14 research outputs found

    Differentiated Papillary NUT Carcinoma: An Unexpected, Deceptively Bland Presentation of a Sinonasal Carcinoma

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    BACKGROUND In recent years, the list of tumor entities in the sinonasal tract has significantly expanded, requiring advanced diagnostic testing. We report the case of a 32-year-old patient with an unusual NUT carcinoma originating in the maxillary sinus, which showed extensive well-differentiated, papillary squamous morphology, similar to the spectrum of the recently described DEK::AFF2 fusion-associated carcinoma. METHODS We performed immunohistochemical and molecular studies including EBV- and HPV-testing, as well as DNA/RNA next generation sequencing. RESULTS The tumor showed predominantly exophytic papillary growth with mature squamous differentiation. An additional component harbored atypical, less differentiated basaloid tumor cells with infiltration of the adjacent stroma. Conspicuous inflammation was evident. There was no evidence of HPV DNA or EBV RNA. Next-generation sequencing revealed a NUT::NSD3 gene fusion corresponding to ("speckled-type") immunopositivity of NUT in the tumor cells. CONCLUSIONS We describe a NUT::NSD3 gene fusion-associated NUT carcinoma of the sinonasal tract with a deceptively well-differentiated papillary growth pattern, thus expanding the morphological spectrum of this typically poorly differentiated neoplasm

    Differentiated Papillary NUT Carcinoma: An Unexpected, Deceptively Bland Presentation of a Sinonasal Carcinoma.

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    BACKGROUND In recent years, the list of tumor entities in the sinonasal tract has significantly expanded, requiring advanced diagnostic testing. We report the case of a 32-year-old patient with an unusual NUT carcinoma originating in the maxillary sinus, which showed extensive well-differentiated, papillary squamous morphology, similar to the spectrum of the recently described DEK::AFF2 fusion-associated carcinoma. METHODS We performed immunohistochemical and molecular studies including EBV- and HPV-testing, as well as DNA/RNA next generation sequencing. RESULTS The tumor showed predominantly exophytic papillary growth with mature squamous differentiation. An additional component harbored atypical, less differentiated basaloid tumor cells with infiltration of the adjacent stroma. Conspicuous inflammation was evident. There was no evidence of HPV DNA or EBV RNA. Next-generation sequencing revealed a NUT::NSD3 gene fusion corresponding to ("speckled-type") immunopositivity of NUT in the tumor cells. CONCLUSIONS We describe a NUT::NSD3 gene fusion-associated NUT carcinoma of the sinonasal tract with a deceptively well-differentiated papillary growth pattern, thus expanding the morphological spectrum of this typically poorly differentiated neoplasm

    Accuracy of High-Resolution Computed Tomography Compared to High-Definition Ear Endoscopy to Assess Cholesteatoma Extension.

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    OBJECTIVE To correlate radiographic evidence of cholesteatoma in the retrotympanum with intraoperative endoscopic findings in cholesteatoma patients and to evaluate the clinical relevance of radiographic evidence of cholesteatoma in the retrotympanum. STUDY DESIGN Case series with chart review. SETTING Tertiary referral center. METHODS Seventy-six consecutive cases undergoing surgical cholesteatoma removal with preoperative high-resolution computed tomography (HRCT) were enrolled in this study. A retrospective analysis of the medical records was conducted. The extension of cholesteatoma regarding different middle ear subspaces, into the antrum and mastoid were reviewed radiologically in preoperative HRCT and endoscopically from surgical videos. Additionally, facial nerve canal dehiscence, infiltration of the middle cranial fossa, and inner ear involvement were documented. RESULTS Comparison of radiological and endoscopic cholesteatoma extension revealed statistically highly significant overestimation of radiological cholesteatoma extension for all retrotympanic regions (sinus tympani 61.8% vs 19.7%, facial recess 69.7% vs 43.4%, subtympanic sinus 59.2% vs 7.9%, and posterior sinus 72.4% vs 4.0%) and statistically significant overestimation for mesotympanum (82.9% vs 56.6%), hypotympanum (39.5% vs 9.2%), and protympanum (23.7% vs 6.6%). No statistically significant differences were found for epitympanum (98.7% vs 90.8%), antrum (64.5% vs 52.6%), and mastoid (26.3% vs 32.9%). Statistically significant radiological overestimation of facial nerve canal dehiscence (54.0% vs 25.0%) and invasion of tegmen tympani (39.5% vs 19.7%) is reported. CONCLUSION Radiologic cholesteatoma extension in different middle ear subspaces is overestimated compared to the intraoperative extension. The preoperative relevance of radiological retrotympanic extension might be limited in the choice of approach and transcanal endoscopic approach is always recommended first

    Variability of the retrotympanum and its association with mastoid pneumatization in cholesteatoma patients.

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    PURPOSE This study aimed to investigate the variability of the retrotympanum in patients undergoing surgical treatment for cholesteatoma. METHODS We included 59 ears of patients undergoing middle ear surgery for cholesteatoma who had preoperative computed tomography scans. A retrospective analysis of the medical records was conducted. The sinus tympani (ST), subtympanic sinus (STS) and facial recess (FR) were classified into types A-C based on the relationship of their extension to the facial nerve. The mastoid and petrous apex were assessed and categorized as normal pneumatized or sclerotic. RESULTS Type A extension was the most frequently found in all sinuses (ST 64%, FR 77%, STS 69%), Type B extension was found more often in ST (34%) and STS (24%) than in FR (15%). A very deep extension was found only rarely (ST 2%, FR 8%, STS 7%). A sclerotic mastoid was found in 67% of cases. Those cases showed a statistically significant difference regarding retrotympanum pneumatization when compared with normal mastoid. CONCLUSION The most frequent variant of retrotympanic pneumatization in relation to the facial nerve was type A in all subsites in cholesteatoma patients. The variability among patients with cholesteatoma is different to previously published results in healthy subjects. Moreover, the pneumatization of the retrotympanum is associated with mastoid pneumatization

    Signal Alteration of the Inner Ear on High-Resolution Three-Dimensional Constructive Interference in Steady State Sequence in Patients with Ménière's Disease and Labyrinthitis.

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    INTRODUCTION The aim of this study is to evaluate signal alteration in the inner ear using three-dimensional (3D)-constructive interference in steady state (CISS) sequence in patients with Ménière's disease and labyrinthitis and its correlation with clinical and audiological parameters. METHODS The medical records of the department of otorhinolaryngology were searched for patients with Ménière's disease or labyrinthitis who underwent MRI with 3D-CISS sequence. Blinded analysis of these patients and of MRI from control subjects without middle or inner ear symptoms was performed to detect any signal asymmetry of the inner ear structures. The results were correlated with clinical symptoms and results of audiological and vestibular tests. RESULTS Fifty-eight patients with definite Ménière's disease and 5 patients with labyrinthitis as well as 41 control exams were included. A separate analysis was performed for patients with probable Ménière's disease (n = 68). A total of 172 3D-CISS sequences were analyzed by 2 blinded independent neuroradiologists. A CISS-hypointense signal of the inner ear structures was found in 3 patients with definite Ménière's disease (5.2%), in 4 patients with probable Ménière's disease (5.9%), and 2 patients with labyrinthitis (40%). No CISS hypointensity was found in the control group. Although no significant difference in symptoms or audiological test results was found between patients with and without this signal change, the side of hypointensity was frequently correlated with the symptomatic side and with hearing impairment. DISCUSSION/CONCLUSION CISS hypointensity of the inner ear structures was evident in patients with clinical conditions other than vestibular schwannoma - more frequently in labyrinthitis than in Ménière's disease. This signal alteration was frequently encountered on the same symptomatic side as that of the pathological audiology tests, but it is not a predictor for hearing or vestibular impairment

    Effectiveness of a national quality improvement programme to improve survival after emergency abdominal surgery (EPOCH): a stepped-wedge cluster-randomised trial

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    Background: Emergency abdominal surgery is associated with poor patient outcomes. We studied the effectiveness of a national quality improvement (QI) programme to implement a care pathway to improve survival for these patients. Methods: We did a stepped-wedge cluster-randomised trial of patients aged 40 years or older undergoing emergency open major abdominal surgery. Eligible UK National Health Service (NHS) hospitals (those that had an emergency general surgical service, a substantial volume of emergency abdominal surgery cases, and contributed data to the National Emergency Laparotomy Audit) were organised into 15 geographical clusters and commenced the QI programme in a random order, based on a computer-generated random sequence, over an 85-week period with one geographical cluster commencing the intervention every 5 weeks from the second to the 16th time period. Patients were masked to the study group, but it was not possible to mask hospital staff or investigators. The primary outcome measure was mortality within 90 days of surgery. Analyses were done on an intention-to-treat basis. This study is registered with the ISRCTN registry, number ISRCTN80682973. Findings: Treatment took place between March 3, 2014, and Oct 19, 2015. 22 754 patients were assessed for elegibility. Of 15 873 eligible patients from 93 NHS hospitals, primary outcome data were analysed for 8482 patients in the usual care group and 7374 in the QI group. Eight patients in the usual care group and nine patients in the QI group were not included in the analysis because of missing primary outcome data. The primary outcome of 90-day mortality occurred in 1210 (16%) patients in the QI group compared with 1393 (16%) patients in the usual care group (HR 1·11, 0·96–1·28). Interpretation: No survival benefit was observed from this QI programme to implement a care pathway for patients undergoing emergency abdominal surgery. Future QI programmes should ensure that teams have both the time and resources needed to improve patient care. Funding: National Institute for Health Research Health Services and Delivery Research Programme

    Effectiveness of a national quality improvement programme to improve survival after emergency abdominal surgery (EPOCH): a stepped-wedge cluster-randomised trial

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    BACKGROUND: Emergency abdominal surgery is associated with poor patient outcomes. We studied the effectiveness of a national quality improvement (QI) programme to implement a care pathway to improve survival for these patients. METHODS: We did a stepped-wedge cluster-randomised trial of patients aged 40 years or older undergoing emergency open major abdominal surgery. Eligible UK National Health Service (NHS) hospitals (those that had an emergency general surgical service, a substantial volume of emergency abdominal surgery cases, and contributed data to the National Emergency Laparotomy Audit) were organised into 15 geographical clusters and commenced the QI programme in a random order, based on a computer-generated random sequence, over an 85-week period with one geographical cluster commencing the intervention every 5 weeks from the second to the 16th time period. Patients were masked to the study group, but it was not possible to mask hospital staff or investigators. The primary outcome measure was mortality within 90 days of surgery. Analyses were done on an intention-to-treat basis. This study is registered with the ISRCTN registry, number ISRCTN80682973. FINDINGS: Treatment took place between March 3, 2014, and Oct 19, 2015. 22 754 patients were assessed for elegibility. Of 15 873 eligible patients from 93 NHS hospitals, primary outcome data were analysed for 8482 patients in the usual care group and 7374 in the QI group. Eight patients in the usual care group and nine patients in the QI group were not included in the analysis because of missing primary outcome data. The primary outcome of 90-day mortality occurred in 1210 (16%) patients in the QI group compared with 1393 (16%) patients in the usual care group (HR 1·11, 0·96-1·28). INTERPRETATION: No survival benefit was observed from this QI programme to implement a care pathway for patients undergoing emergency abdominal surgery. Future QI programmes should ensure that teams have both the time and resources needed to improve patient care. FUNDING: National Institute for Health Research Health Services and Delivery Research Programme

    Tipps und Tricks bei der Behandlung einer Epistaxis

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    How to treat an epistaxis: Tips and pitfalls Abstract. Nosebleed is a common and usually self-limiting symptom. Only 6 % of those affected need medical attention. Patients often present themselves to their family doctor or in an emergency setting. The aim of this article is to convey the important points in the diagnosis and therapy of epistaxis

    Surgical implications of 3D vs 2D endoscopic ear surgery: a case-control study

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    PURPOSE To compare 3D to 2D technology in endoscopic ear surgery (EES); to report surgeons' feedback on the use of 3D in EES; to describe the operative setting for 3D EES. METHODS A case-control study on EES was performed at a tertiary university center. All consecutive cases of 3D EES (case group) were matched to a control group operated with the standard 2D technique. Data on surgical approach, type of surgery, operative time, outcomes, and complications were compared between the two groups. After each surgery, the operating surgeons were asked to give a feedback on the use of 3D endoscopy, filling in a questionnaire based on 5-point Likert scales. RESULTS None of the 3D procedures was switched to 2D. Nor intraoperative or long-term complications were recorded. The operative time was similar in both groups. Postoperative hearing function did not show any statistically significant difference between 3 and 2D patients. Ninety-six percent of participants agreed or strongly agreed on better views of anatomy and pathology with the 3D technique. Discomfort induced by 3D vision was rarely reported. CONCLUSION Surgical and functional results from EES respectively performed with 3D and 2D systems are overall similar, suggesting that both techniques are safe and effective. According to the surgeons' feedback, 3D provides better depth perception and improved view of anatomy and pathology. Several surgeons are willing to use the 3D system for future EES. To guarantee the best 3D EES experience, the setting in the operating room plays a crucial role

    Sémont Maneuver for Benign Paroxysmal Positional Vertigo Treatment: Moving in the Correct Plane Matters.

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    HYPOTHESIS We aimed to investigate the effect of the head excursion angle on the success of the Sémont Maneuver (SM). BACKGROUND SM is performed with the head turned by 45 degrees toward the unaffected ear. In clinical routine, it is unlikely that physicians can turn the head to a position of exactly 45 degrees. Moreover, it is unclear how possible deviations from 45 degrees would affect the outcome with SM. METHODS We used an in vitro model (upscaled by ×5) of a posterior semicircular canal with canalithiasis to study head excursion angles (0-75 degrees) and minimum waiting times in SM. Additionally, we measured actual head excursion angles performed by trained physicians during SM on a healthy subject. RESULTS Successful canalith repositioning to the utricle was possible at head excursion angles between 21 and 67 degrees. Waiting time increased from 16 to 30 seconds with increasing deviation from 45 degrees. Angles larger than 67 degrees or smaller than 21 degrees did not lead to successful repositioning even after a waiting period of 5 minutes. Physicians set head excursion angles of 50 degrees ±SD 4.8 degrees while performing the SM. CONCLUSION Angular deviations up to ±20 degrees from the ideal SCC plane (45 degrees) still allows for successful SM. Although the tested physicians tended to underestimate the actual head excursion angle by 5 degrees (and more), the success of SM will not be affected provided that the waiting time is sufficiently long. Further, the results suggest that the Brandt-Daroff maneuver is a form of habituation training rather than a liberatory maneuver
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