Richard Gullan

Spinal meningiomas : a 20-year review

The long-term outcome of 78 patients with spinal meningiomas operated on over 20 years at a single neurosurgical unit was analysed. Age, sex and tumour location were similar to those reported by others. Overall, 95% of our patents were independently mobile postoperatively, despite 25% of the group being unable to walk before operation, including four paraplegic patients. Only two tumours were entirely extradural, and a further two were both intra- and extradural. In all cases, tumour exposure was by posterior laminectomy, without recourse to more complex approaches. Complete tumour resection was achieved in 77 (98%) of cases. The dural attachment was excised in 20 cases and diathermy was applied in 58. There was one recurrence, 14 years after the original surgery. Complex and technically challenging surgical approaches are unnecessary to obtain complete removal even for anteriorly placed tumours. Excision of the dural base would seem unnecessary to attain a low recurrence rate.

Cauda equina syndrome: outcome and implications for management.

A retrospective review of all patients who had surgery for cauda equina syndrome (CES) due to a herniated lumbar disc between January 1996 and November 1999 was undertaken. All underwent laminectomy and discectomy, and had been admitted as emergencies with cauda equina syndrome. Ten women and 10 men with a mean age of 45 years (range 33-67) had their diagnosis verified with MRI in 19 cases and CT in one case. Only half the patients had been catheterized at the time of admission to the neurosurgical unit. Nine patients had emergency decompressive surgery within 5 h of presentation to our unit. The others had surgery on the next available list, but within 24 h of admission. No difference was found between urgently operated patients and those operated on the next available list when urological outcome and quality of life assessments were made using a validated questionnaire at a mean time of 16 months after surgery (range 10-48). Twenty per cent of a control group who had undergone laminectomy and discectomy for large disc herniations, but without CES had new urological symptoms when questioned postoperatively, but similar quality of life status. Emergency decompressive surgery did no significantly improve outcome in CES compared with a delayed approach.

Pilocytic astrocytoma of the adult--clinical features, radiological features and management

Pilocytic astrocytoma is a histological subtype of astrocytoma classically located in the cerebellum of children and young adults. Cases occurring over the age of 30 years are rare and have not been classified in terms of their clinical features and management. Suitable cases were identified using diagnostic coding and by reviewing a neuropathology database. Casenotes and neuroradiology were reviewed retrospectively. Ten cases were identified over a 6 year period with an incidence of 0.49 cases per million population per year. Tumours were equally distributed between the supra- and infra-tentorial spaces. The most common symptom was headache occurring in 90%. No patient suffered seizures. Total macroscopic treatment remains the treatment of choice. Pilocytic astrocytoma of the adult is a rare tumour with a favourable prognosis.

The Early Effects of Radiotherapy on Intellectual and Cognitive Functioning in Patients with Frontal Brain Tumours: The Use of a New Neuropsychological Methodology

Investigations of the effects of radiation on neuropsychological functions have revealed variable outcomes, ranging from no effect to severe cognitive impairment. However, many of the previous studies have relied on retrospective data or have been limited by methodological problems. In this study, prospective neuropsychological assessments were compared at baseline (after surgery and before radiotherapy) and within 4 months of completion of radiotherapy (except one case), to examine early-delayed effects of radiation on intellectual and cognitive functioning. Sixteen adult patients with either low- or high-grade brain tumours, 15 of whom were treated with radiotherapy, were compared with 8 control participants with nonmalignant brain tumours whom did not undergo radiotherapy. All participants had lesions situated mainly in the frontal lobes. All groups of patients had evidence of intellectual and cognitive impairment at baseline. The low- and high-grade brain tumour groups showed a differential pattern of performance following radiotherapy, with the low-grade tumour groups performance being more competent on all of the five main neuropsychological measures. Their pattern of improvement was very similar to that of the nonmalignant brain tumour group who had not undergone radiotherapy. The present study provides some preliminary information about the neuropsychological deficits associated with primary brain tumours, their severity, and the relationship between neuropsychological functioning and brain tumours and radiotherapy.

Primary glioblastoma with oligodendroglial differentiation has better clinical outcome but no difference in common biological markers compared with other types of glioblastoma

BACKGROUND Glioblastoma multiforme with an oligodendroglial component (GBMO) has been recognized in the World Health Organization classification-however, the diagnostic criteria, molecular biology, and clinical outcome of primary GBMO remain unclear. Our aim was to investigate whether primary GBMO is a distinct clinicopathological subgroup of GBM and to determine the relative frequency of prognostic markers such as loss of heterozygosity (LOH) on 1p and/or 19q, O(6)-methylguanine-DNA methyltransferase (MGMT) promoter methylation, and isocitrate dehydrogenase 1 (IDH1) mutation. METHODS We examined 288 cases of primary GBM and assessed the molecular markers in 57 GBMO and 50 cases of other primary GBM, correlating the data with clinical parameters and outcome. RESULTS GBMO comprised 21.5% of our GBM specimens and showed significantly longer survival compared with our other GBM (12 mo vs 5.8 mo, P = .006); there was also a strong correlation with younger age at diagnosis (56.4 y vs 60.6 y, P = .005). Singular LOH of 19q (P = .04) conferred a 1.9-fold increased hazard of shorter survival. There was no difference in the frequencies of 1p or 19q deletion, MGMT promoter methylation, or IDH1 mutation (P = .8, P = 1.0, P = 1.0, respectively). CONCLUSIONS Primary GBMO is a subgroup of GBM associated with longer survival and a younger age group but shows no difference in the frequency of LOH of 1p/19q, MGMT, and IDH1 mutation compared with other primary GBM.

Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft part sarcoma (ASPS) is a rare but well‐studied subtype of sarcoma. A 39‐year‐old man presented with seizures due to a left temporal meningeal‐enhancing lesion with striking brain edema on MRI. The patient underwent neurosurgical resection for suspected meningioma. Histology showed large tumor cells clustering and forming small nests, in places with pseudoalveolar pattern. Diastase‐resistant periodic acid‐Schiff revealed very rare granular and rod‐like cytoplasmic inclusions. Immunohistochemistry showed convincing positivity only with vimentin and smooth muscle actin. The histological features were strongly suggestive of ASPS. At the molecular level RT‐PCR and sequencing analysis demonstrated ASPCR1‐TFE3 fusion confirming the histological diagnosis of ASPS. There was no evidence of primary extracranial tumor by physical examination and on chest and abdominal CT scan 11 months after presentation. ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course. This case may represent primary meningeal ASPS although metastatic deposit from an undiscovered primary site cannot be entirely excluded.

Management of infratentorial subdural hygroma complicating foramen magnum decompression: a report of three cases

Decompression of the foramen magnum for symptomatic Chiari malformation attends a small but significant risk of infratentorial subdural extra-arachnoid hygroma when an arachnoid-sparing procedure is attempted. We present three cases whereby an arachnoid-sparing procedure was carried out and resulted in infratentorial subdural hygroma and hydrocephalus. The complication was managed by re-exploration of the posterior fossa and wide arachnoidotomy. In cases whereby the decision has been made to open the dura, we recommend routine arachnoidotomy in foramen magnum decompression, avoiding the risks of infratentorial subdural hygroma. In cases where arachnoid-sparing procedures have been attempted and subdural hygroma subsequently develops, we advocate re-exploration of the posterior fossa rather than cerebrospinal fluid diversion.

Active dendritic cell immunotherapy for glioblastoma: Current status and challenges

Abstract Dendritic cell (DC) immunotherapy is developing as a promising treatment modality for patients with glioblastoma multiforme (GBM). The aim of this article is to review the data from clinical trials and prospective studies evaluating the safety and efficacy of DC vaccines for newly diagnosed (ND)- and recurrent (Rec)-GBM and for other high-grade gliomas (HGGs). By searching all major databases we identified and reviewed twenty-two (n = 22) such studies, twenty (n = 20) of which were phase I and II trials, one was a pilot study towards a phase I/II trial and one was a prospective study. GBM patients were exclusively recruited in 12/22 studies, while 10/22 studies enrolled patients with any diagnosis of a HGG. In 7/22 studies GBM was newly diagnosed. In the vast majority of studies the vaccine was injected subcutaneously or intradermally and consisted of mature DCs pulsed with tumour lysate or peptides. Median overall survival ranged between 16.0 and 38.4 months for ND-GBM and between 9.6 and 35.9 months for Rec-GBM. Vaccine-related side effects were in general mild (grade I and II), with serious adverse events (grade III, IV and V) reported only rarely. DC immunotherapy therefore appears to have the potential to increase the overall survival in patients with HGG, with an acceptable side effect profile. The findings will require confirmation by the ongoing and future phase III trials.

Spontaneous Angiographic Disappearance of an Intracranial Arteriovenous Malformation

A deep right hemisphere AVM was demonstrated by angiography in an 8-year-old girl soon after her presentation with a subarachnoid haemorrhage. Eight years later the AVM was no longer visible on angiography. During the interval she had been completely asymptomatic and had received no treatment. There was no residual neurological deficit or epilepsy. A similar case has previously been described and is discussed briefly along with other cases of complete AVM regression.

Resection of olfactory groove meningioma - a review of complications and prognostic factors

Introduction. High complication rates have been cited following olfactory groove meningioma (OGM) resection but data are lacking on attendant risk factors. We aimed to review the complications following OGM resection and identify prognostic factors. Methods. A retrospective review was performed on 34 consecutive patients who underwent primary OGM resection at a single London institution between March 2008 and February 2013. Collected data included patient comorbidities, pre-operative corticosteroid use, tumour characteristics, imaging features, operative details, extent of resection, histology, use of elective post-operative ventilation, complications, recurrence and mortality. Results. Complication rate was 39%. 58% of complications required intensive care or re-operation. Higher complication rates occurred with OGM > 40 mm diameter versus ≤ 40 mm (53 vs. 28%; p = 0.16); OGM with versus without severe perilesional oedema (59 vs. 19%; p = 0.26), more evident when corrected for tumour size; and patients receiving 1–2 days versus 3–5 days of pre-operative dexamethasone (75 vs. 19%; p = 0.016). Patients who were electively ventilated post-operatively versus those who were not had higher risk tumours but a lower complication rate (17 vs. 44%; p = 0.36) and a higher proportion making a good recovery (83 vs. 55%; p = 0.20). Complete versus incomplete resection had a higher complication rate (50 vs. 23%; p = 0.16) but no recurrence (0 vs. 25%; p = 0.07). Conclusion. Risk of morbidity with OGM resection is high. Higher complication risk is associated with larger tumours and greater perilesional oedema. Pre-operative dexamethasone for 3–5 days versus shorter periods may reduce the risk of complications. We describe a characteristic pattern of perilesional oedema termed ‘sabre-tooth’ sign, whose presence is associated with a higher complication rate and may represent an important radiological prognostic sign. Elective post-operative ventilation for patients with high-risk tumours may reduce the risk of complications.