Search

Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine carcinoma. It occurs predominantly in the head and neck area and often behaves aggressively. In this single-institution retrospective observational cohort study, we describe the results of a treatment strategy that we developed over the past decades. Endpoints of this study were local, regional and distant control, disease-specific survival and overall survival. In total 47 patients with head and neck MCC, diagnosed in the Netherlands Cancer Institute-Antoni van Leeuwenhoek (NKI-AvL) between 1984 and 2012, were included in this study. Local tumor control was 82 % (95 % CI 71-95 %) at 5 years. Regional lymph node metastases were found at the moment of diagnosis in 13 cases (28 %). In the group of patients who were initially cN0, the 5-year regional control was 80 % (68-95 %). The 5-year metastasis-free interval probability was 80 % (68-94 %). The disease-specific survival (DSS) at 5 years was 70 % (56-86 %). An overall survival of 54 % (40-72 %) was found at 5-year follow-up and of 37 % (23-59 %) at 10-year follow-up. Univariable Cox regression analysis of many clinical and pathological variables did not identify any predictors for DSS. The MCC has a high propensity for locoregional and distant spread in the head and neck region. Undertreatment, especially of the lymph nodes in the neck, is a serious problem as regional (micro)metastasis are common even in T1 tumors. Future research will have to elucidate the role of the sentinel lymph node procedure versus the elective selective node dissection and standardized elective local and regional radiotherapy in the head and neck area.

The objective of this study is to evaluate the functional and aesthetic results obtained from the use of the lateral crural underlay spring (LCUS) graft for the treatment of internal and external nasal valve collapse. In this retrospective study, preoperative and postoperative functional and aesthetic results were compared in patients undergoing treatment for internal or external nasal valve collapse. Results were scored by means of a questionnaire and visual analog scale completed by the patients. Eight patients were recruited and included in this study: 6 (75%) had an improvement in their functional scores, 1 (12%) remained unchanged, and 1 (12%) scored worse. The mean difference in functional scores after the intervention was 9.4 points (p < 0.005). There was no significant difference in aesthetic scores. We found evidence that the LCUS graft is effective for relieving nasal obstruction in patients with internal, external, or combined nasal valve collapse. The amount of increased sidewall tension and rigidity as well as the increase in nasal valve angle and cross-sectional area are determined by the length of the graft, which can be varied according to need., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

A 13-year-old patient was referred to the ENT department because of a ring-shaped radiopaque foreign body in her right nasal cavity demonstrated on an orthopantomogram. Her medical history revealed long-standing right-sided rhinorrhea thought to be caused by an inhalation allergy. In case of unilateral rhinorrhea in a child a nasal foreign body has to be excluded.

Objectives: The aim of this study was to formulate a predictive rule for vestibular schwannoma growth during the initial observation period after diagnosis., Methods: Logistic regression models were fitted, with tumor growth in the first year as the dependent variable and patient characteristics as the independent variables. Backward selection was used to eliminate superfluous predictors. The area under the receiver operating characteristic curve was taken as a measure of the model's discriminative power., Results: Eventually, the model or rule consisted of 4 significant growth predictors: localization (if extrameatal, +1; if intrameatal, 0), sudden sensorineural hearing loss (if present, -1; if absent, 0), balance symptoms (if present, +1; if absent, 0), and complaints of hearing loss for less than 2 years (if present, +1; if absent, or present for more than 2 years, 0). A higher score indicates a higher likelihood of tumor growth during the period of observation after diagnosis. If the total score is 0 or less, the likelihood of tumor growth during the first year after diagnosis is less than 10%. If the score is 3, the likelihood of growth during the first year after diagnosis is more than 70%., Conclusions: We were able to create a useful rule to predict vestibular schwannoma growth during the first year after diagnosis.

Objectives/hypothesis: Gamma knife radiosurgery (GKRS) has become an important treatment modality for vestibular schwannomas. The primary aim of this study was to investigate whether tumor growth at the moment of GKRS has any correlation with the outcome. The secondary aim was to identify clinical predictors of radioresistance in vestibular schwannoma patients treated with GKRS., Study Design: One hundred vestibular schwannoma patients, treated with GKRS, were divided into two groups: 1) proven tumor growth preceding GKRS; and 2) previous history of growth unknown. GKRS outcome was defined in two ways. According to the first definition, GKRS was said to have failed when additional treatment had taken place. According to the second one, a volume decrease >20% after 2 years marked successful treatment., Methods: Correlations between outcome and growth status were determined with SPSS software. Furthermore, the study assessed how different variables (patient data, history, tumor characteristics, imaging, and audiovestibular examinations) correlated with the outcome of GKRS., Results: No significant difference regarding success and failure of GKRS was found between the two patient groups. The mean reduction in tumor volume after GKRS was less pronounced in patients in whom tumor growth was demonstrated before treatment, but this finding was not significant. No significant predictors (P < .05) could be identified in this data set., Conclusions: This study found no indication that growth at the moment of GKRS influences therapeutic outcome, nor did it identify any predictors of the outcome after GKRS in vestibular schwannoma patients., (Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.)

Introduction: Treatment strategies for vestibular schwannoma include microsurgery, stereotactic radiotherapy and conservative management (wait and scan). To avoid neurological complications or even death, surgery is the preferred treatment for large tumours with an extrameatal diameter > 3.0 cm., Objective/methods: We present the case history of a man with a large vestibular schwannoma who had refused treatment and was seen again eighteen years later., Results: This patient had not developed symptoms other than the initial hearing loss. Repeated imaging showed that the tumour had not grown and the brainstem compression had not progressed., Conclusion: This case history illustrates the unpredictable growth pattern of vestibular schwannomas. Apparently, even large tumours in close proximity to the brainstem may remain stable for many years. However, there still are no valid arguments to refrain from therapy in patients with a large vestibular schwannoma, since reliable growth predictors are not available.

This study evaluates the impact of gamma knife radiosurgery (GKRS) on the quality of life (QOL) of patients with a sporadic vestibular schwannoma (VS). This study pertains to 108 VS patients who had GKRS in the years 2003 through 2007. Two different QOL questionnaires were used: medical outcome study short form 36 (SF36) and Glasgow benefit inventory (GBI). Radiosurgery was performed using a Leksell 4C gamma knife. The results of the QOL questionnaires in relation to prospectively and retrospectively gathered data of the VS patients treated by GKRS. Eventually, 97 patients could be included in the study. Their mean tumor size was 17 mm (range 6-39 mm); the mean maximum dose on the tumor was 19.9 Gy (range 16-25.5 Gy) and the mean marginal dose on the tumor was 11.1 (range 9.3-12.5 Gy). SF36 scores showed results comparable to those for a normal Dutch population. GBI showed a marginal decline in QOL. No correlation was found between QOL and gender, age, tumor size, or radiation dose. Increased audiovestibular symptoms after GKRS were correlated with a decreased GBI score, and decreased symptoms were correlated with a higher QOL post-GKRS. In this study shows that GKRS for VS has little impact on the general QOL of the VS patient. However, there is a wide range in individual QOL results. Individual QOL was influenced by the audiovestibular symptoms. No predictive patient, tumor, or treatment factors for QOL outcome after GKRS could be determined. Comparison with microsurgery is difficult because of intra group variability.

Objectives/hypothesis: This study was designed to evaluate hearing preservation after gamma knife radiosurgery (GKRS) and to determine the relation between hearing preservation and cochlear radiation dose in patients with a sporadic vestibular schwannoma (VS)., Methods: Prospective study involving patients suffering from VS who received GKRS from June 2003 until November 2007. Pure tone and speech audiometry were conducted before and after GKRS. The thresholds at pure tone audiometry were taken as a measure of hearing. Pure tone average (PTA) was defined as the mean threshold at 0.5 kHz, 1.0 kHz, 2.0 kHz, and 4.0 kHz. Hearing was classified according to the 2003 consensus meeting in Tokyo. Stereotactic surgery was performed using a Leksell 4C Gamma Knife (Elekta, Stockholm, Sweden)., Results: A total of 69 patients were included in the study. Mean tumor size was 17 mm. Mean marginal dose at the tumor was 11.0 Gy (range, 9.3 Gy-12.3 Gy), mean maximal dose was 19.7 Gy (range, 16 Gy-25.5 Gy). Mean maximal dose at the cochlea was 10.27 Gy (range, 3.1 Gy-16.1 Gy), and mean minimal dose at the cochlea was 2.6 Gy (range, 0.9 Gy-7.4 Gy). Mean PTA before GKRS was 43 dB (standard deviation [SD] 20 dB), mean PTA after GKRS was 63 dB (SD 30 dB). Mean interval between pre-GKRS audiometry and GKRS was 8.0 months. Between GKRS and post-GKRS audiometry, mean interval was 14.2 months. Hearing was considered to be preserved (max +1 class, Tokyo classification) in 52 (75%) of 69 patients. However, only 32 patients had class A, B, or C (serviceable hearing) before GKRS. Within this group, only 13 patients (41%) had a hearing class A, B, or C after GKRS. A significant relation was found between the maximal cochlear dose and the difference in PTA before and after GKRS., Conclusions: Hearing preservation is correlated to the maximal radiation dose at the cochlea. The purpose of developing GKRS techniques was to avoid collateral damage in healthy tissues. This study emphasizes the need for exact radiation planning to reduce the cochlear radiation dose if the hearing is to be preserved. Laryngoscope, 2009.

Management of a sporadic vestibular schwannoma (VS) is still a subject of controversy, mainly due to distinct and unpredictable growth patterns. To embark on an appropriate therapy it is necessary to dispose of a reliable prediction about tumor progression. This study aims to design a risk profile with predictors for VS growth. A total of 234 VS patients who were managed conservatively were included. Data concerning (duration of) symptoms and localization of VS were analyzed with Cox proportional hazards regression models. Predictors for growth are unsteadiness/vertigo, no sudden onset of hearing loss and short duration of hearing loss. High-risk patients have (1) VS with an extrameatal localization, short duration of hearing loss and at least one of the two other predictors (unsteadiness/vertigo or no sudden sensorineural hearing loss) or (2) VS with an intrameatal localization and all three other predictors. Low-risk patients have (1) VS with an extrameatal component and no other predictor or (2) VS with an intrameatal localization and at most one other predictor. High-risk patients have a risk of growth of 36.9% in the first year and 64.6% in the second year. For patients with a low risk this is 2.5 and 12.7%, respectively. Simple data gathered at the moment of diagnosis may provide useful information since they may lead to a risk profile for growth.

Objective: The objective of this study was to explore the useful of vestibular evoked myogenic potential (VEMP) testing for detecting endolymphatic hydrops, especially in the second ear of patients with unilateral Ménière disease (MD)., Methods: This study was performed at a tertiary care academic medical center. Part I consisted of postmortem temporal bone specimens from the temporal bone collection of the Massachusetts Eye & Ear Infirmary; part II consisted of consecutive consenting adult patients (n = 82) with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery criteria case histories. Outcome measures consisted of VEMP thresholds in patients and histologic saccular endolymphatic hydrops in postmortem temporal bones., Results: Saccular hydrops was observed in the asymptomatic ear in six of 17 (35%) of temporal bones from donors with unilateral MD. Clinic patients with unilateral MD showed elevated mean VEMP thresholds and altered VEMP tuning in their symptomatic ears and, to a lesser degree, in their asymptomatic ears. Specific VEMP frequency and tuning criteria were used to define a "Ménière-like" response. This "Ménière-like" response was seen in 27% of asymptomatic ears of our patients with unilateral MD., Conclusions: Bilateral involvement is seen in approximately one third of MD cases. Saccular hydrops appears to precede symptoms in bilateral MD. Changes in VEMP threshold and tuning appear to be sensitive to these structural changes in the saccule. If so, then VEMP may be useful as a detector of asymptomatic saccular hydrops and as a predictor of evolving bilateral MD.

Objective: In this retrospective study, we tested the hypothesis that vestibular evoked myogenic potential (VEMP) thresholds are more often elevated or absent in patients with Ménière's disease experiencing Tumarkin drop attacks than in other patients with Ménière's disease., Methods: Subjects included normal subjects (n = 14) and patients with unilateral Ménière's disease by AAO-HNS (1995) diagnostic criteria with (n = 12) and without (n = 82) Tumarkin drop attacks at a large specialty hospital otology service. VEMP threshold testing was conducted using 250, 500, and 1,000 Hz tone burst stimuli., Results: VEMP responses were present in at all frequencies in both ears of all normal subjects. In unaffected ears of patients with unilateral Ménière's disease, VEMPs were undetectable in 13% of measurements attempted. This number rose to 18% in affected ears of patients with unilateral Ménière's disease and to 41% in Meniere ears with Tumarkin drop attacks. Frequency tuning of the VEMP response in normal subjects showed lowest thresholds at 500 Hz. In Meniere ears, the tuning was altered such that the 500-Hz thresholds were higher than the 1,000-Hz thresholds. There was a gradient of threshold elevation and altered tuning that corresponded to the gradient of worsening disease., Conclusion: Our findings support the hypothesis that Tumarkin drop attacks arise from advanced disease involving the saccule and that VEMP may be a clinically valuable metric of disease severity or progression in patients with Ménière's disease.

In this study the prevalence of nocturnal enuresis defined according to the DSM III criteria was determined in a random sample of 2070 children aged from 4 to 16 years. Nocturnal enuresis declines in prevalence with age and is more frequent in boys than in girls. Moreover, the decline in prevalence with age was found to take place earlier in girls than in boys. It is therefore argued that the DSM III age limit for enuresis should be raised to 8 years for boys.