Implementation of neuro-oncology service reconfiguration in accordance with NICE guidance provides enhanced clinical care for patients with glioblastoma multiforme.

BACKGROUND: Brain tumours account for <2% of all primary neoplasms but are responsible for 7% of the years of life lost from cancer before age 70 years. The latest survival trends for patients with CNS malignancies have remained largely static. The objective of this study was to evaluate the change in practice as a result of implementing the Improving Outcomes Guidance from the UK National Institute for Health and Clinical Excellence (NICE). METHODS: Patients were identified from the local cancer registry and hospital databases. We compared time from diagnosis to treatment, proportion of patients discussed at multidisciplinary team (MDT) meetings, treatment received, length of inpatient stay and survival. Inpatient and imaging costs were also estimated. RESULTS: Service reconfiguration and implementation of NICE guidance resulted in significantly more patients being discussed by the MDT--increased from 66 to 87%, reduced emergency admission in favour of elective surgery, reduced median hospital stay from 8 to 4.5 days, increased use of post-operative MRI from 17 to 91% facilitating early discharge and treatment planning, and reduced cost of inpatient stay from £2096 in 2006 to £1316 in 2009. Patients treated with optimal surgery followed by radiotherapy with concomitant and adjuvant temozolomide achieved outcomes comparable to those reported in clinical trials: median overall survival 18 months (2-year survival 35%). CONCLUSIONS: Advancing the management of neuro-oncology patients by moving from an emergency-based system of patient referral and management to a more planned elective outpatient-based pattern of care improves patient experience and has the potential to deliver better outcomes and research opportunities.

CpG island hypermethylation of the neurofibromatosis type 2 (NF2) gene is rare in sporadic vestibular schwannomas.

AIMS: Loss of both wild-type copies of the neurofibromatosis type 2 (NF2) gene is found in both sporadic and neurofibromatosis type 2-associated vestibular schwannomas (VS). Previous studies have identified a subset of VS with no loss or mutation of NF2. We hypothesized that methylation of NF2 resulting in gene silencing may play a role in such tumours. METHODS: Forty sporadic VS were analysed by array comparative genomic hybridization using 1 Mb whole genome and chromosome 22 tile path arrays. The NF2 genes were sequenced and methylation of NF2 examined by pyrosequencing. RESULTS: Monosomy 22 was the only recurrent change found. Twelve tumours had NF2 mutations. Eight tumours had complete loss of wild-type NF2, four had one mutated and one wild-type allele, 11 had only one wild-type allele and 17 showed no abnormalities. Methylation analysis showed low-level methylation in four tumours at a limited number of CpGs. No high-level methylation was found. CONCLUSIONS: This study shows that a significant proportion of sporadic VS (>40%) have unmethylated wild-type NF2 genes. This indicates that other mechanisms, yet to be identified, are operative in the oncogenesis of these VSs.

An emergency EVAR service reduces mortality in ruptured abdominal aortic aneurysms.

OBJECTIVES: The aim of this study was to compare all in-hospital mortality for ruptured abdominal aortic aneurysms (rAAAs) before and after the establishment of an emergency EVAR (eEVAR) service. DESIGN AND METHODS: An eEVAR service was established in January 2006, since when all patients presenting with rAAAs have been considered for endovascular repair. Data for all rAAAs presenting between January 2006 and December 2007 was prospectively collected (Group 1). This patient group was compared to those presenting with rAAA between January 2003 and December 2005 when eEVAR was not offered at our institution (Group 2). These records had also been collected prospectively and submitted to the National Vascular Database (NVD). RESULTS: A total of 50 rAAAs (17 eEVAR, 29 open repairs, 4 palliated) presented after the introduction of eEVAR (Group 1) and 71 in the historical Group 2 of which 54 underwent open repair and 17 were palliated. The total in-hospital mortality was significantly lower in Group 1 20% (eEVAR (n=1), 6%: Open (n=5), 17%: palliated (n=4), 100%) when compared to Group 2 54% (Open (n=21), 39%: palliated (n=17), 100%) (p=0.000001). Furthermore similar significant differences were seen in 30-day operative mortalities between the two groups 13% in Group 1 versus 39% in Group 2 (p=0.0003). In addition the proportion of patients who were palliated has significantly decreased (8% Group 1 versus 24% Group 2, p=0.01). CONCLUSIONS: The establishment of an eEVAR service has significantly reduced in-hospital mortality for patients presenting with ruptured abdominal aortic aneurysms.

Actinomycosis of the middle ear.

OBJECTIVE: To present the first case of middle-ear actinomycosis in the UK in the last 60 years. The diagnosis and management of actinomycosis of the middle ear is also presented, as well as a review of the recent literature. CASE REPORT: This paper reports the case of a nine-year-old girl who presented with recurrent otorrhoea caused by actinomycosis. Mastoid exploration with clearance of the infected tissue, in conjunction with six months amoxicillin, resulted in long-term disease remission. Histology revealed Gram-positive, silver avid (on Grocott staining) and diastase-resistant periodic acid-Schiff negative staining of colonies; this profile was in keeping with the characteristic 'sulphur granules' of actinomycosis. CONCLUSION: Actinomycosis of the middle ear and mastoid is rare, with less than 45 cases presented in the worldwide literature. This case confirms that the disease should be treated with full surgical clearance and long-term antibiotics.