Quality of life outcomes in incidental and operated meningiomas (QUALMS): a cross-sectional cohort study.

INTRODUCTION: Few studies have evaluated meningioma patients' longer-term health-related quality of life (HRQoL) following diagnosis and treatment, particularly in those with incidental, actively monitored tumours. METHODS: A single-center, cross-sectional study was completed. Adult patients with surgically managed or actively monitored meningioma with more than five years of follow-up were included. The patient-reported outcome measures RAND SF-36, EORTC QLQ-C30 and QLQ-BN20 were used to evaluate HRQoL. HRQoL scores were compared to normative population data. Outcome determinants were evaluated using multivariate linear regression analysis. RESULTS: 243 patient responses were analyzed, and the mean time from diagnosis was 9.8 years (range 5.0-40.3 years). Clinically relevant, statistically significant HRQoL impairments were identified across several SF-36 and QLQ-C30 domains. Increasing education level (ß = 2.9, 95% CI 0.9 to 4.9), P = .004), employment (ß = 7.7, 95% CI 2.2 to 13.1, P = .006) and absence of postoperative complications (ß=-6.7, 95% CI -13.2 to (-)0.3, P = .041) were associated with a better QLQ-C30 summary score. Other tumour and treatment variables were not. CONCLUSION: This study highlights the longer-term disease burden of patients with meningioma nearly one decade after diagnosis or surgery. Patients with actively monitored meningioma have similar HRQoL to operated meningioma patients. Healthcare professionals should be mindful of HRQoL impairments and direct patients to sources of support as needed.

Development and Growth of Intracranial Meningiomas in Transgender Women Taking Cyproterone Acetate as Gender-Affirming Progestogen Therapy: A Systematic Review.

Background: Gender-affirming hormone therapy is critical to the management of transgender persons. Cyproterone acetate (CPA) is a synthetic, progesterone-like compound commonly used in high doses as gender-affirming progestogen therapy in transgender women. An association between high-dose CPA and the development and growth of intracranial meningioma, including case reports in transgender women, has been described. This systematic review summarizes these cases at the patient level and discusses their management. Methods: This systematic review was registered with PROSPERO (CRD42020191965). A detailed search of the PubMed, EMBASE, and Web of Science electronic bibliographic databases was performed (inception-December 20, 2020). Two review authors independently completed screening, data extraction, and risk of bias assessment in duplicate. Results: Nine records were included describing (n=12) individual case reports and (n=35) intracranial meningiomas. The median age at presentation was 48 years (interquartile range [IQR], 43-55 years), most frequent daily CPA doses were 50 mg/day (n=5) and 100 mg/day (n=5), and the median duration of CPA use was 9.5 years (IQR, 6.5-17.5 years). Multiple meningiomas were common (n=7). For most cases (n=10), surgical resection was the initial preferred management strategy, but two were successfully managed by CPA cessation. Conclusions: Transgender women receiving high doses of CPA may be at increased risk of intracranial meningioma development and/or growth, although this remains a rare disease. For presumed CPA-associated meningioma, drug cessation appears to be an appropriate management strategy when surgery is not imminently required to manage raised intracranial pressure or prevent neurological deterioration. Given the importance of gender-affirming hormone therapy to transgender persons, a suitable alternative hormone regimen should be offered, although the use of CPA in both high doses and for prolonged periods of time is now in decline.

The growth rate and clinical outcomes of radiation induced meningioma undergoing treatment or active monitoring.

INTRODUCTION: Radiation induced meningioma (RIM) incidence is increasing in line with improved childhood cancer survival. No optimal management strategy consensus exists. This study aimed to delineate meningioma growth rates from tumor discovery and correlate with clinical outcomes. METHODS: Retrospective study of patients with a RIM, managed at a specialist tertiary neuroscience center (2007-2019). Tumor volume was measured from diagnosis and at subsequent interval scans. Meningioma growth rate was determined using a linear mixed-effects model. Clinical outcomes were correlated with growth rates accounting for imaging and clinical prognostic factors. RESULTS: Fifty-four patients (110 meningiomas) were included. Median duration of follow-up was 74 months (interquartile range [IQR], 41-102 months). Mean radiation dose was 41 Gy (standard deviation [SD] = 14.9) with a latency period of 34.4 years (SD = 13.7). Median absolute growth rate was 0.62 cm3/year and the median relative growth rate was 72%/year. Forty meningiomas (between 27 patients) underwent surgical intervention after a median follow-up duration of 4 months (IQR 2-35). Operated RIMs were clinically aggressive, likely to be WHO grade 2 at first resection (43.6%) and to progress after surgery (41%). Median time to progression was 28 months (IQR 13-60.5). A larger meningioma at discovery was associated with growth (HR 1.2 [95% CI 1.0-1.5], P = 0.039) but not progression after surgery (HR 2.2 [95% CI 0.7-6.6], P = 0.181). Twenty-seven (50%) patients had multiple meningiomas by the end of the study. CONCLUSION: RIMs exhibit high absolute and relative growth rates after discovery. Surgery is recommended for symptomatic or rapidly growing meningiomas only. Recurrence risk after surgery is high.

Growth Patterns of Residual Tumor in Preoperatively Growing Vestibular Schwannomas.

Objectives To analyze growth of residual vestibular schwannoma (VS) following incomplete tumor resection and determine the influence of residual location and size. Design Retrospective case note and scan review. Setting Tertiary skull base unit. Participants Patients with residual tumor following primary surgery for medium and large unilateral growing vestibular schwanomas between 2006 and 2009. Main Outcome Measures Location of residual VS and post-operative growth, comparing those with more (>5%) or less than 5% of tumor residual (<5%). Results Fifty-two patients had visible residual tumor left behind at surgery. Twenty had < 5% and 32 had > 5% residual. The residual growth rates were 38% overall, 20% in < 5%, and 50% in > 5% residuals. There was no significant difference in growth rates at different residual locations. Median follow-up was 6.4 years. Conclusions There is a greater risk of regrowth of residuals > 5%. All positions of residual tumor can regrow, and the preoperative tumor size plays a role in this. Further data is needed to confirm if residual tumor in the fundus is less likely to grow.

Review of radiation therapy services for neurofibromatosis (NF2) patients in England.

OBJECTIVE: It is widely accepted that stereotactic radiosurgery (SRS) is less effective in controlling NF2-related vestibular schwannomas (VS-NF2), than sporadic VS. Concerns persist regarding the risks of malignant transformation and the development of secondary tumours. In recent years there has been an increase in the number of centres offering SRS and fractionated stereotactic radiotherapy (SRT) services for VS. The NF2 National Commissioning Group service framework has recommended limiting radiation therapies for VS-NF2 to SRS (Gamma Knife) provided in Sheffield. The aims of this review were to: 1) Establish the current provision of SRT and SRS services in England. 2) Determine the radiation therapies employed for NF2 patients since 2000. 3) Determine the feasibility of identifying a national cohort of NF2 patients in England who have received radiation therapy since 2000. METHODS: The lead clinicians managing VS patients in the major regional neurosurgical units in England, and those providing SRS services to NF2 patients were contacted and when possible, databases were examined. RESULTS: A total of 18 NHS centres and 2 private centres were included. This included the four NF2 'hub centres' (Cambridge, London, Oxford and Manchester). Their NF2 databases identified 4, 8, 23 and 42 VS-NF2 treatments with SRS or SRT, respectively since 2000. Eleven centres reported that they had referred VS-NF2 patients exclusively to Sheffield. Each estimated that they had referred no more than one patient per year. The survey identified four Gamma Knife Centres and six Linac SRS/SRT centres with the capacity to treat VS and VS-NF2 patients. Of these, four centres confirmed that they had treated VS-NF2 patients. CONCLUSION: Since 2000 fewer than 100 SRS and SRT treatments have been undertaken for VS-NF2 patients. Approximately 60% of these have been performed in Sheffield. There is considerable uncertainty regarding the role of radiation therapy in the management of VS-NF2 and consequently a range of views and practice.

Impulse control disorder in a patient on long-term treatment with bromocriptine for a macroprolactinoma.

Impulse control disorders (ICDs) constitute socially disruptive behaviors such as pathological gambling, impulsive eating, compulsive shopping, and hypersexuality. These conditions are well recognized in patients on dopamine agonist (DA) therapy for Parkinson disease. Dopamine agonists are widely used as first-line agents in the treatment of prolactinomas, but ICDs in this group of patients are relatively rare, perhaps because of lower therapeutic doses used. A review of the literature yielded only a few cases of ICDs in patients on DA treatment for prolactinomas. These symptoms are perhaps underreported because of lack of awareness among patients and health care professionals. Impulse control disorders are recognized psychiatric disorders that have significant psychological and social implications, and patients need to be counselled about this rare possibility when embarking on prolonged DA therapy. We describe a young patient with severe, socially disruptive impulsivity manifesting with pathological gambling who had been on long-term bromocriptine therapy for a macroprolactinoma.

Brain biopsy in cryptogenic neurological disease.

OBJECTIVE: Brain biopsy is standard clinical practice when CNS malignancy is suspected. Its value has not otherwise been clearly established. We reviewed the indications for, complications and outcome of diagnostic brain biopsies performed between 2003 and 2008 in a single UK centre. METHODS: Subjects were retrospectively identified using theatre log books and histopathology reports. Case records were analysed by a neurologist and neurosurgeon. Cases were excluded when the pre-operative diagnosis was clearly malignancy or infection. RESULTS: Of all (432) brain biopsies performed, 56 were performed in 52 patients with cryptogenic neurological disease. There were no permanent deficits or deaths. Histopathological reports were classified as definitive (45%), suggestive (20%) or non-diagnostic (36%). Brain biopsy made an immediate contribution to determination of diagnosis in 55% (31 of 56) and a confident diagnosis was eventually made in 40 of 52 patients (77%). Management was altered as a consequence of biopsy in 63%. Successful biopsy of a radiologically identified target increased the proportion of biopsies considered diagnostic to 78% (odds ratio 8.9) whereas non-targeted biopsy was non-diagnostic in 71%. Although a significant proportion of patients died or had progressive disease, this was not uniformly the case; 31% stabilised and 27% improved. CONCLUSION: We present the highest reported frequency of brain biopsy for cryptogenic neurological disease. The risk associated with the procedure was low and the biopsy results impacted significantly upon diagnosis and management. We therefore propose that the procedure should no longer be considered one of last resort.

An account of the life and achievements of Miss Diana Beck, neurosurgeon (1902-1956).

DIANA BECK (1902-1956) WAS probably the first female neurosurgeon in the world and played a large part in establishing neurosurgery in the United Kingdom. She served as the consultant advisor in neurosurgery for the Emergency Medical Service in the southwest region during the Second World War and subsequently set up the neurosurgical unit at Frenchay Hospital in Bristol before becoming a consultant at The Middlesex Hospital in London in 1947. There, she was the first woman to be given charge of men in a consultant capacity in a major London teaching hospital. During her 31-year career, she was the only female neurosurgeon in Western Europe and the United States. Miss Beck came to the attention of the general public when she operated on Alan Alexander Milne in 1952. However, she was better known in neurosurgical circles for her interest in intracranial hemorrhage. In this article, some of her original research, case reports, and her seminal paper on the surgical treatment of intracerebral hemorrhage are presented. They are a stark reminder of the limited investigations available at that time. In addition to her neurosurgical achievements, Miss Beck was a remarkable character and teacher. Her Saturday ward rounds proved so popular that they competed successfully with the students' weekend plans. In a specialty in which women continue to represent a disproportionately small percentage of the workforce, Miss Beck was one of our earliest role models.