BACKGROUND AND PURPOSE:Endolymphatic hydrops has been recognized as the underlying pathophysiology of Menière disease. We used 3T MR imaging to detect and grade endolymphatic hydrops in patients with Menière disease and to correlate MR imaging findings with the clinical severity.
SUMMARY:The relevant aspects of cholesteatomas are reviewed with the emphasis on their diagnosis by using cross-sectional imaging. The indications and limitations of CT and MR imaging and the use of novel MR imaging techniques in the diagnosis of cholesteatomas are described. HRCT of the temporal bone has an excellent spatial resolution, thus even small soft-tissue lesions can be accurately delineated (high sensitivity). However, CT has poor specificity (ie, soft-tissue structures cannot be differentiated). MR imaging with the conventional sequences (T1WI, T2WI, postcontrast T1WI) provides additional information for distinguishing different pathologic entities and for accurately diagnosing primary (nonsurgical) and residual/recurrent (surgical) cholesteatomas. Higher diagnostic specificity is achieved by introducing DW-EPI, delayed postcontrast imaging, DW-non-EPI, and DWI-PROPELLER techniques. Studies using DW-non-EPI and DWI-PROPELLER sequences show promising results related to improved diagnostic sensitivity and specificity for even small (Ͻ5 mm) cholesteatomas, thus allowing avoidance of second-look surgery in the future.ABBREVIATIONS: DWI ϭ diffusion-weighted imaging; DW-EPI ϭ diffusion-weighted echo-planar imaging; DWI-PROPELLER ϭ diffusion-weighted imaging with periodically rotated overlapping parallel lines with enhanced reconstruction; DW-non-EPI ϭ diffusion-weighted non-echo-planar imaging; EAC ϭ external auditory canal; EACC ϭ external auditory canal cholesteatoma; EPI ϭ echo-planar imaging; FIESTA ϭ fast imaging employing steady-state acquisition; FLASH ϭ fast low-angle shot; HRCT ϭ high-resolution CT; NPV ϭ negative predictive value; PPV ϭ positive predictive value; T1WI ϭ T1-weighted imaging; T2WI ϭ T2-weighted imaging; TM ϭ tympanic membrane C holesteatoma has been known for more than 300 years in the medical literature; still its precise detection with the use of cross-sectional imaging techniques remains challenging. As before, the diagnosis of a cholesteatoma at first presentation is mainly based on clinical suspicion. HRCT provides information about bony changes and intracranial complications; however, it is inaccurate for characterizing the soft-tissue mass in the temporal bone. In the past 7 years, improvements in MR imaging techniques have enhanced the sensitivity and specificity of radiologic diagnosis, which may lead to future avoidance of second-look surgeries in cases of residual/recurrent cholesteatomas.The purpose of this review article is to summarize all aspects of cholesteatomas, including their definition, history, etymology, epidemiology, classification, histology, pathophysiology, clinical signs, and neuroradiologic diagnosis. We review the latest studies on the application of new MR imaging techniques for the accurate diagnosis of cholesteatomas. Definition"Cholesteatoma" is a well-demarcated non-neoplastic lesion in the temporal bone, which is commonly described as "skin in the wrong place." History and EtymologyJoseph-Guichard Duverney, a French anatomist, was the first to d...
Background: Patients with glioblastoma (GBM) inevitably develop recurrent or progressive disease after initial multimodal treatment and have a median survival of 6–9 months from time of progression. To date, there is no accepted standard treatment for GBM relapse or progression. Patupilone (EPO906) is a novel natural microtubule-stabilizing cytotoxic agent that crosses the blood-brain barrier and has been found to have preclinical activity in glioma models. Methods: This is a single-institution, early-phase I/II trial of GBM patients with tumor progression who qualified for second surgery with the goal of evaluating efficacy and safety of the single-agent patupilone (10 mg/m2, every 3 weeks). Patients received patupilone 1 week prior to second surgery and every 3 weeks thereafter until tumor progression or toxicity. Primary end points were progression-free survival (PFS) and overall survival (OS) at 6 months as well as patupilone concentration in tumor tissue. Secondary end points were toxicity, patupilone concentration in plasma and translational analyses for predictive biomarkers. Results: Nine patients with a mean age of 54.6 ± 8.6 years were recruited between June 2008 and April 2010. Median survival and 1-year OS after second surgery were 11 months (95% CI, 5–17 months) and 45% (95% CI, 14–76), respectively. Median PFS was 1.5 months (95% CI, 1.3–1.7 months) and PFS6 was 22% (95% CI, 0–46), with 2 patients remaining recurrence-free at 9.75 and 22 months. At the time of surgery, the concentration of patupilone in tumor tissue was 30 times higher than in the plasma. Tumor response was not predictable by the tested biomarkers. Treatment was generally well tolerated with no hematological, but cumulative, though reversible sensory neuropathy grade ≤3 was seen in 2 patients (22%) at 8 months and grade 4 diarrhea in the 2nd patient (11%). Non-patupilone-related peri-operative complications occurred in 2 patients resulting in discontinuation of patupilone therapy. There were no neurocognitive changes 3 months after surgery compared to baseline. Conclusions: In recurrent GBM, patupilone can be given safely pre- and postoperatively. The drug accumulates in the tumor tissue. The treatment results in long-term PFS in some patients. Patupilone represents a valuable novel compound which deserves further evaluation in combination with radiation therapy in patients with GBM.
Our case supports the existence of a late onset adult form of LCC.
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