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Glomangiomyomatosis is an extremely rare variant of glomus tumours. We describe the first known case of paravertebral glomangiomyomatosis in the literature to cause spinal cord compression. A 45-year old female patient presented with sudden onset of left leg pain and progressive weakness in left-sided hip flexion. An MRI spine revealed a large, lobulated, heterogeneous mass cantered on the left L2/3 foramen, mimicking a dumbbell nerve sheath tumour. The mass was invading the psoas muscle and displayed evidence of recent haemorrhage. The patient underwent debulking of the lesion via a left retroperitoneal approach. Surgery was uneventful, with clinical improvement and resolution of leg pain post-operatively. Histopathology of the tumour revealed delineated glomus-like cells and foci of spindled shaped cells resembling myoid differentiation. Immuno-histochemical features of the tumour confirmed the diagnosis of glomangiomyomatosis. The patient continued under close follow up, representing 18 months later with clinical and radiological progression of the disease with similar symptoms of leg pain but no weakness. Follow up MRI revealed progression of the intraspinal and paraspinal components of the tumour with thecal compression. A posterior approach was utilized in order to decompress the intraspinal component, which again was uneventful, and improved the patient's symptoms. This is the first known case of paravertebral glomangiomyomatosis in the literature and this rare entity should be considered in the differential diagnosis of nerve sheath tumours due to risk of progression and recurrence.

In this study, we report three paediatric cases of Diffuse Glioneuronal Tumours with Oligodendroglioma-like features and Nuclear Clusters (DGONC).

Constitutional mismatch repair deficiency syndrome (CMMRD) is a rare cancer-predisposition syndrome associated with a high risk of developing a spectrum of malignancies in childhood and adolescence, including brain tumours. In this report, we present the case of an 8-year-old boy with acute headache, vomiting and an episode of unconsciousness in whom brain imaging revealed a high-grade glioma (HGG). The possibility of an underlying diagnosis of CMMRD was suspected radiologically on the basis of additional neuroimaging findings, specifically the presence of multiple supratentorial and infratentorial developmental venous anomalies (DVAs) and malformations of cortical development (MCD), namely, heterotopic grey matter. The tumour was debulked and confirmed to be a HGG on histopathology. The suspected diagnosis of CMMRD was confirmed on immunohistochemistry and genetic testing which revealed mutations in PMS2 and MSH6. The combination of a HGG, multiple DVAs and MCD in a paediatric or young adult patient should prompt the neuroradiologist to suggest an underlying diagnosis of CMMRD. A diagnosis of CMMRD has an important treatment and surveillance implications not only for the child but also the family in terms of genetic counselling.

Purpose: The 2016 WHO tumour classification highlights the role of IDH1/2 gene mutation and 1p/19q co-deletion in classifying grade II/III gliomas. A recent cIMPACT-NOW update proposes the use of t...

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.

Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare tumours. We describe a CAPNON in the posterior fossa and its associated neuropsychological sequelae to provide further evidence for the role of cerebellum in cognitive function. We report the clinical details, imaging, pre-operative neuropsychological assessment, histological features and management of a patient with such a tumour in the posterior fossa location. Detailed pre-operative neuropsychological assessment identified a number of cognitive deficits that had the hallmarks of dysexecutive syndrome. Post-surgery, there was considerable improvement, most notably on processing speed tasks and selected executive tests. This rare case provides further evidence for the role of cerebellum in cognitive function.

Sylvian fissure meningiomas (SFMs) represent a rare subgroup of nondural-based tumors arising from the meningothelial cells within the arachnoid of the Sylvian fissure. SFMs are more frequent in young males, usually manifest with seizures and display the same radiological features of meningiomas in other locations. Although the absence of dural attachment makes these tumors suitable for a complete resection, their anatomical relationships with the middle cerebral artery branches have impaired its achievement in half of them. To the best of our knowledge, only five atypical WHO grade II SFMs have been previously described. We provide a literature review of SFMs WHO grades I–II and discuss common characteristics and surgical challenges we found in a similar case.

Introduction: Unicameral bone cysts (UBC), also known as simple bone cysts, are common benign bone lesions filled with fluid, primarily occurring in children and adolescents. Although they can deve...

Primary involvement of the spinal cord by non-Hodgkin lymphoma is exceptionally rare. We report one such case in a 46-year-old gentleman which was successfully treated with surgery and adjuvant chemotherapy.

Background: MGMT methylation, along with 1p/19q co-deletion and IDH1 mutation, is an important biomarker in high grade gliomas. MGMT methylation indicates an improved response to temozolomide chemotherapy; patients with 1p/19q co-deleted anaplastic oligodendrogliomas benefit preferentially from adjuvant chemotherapy. Pyrosequencing is a method that allows the level of MGMT methylation to be measured in a quantitative manner. Aim: To compare the mean MGMT promoter methylation level of high grade gliomas and correlate it with other clinical parameters and markers including 1p/19q co-deletion and mutation to IDH1or IDH2. Methods: Pyrosequencing was used to quantitatively detect the level of MGMT promoter methylation for 171 high grade gliomas mutations to IDH1 and IDH2 genes were also detected by pyrosequencing, or immunohistochemistry (n=166). Screening for 1p/19q deletion was by fluorescence in situ hybridisation (n=46). Statistical analysis was performed using R-Stats v2.15.2. Results: Higher methylation was correlated with lower grade and mutation to either IDH1 or IDH2 (27.0% vs. 16.6% p = 0.008; and 27.5 vs. 16.1 p = 0.002 respectively). 1p/19q co-deletion versus non co-deletion was associated with a particularly high level of methylation (42.2% vs. 17.7% p = 0.001). No significant differences were seen for age or gender. Conclusions: The results offer a potential explanation for the improved prognosis seen in glioma patients with 1p/19q co-deletion.

PNR-02. DESMOPLASTIC NON-INFANTILE GANGLIOGLIOMA: A RARE CASE OF MALIGNANT TRANSFORMATION Natalie Smith, Bhaskar Thakur, Miren Aizpurua, Angela Calado Bravo, Safa Al-Sarraj, Sanjeev Bassi, Chris Chandler, and Bassel Zebian; King’s College Hospital, London, UK INTRODUCTION: Desmoplastic infantile gangliogliomas (DIG) are rare, supratentorial glioneuronal tumours of infants. They are histologically benign and consideredasWHO grade I tumours.When presenting in childrenover the age of 1, they are termed desmoplastic non-infantile ganglioglioma (DNIG). They are not usually associated with malignant behaviour. We present a rare case of recurrent DNIG with malignant transformation. CASE REPORT: A four year-old girl presented with a four-month history of worsening headaches, aggressive behaviour and left facial droop. MRI revealed a large right parieto-occipital lesion with mass effect. The lesion was subtotally resected. Histology was consistent with desmoplastic infantile ganglioglioma, WHO Grade I. Follow up MRIs demonstrated aggressive growth requiring further debulking in 7 months and 10 months. The subsequent biopsies showed similar features; however increasing mitotic activity in the predominant desmin positive astrocytic cells. The proliferation index (Ki67) was focally up to 4% in the first biopsy; increasing to 8% and 12% in the subsequent biopsies. Histology from the third resection was consistent with anaplastic ganglioglioma, WHO Grade III. CONCLUSION: This case of DNIG demonstrated aggressive growth despite multiple operative interventions, and histology strongly suggesting malignant transformation. Histology has shown malignant transformation, which has only been reported in a handful of cases in the literature. This has important implication for the follow up and management of these lesions. Neuro-Oncology 18:iii7–iii15, 2016. doi:10.1093/neuonc/now067.2 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

INTRODUCTION: Previously we have shown that glioblastomas with an oligodendroglial component (GBMO) did not differ from other glioblastomas in the frequency of 1p/19q deletion or isocitrate dehydrogenase mutation. Here we seek to determine whether anaplastic oligodendrogliomas with necrosis and very prominent endothelial cell hyperplasia (AO + N), often diagnosed as GBMO, differ in prognosis from the classic grade III counterpart (AOIII) and GBMO. METHOD: Anaplastic oligodendrogliomas with 1p/19q co-deletion and mutation to IDH1 or IDH2 were classed as either grade III or IV dependent upon the presence of the above mentioned high grade features. GBMO with neither 1p/19q co-deletion nor mutation to IDH1 or IDH2 were also selected for comparison. Statistical analyses were performed using R-Stats v2.15.2. RESULTS: AO + N (n = 16) with a median survival of 66.5 months had a worse prognosis than AOIII (n = 30) (median not reached, p = 0.04) and a better prognosis than GBMO (n = 46) (median survival 9 months, p = 1.04X10-5). The difference in survival between AO + N and GBMO remained significant when adjusted for age and gender (HR 0.17 95% CI 0.07-0.4, 6.3X10-5). CONCLUSION: The results suggest that anaplastic oligodendroglioma with necrosis and prominent endothelial cell hyperplasia behave worse than classic anaplastic oligodendroglioma and better than GBMO. Proper consideration should be given to including these neoplasms as a novel WHO grade IV classification if these results can be replicated in a larger cohort.

e21021 Background: Available protocols of response evaluation after therapy are based on the presence of viable cells, fibrosis and necrosis. Senescence is an irreversible cell cycle arrest that can be activated by different kinds of stress like DNA damage and cytotoxic drugs. Senescent cells are histologically very similar to viable neoplasic cells and usually evaluated alike. Our hypothesis is that neoadjuvant treatment can induce senescence as part of the therapeutic response and its presence may impact the prognosis. Methods: Tumor samples of 45 consecutive patients (pts) with RC treated between sept/02 and feb/2007 with fluorouracil-based chemotherapy and neoadjuvant radiotherapy, and of 33 non-treated colon cancer pts (controls), were selected. p53 and P16-ki67 double immunostaining were retrospectively evaluated in endoscopic-pretreatment biopsies, post-therapy specimens, and controls. P16+/ki67- malignant glands were considered as senescent-like glands. SPSS v20.0 (Kruskal-Wallis, Cox regression and log-rank test) was employed for analysis. Results: After a median follow up of 60 months 13 pts had relapsed. Significant differences in the percentage of senescent-like glands were observed between treated carcinomas and their pre-treatment samples (p=0.0001) and also with the colonic-non-treated carcinomas (p=0.0001). A tendency toward a better disease free survival (DFS) (p=0.236) in those patients with more than 30% of senescent glands after treatment was observed. No differences in p53 were found between the 3 groups. However, low levels of p53 expression in pretreatment-rectal biopsies correlate with a better pathologic response (GR) (p=0.029), and better DFS (p=0.0958). Conclusions: The number of senescent-like glands increases after neoadjuvant therapy. There was a tendency for a better DFS in the pts with treatment-induced senescence. Moreover, an inverse correlation between p53 expression in pretreated endoscopic-samples and pathologic response and DFS was observed. Further and larger studies should be performed to confirm the prognostic implications of evaluating senescence markers in treated rectal carcinomas.