evaluation and skillful microsurgery are mandatory in successful total removal of intramedullary hemangioblastomas. The management of spinal hemangioblastomas associated with von Hippel-Lindau disease requires a cautious observation of clinical course and timely surgical intervention of symptomatic lesions to avoid possible neurological deficit.
Journal of the Formosan Medical Association 01/2004; 102(12):868-75. · 1.13 Impact Factor
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ABSTRACT: Spinal epidural lipomatosis (SEL), an abnormal localized or tumor-like accumulation of fat in the epidural space, is an infrequent complication of chronic steroid usage and an uncommon cause of spinal cord compression. During the period of 1990 to 2006, we have two cases of medically heath SEL patients without history of steroid administration. Their initial clinical manifestations were low back pain, progressive lower extremities weakness, numbness, followed by rapid deterioration of neurogenic intermittent claudication. They were misdiagnosed and treated as degenerative spinal disease for a long time. Due to prominent neurological deficit, lumbar magnetic resonance image (MRI) was obtained and showed SEL. These 2 patients all underwent laminectomy and removal of epidural fat. Postoperatively, they both showed improvement. We reviewed the literature and discussed the current concept in the management of SEL.
Chang Gung medical journal 32(6):662-7.
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Neurology India 60(1):102-3. · 0.96 Impact Factor
