Intracranial sclerosing extramedullary haematopoietic tumour mimicking meningioma in a patient with myelofibrosis: Case report.

Sclerosing extramedullary hematopoietic tumour is a rare tumour which can occur in certain myeloproliferative diseases, typically myelofibrosis. Typically these tumours present as a discrete retroperitoneal mass or masses, however they can occur in other sites within the body. In this case report we describe a 61-year old woman who underwent stealth guided bi-temporal craniotomy for resection of what was expected to be a large falx meningioma, however histopathology revealed the lesion to be a sclerosing extramedullary hematopoietic tumour. Following surgery, the patients post-operative recovery was complicated by intracerebral haemorrhage which required emergency evacuation and the patient remained in intensive care on a ventilator for 3 weeks. At one year follow up the patient reports her presenting symptoms of headaches, nausea, and vertigo had resolved.

Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort.

PURPOSE: Substantial evidence exists describing differences between paediatric and adult Chiari 1 malformation (CM1) patients. Differences in clinical presentation between very young (0-6 years old) and older (7-18 years old) paediatric patients is similarly well-established. However, progression on these findings with regard to surgical outcomes is limited. We aimed to establish whether inter-paediatric age group modifies surgical outcome for CM1 decompression. METHODS: Retrospective chart review was conducted for 65 patients receiving posterior fossa decompression between 2006 and 2018. Presenting features, surgical management, and outcome were evaluated and stratified into very young patients (0-6 years) or older patients (7-18 years). Outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS), a validated 16-point framework for comparison. RESULTS: Very young patients (21 patients) scored significantly lower in surgical outcome overall compared with older patients (44 patients) (12.1 ± 3.2/16 vs 14.2 ± 1.6/16, p = 0.011), and across 3/4 CCOS subscores: non-pain symptoms, functionality, and complications. Very young patients also returned to theatre more commonly (47.6% vs 13.6%, p = 0.003), primarily for re-do decompression (7/10 patients, 70%). Finally, the presentation of very young patients differed to older patients with significantly more oropharyngeal (38.1% vs 9.0%, p = 0.014) and motor symptoms (47.6% vs 22.7%, p = 0.042). DISCUSSION: Very young patients (0-6 years) do not appear to respond as well to standard posterior fossa decompression, as their older (7-18 years) paediatric counterparts, in the absence of several baseline cohort characteristic differences. We hypothesise underlying anatomical differences may contribute to this finding.

Endoscopic approach and stereotactic radiosurgery for a posterior third ventricular Central Neurocytoma - case report and literature review.

INTRODUCTION: Central Neurocytomas (CN) are a rare intracranial tumour, most often arising in the lateral ventricles and presenting with obstructive hydrocephalus. Isolated lesions in the third ventricle are uncommon. We present the fourth reported case of posterior third ventricular CN successfully managed surgically via endoscopy, allowing for concurrent biopsy and therapeutic endoscopic third ventriculostomy (ETV). Stereotactic radiosurgery was administered for the residual lesion. A brief review of CNs and previous similar cases is also provided. PRESENTATION OF CASE: A 58-year-old male presented with progressive decline in cognition and gait. Subsequent Magnetic Resonance Imaging revealed obstructive hydrocephalus secondary to a posterior third ventricular lesion. An endoscopic biopsy and concurrent cerebrospinal fluid diversion by ETV was performed. Pathological analysis was consistent with a CN with positivity to Synaptophysin. MIB-1 proliferation index was 1%. There was good clinical recovery, and the patient underwent adjuvant stereotactic radiosurgery 1.5 months post-surgery. DISCUSSION: Due to the rarity of CNs arising from the third ventricle, there are only three previous reports of these approached endoscopically. Such a technique allows for good visualisation of the lesion, and therapeutic ETV to relieve obstructive hydrocephalus. This case supports this approach as a valid, minimally invasive option. Additionally, this is the first case to report the MIB-1 proliferation index, contributing to future outcome evaluation of endoscopic approaches to typical (MIB-1 < = 2%) verses atypical (MIB-1 > 2%) CNs. CONCLUSION: Endoscopic biopsy with concurrent ETV and adjuvant stereotactic radiosurgery is a valid treatment option for deep seated isolated small posterior third ventricular CNs.

Publisher Correction: A reference collection of patient-derived cell line and xenograft models of proneural, classical and mesenchymal glioblastoma.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Diffuse midline glioma metastasis to the peritoneal cavity via ventriculo-peritoneal shunt: Case report and review of literature.

Extraneural dissemination of primary intracranial tumours to the peritoneal cavity via ventriculoperitoneal shunts is rare, with medulloblastoma and germ-cell tumours most common and gliomas seldom implicated. This report is the first described case of a diffuse midline glioma H3 K27M-mutant disseminating to the peritoneal cavity via a shunt. A four-year-old female presented with a large solid-cystic lesion centred on the suprasellar cistern, histologically revealed to be diffuse midline glioma H3 K27M-mutant. The patient received multiple courses of radiotherapy to the primary lesion and metachronous spinal metastases, and underwent bilateral ventriculoperitoneal shunts. She presented fourteen months following diagnosis with acute hydrocephalus and massive ascites revealed to be due to histologically confirmed intra-abdominal glioma metastasis secondary to shunting. Bilateral ventriculoatrial shunts along with targeted abdominal radiotherapy and repeated ascitic drainage were performed. The patient died one month later. A literature review demonstrated that intra-abdominal glioma metastasis is an extremely rare complication of cerebrospinal fluid diversion predominantly affecting paediatric patients with high-grade lesions within the first year after diagnosis and portends poor prognosis. Predisposition to metastasis is likely associated with tumour proximity to cerebrospinal fluid spaces and tumour biology. Contraindicating shunting in the presence of an intracranial tumour cannot be endorsed but rather shunt-related metastasis should be an acknowledged risk, and not-to-be-forgotten presentation.

A reference collection of patient-derived cell line and xenograft models of proneural, classical and mesenchymal glioblastoma.

Low-passage, serum-free cell lines cultured from patient tumour tissue are the gold-standard for preclinical studies and cellular investigations of glioblastoma (GBM) biology, yet entrenched, poorly-representative cell line models are still widely used, compromising the significance of much GBM research. We submit that greater adoption of these critical resources will be promoted by the provision of a suitably-sized, meaningfully-described reference collection along with appropriate tools for working with them. Consequently, we present a curated panel of 12 readily-usable, genetically-diverse, tumourigenic, patient-derived, low-passage, serum-free cell lines representing the spectrum of molecular subtypes of IDH-wildtype GBM along with their detailed phenotypic characterisation plus a bespoke set of lentiviral plasmids for bioluminescent/fluorescent labelling, gene expression and CRISPR/Cas9-mediated gene inactivation. The cell lines and all accompanying data are readily-accessible via a single website, Q-Cell (qimrberghofer.edu.au/q-cell/) and all plasmids are available from Addgene. These resources should prove valuable to investigators seeking readily-usable, well-characterised, clinically-relevant, gold-standard models of GBM.

Glioma surgical aspirate: a viable source of tumor tissue for experimental research.

Brain cancer research has been hampered by a paucity of viable clinical tissue of sufficient quality and quantity for experimental research. This has driven researchers to rely heavily on long term cultured cells which no longer represent the cancers from which they were derived. Resection of brain tumors, particularly at the interface between normal and tumorigenic tissue, can be carried out using an ultrasonic surgical aspirator (CUSA) that deposits liquid (blood and irrigation fluid) and resected tissue into a sterile bottle for disposal. To determine the utility of CUSA-derived glioma tissue for experimental research, we collected 48 CUSA specimen bottles from glioma patients and analyzed both the solid tissue fragments and dissociated tumor cells suspended in the liquid waste fraction. We investigated if these fractions would be useful for analyzing tumor heterogeneity, using IHC and multi-parameter flow cytometry; we also assessed culture generation and orthotopic xenograft potential. Both cell sources proved to be an abundant, highly viable source of live tumor cells for cytometric analysis, animal studies and in-vitro studies. Our findings demonstrate that CUSA tissue represents an abundant viable source to conduct experimental research and to carry out diagnostic analyses by flow cytometry or other molecular diagnostic procedures.

An unusual presentation of a solitary benign giant neurofibroma. Case report.

The authors report on a 34-year-old man who presented with acute enlargement of an extraspinal mass secondary to a hemorrhage following minor trauma. The mass had been present from birth, had slowly enlarged over time, and previous imaging had suggested an extraspinal fibrolipoma measuring 10 x 6 x 4 cm. Following minor trauma (scratching the skin overlying the tumor), the mass rapidly enlarged to approximately double in size over a period of 4 hours. A CT scan and MR imaging confirmed an extraspinal tumor that was 15 x 11 x 11 cm, with an associated acute hematoma of similar dimensions. The patient was taken to the operating room for hematoma evacuation and tumor resection. Histopathological investigation identified a benign, diffuse neurofibroma with associated dysplastic blood vessels exhibiting irregular areas of tunica media and sinusoidal-like vascular channels. To the authors' knowledge, a solitary giant neurofibroma in a patient without neurofibromatosis presenting with acute enlargement secondary to hemorrhage following minor trauma has not been previously described. The authors suggest that the source of the acute hemorrhage may be related to the neurofibroma-associated vascular dysplasia and the resultant increased vascular fragility.