IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma.

Hypertrophic pachymeningitis is a rare disorder of the dura mater of the spine or brain. It can be caused by inflammatory, infective or neoplastic conditions or can be idiopathic. We report a man with hypertrophic pachymeningitis and bilateral chronic subdural haematoma caused by IgG4-related disease. We highlight the diagnostic challenges and discuss possible underlying mechanisms of subdural haematoma formation in inflammatory conditions. Isolated IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma is very rare; previously reported cases have suggested a possible predilection for men in their sixth decade, presenting with headache as the dominant symptom. Given the rarity and complexity of the condition, it should be managed in a multidisciplinary team setting.

Adult WHO grade II ependymomas: is Ki67 a sex-specific proliferation marker?

Ki67 is a marker for proliferation of a given cell population. Low expression of Ki67 may be associated with a favourable outcome. We investigate how the proliferation index correlates with the location, morphology and behaviour of WHO grade II ependymomas with a single-centre cohort study of adult patients admitted for surgery of WHO grade II ependymomas between 2008 and 2018. Seventeen patients were included, seven had supratentorial and 10 had infratentorial tumours. Three patients died and eight had recurrent disease. Age, gender, location, extent of resection, chemotherapy, radiotherapy and histological markers were not associated with tumour progression. Both unadjusted and adjusted analysis confirmed a higher Ki67 index in male patients. Sensitivity analysis further supported the correlation between Ki67 and male gender. Ki67 may be sex specific but does not seem to correlate with survival and time to recurrence in this series.

Somatotopy of corticospinal tract fibres of the intercostal muscles: report of a case and review of literature.

This study aimed at describing the first case of subcortical stimulation of the corticospinal tract leading to selective contraction of the intercostal muscles during surgery for removal of a tumour centred in the right central lobule/supplementary motor area. A 53-year-old male presented with partial motor seizures. Imaging demonstrated a low-grade glioma affecting the posterior aspect of the superior and middle frontal gyri and invading the precentral gyrus. Preoperative motor Transcranial Magnetic Stimulation and advanced diffusion tractography were performed to establish the relationship of the tumour with the motor cortex and corticospinal tract. Intraoperative motor mapping and monitoring were performed with monopolar stimulation ("train of 5" technique). At the posterior margin of resection, subcortical stimulation demonstrated a selective response from intercostal muscles, medial to responses from the lower limb and lateral to responses from the upper limb. PubMed literature search was performed to identify any case reporting similar findings. There were no cases previously reported in the literature. The location of the subcortical response for intercostal muscles confirms the somatotopy of the corticospinal tract. Intercostal muscles are controlled by selective fibres within the corticospinal tract. Damage to these fibres can lead to paralysis of voluntary respiratory muscles. Further studies are needed to define the cortico-subcortical network controlling voluntary respiratory muscles.

5-ALA fluorescence in intraparenchymal endodermal cysts.

In recent years, new indications have been suggested for 5-ALA, particularly for cystic lesions. We report the use of 5-ALA fluorescence in an intraparenchymal supratentorial endodermal cyst of a 52-year-old female presenting with headache, progressive right side hemiparesis and anomic aphasia. She underwent an image-guided 5-ALA-assisted left minicraniotomy for fenestration of the cystic lesion into the ventricular system. The capsule of the cyst was noted to fluoresce with 5-ALA. She recovered from the previous deficits and the cyst decreased in size. To the best of our knowledge, this is the first time 5-ALA fluorescence is reported in a case of endodermal cyst.

Third-ventricle enterogenous cyst presentation mimicking a colloid cyst: uncommon presentation of a rare disease and literature review.

Enterogenous cysts (ECs) are endodermal lesions resulting from splitting anomalies in the neuroenteric canal. We report the case of a 64-year-old patient who presented with a sudden headache followed by collapse. Brain computed tomography revealed a hyperdense lesion in the anterior part of the third ventricle with obstructive hydrocephalus. A presumptive diagnosis of colloid cyst was made and he underwent a right transcortical approach for lesion resection. The histopathological examination revealed an EC. ECs are common lesions in the cervical-thoracic spine but rare in the supratentorial compartment with only two previously described cases occurring in the third ventricle.

Meningiomas occurring during long-term survival after treatment for childhood cancer.

Childhood cancer is rare but improvements in treatment over the past five decades have resulted in a cohort of more than 30,000 long-term survivors of childhood cancer in the UK with more added annually. These long-term survivors are at risk of late effects of cancer treatment which replace original tumour recurrence as the leading cause of premature death. Second neoplasms are a particular risk and in the central nervous system meningiomas occur increasingly with increased radiation dose to central nervous system tissue and length of time after exposure, resulting in a 500-fold increase above that expected in the normal population by 40 years of follow up. This multidisciplinary author group and others met to discuss the issue. Our pooled information, and consensus that screening should only follow symptoms, was published online by the Royal College of Radiologists in 2013. We outline here the current knowledge and management of these neoplasms secondary to childhood cancer treatment.

Applications of positron emission tomography in neuro-oncology: a clinical approach.

The field of neuro-oncology is concerned with some of the most challenging and difficult to treat conditions in medicine. Despite modern therapies patients diagnosed with primary brain tumours often have a poor prognosis. Imaging can play an important role in evaluating the disease status of such patients. In addition to the structural information derived from MRI and CT scans, positron emission tomography (PET) provides important quantitative metabolic assessment of brain tumours. This review describes the use of PET with radiolabelled glucose and amino acid analogues to aid in the diagnosis of tumours, differentiate between recurrent tumour and radiation necrosis and guide biopsy or treatment. [(18)F]Fluorodeoxyglucose (FDG) is the tracer that has been used most widely because it has a 2 h half life and can be transported to imaging centres remote from the cyclotron and radiochemistry facilities which synthesise the tracers. The high uptake of FDG in normal grey matter however limits its use in some low grade tumours which may not be visualised. [(11)C] methionine (MET) is an amino acid tracer with low accumulation in normal brain which can detect low grade gliomas, but its short 20 min half life has limited its use to imaging sites with their own cyclotron. The emergence of new fluorinated amino acid tracers like [(18)F]Fluoroethyl-l-tyrosine (FET) will likely increase the availability and utility of PET for patients with primary brain tumours. PET can, further, characterise brain tumours by investigating other metabolic processes such as DNA synthesis or thymidine kinase activity, phospholipid membrane biosynthesis, hypoxia, receptor binding and oxygen metabolism and blood flow, which will be important in the future assessment of targeted therapy.

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.