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Attitudes to surgery for paediatric thalamic tumours have evolved due to improved preoperative imaging modalities and the advent of intraoperative MRI (iMRI) as well as enhanced understanding of tumour biology. We review the developments in our local practice over the last three decades with particular attention to the impact of iMRI. We identified all paediatric patients from a prospectively maintained neuro-oncology database who received surgery for a thalamic tumour (n = 30). All children were treated in a single UK tertiary paediatric neurosurgery centre between January 1991 and June 2020. Twenty patients underwent surgical resection, the remainder (10) undergoing biopsy only. Pre-operative surgical intent (biopsy versus debulking, near-total resection, or complete resection) as well as the use of iMRI were prospectively recorded. Complications recorded in clinical documentation between postoperative days 0 and 30 were retrospectively graded using a modified version of the Clavien Dindo scale. The extent of resection with respect to the pre-determined surgical aim was also recorded. Data on patient survival and disease progression status were obtained retrospectively. In our series, there were 42 procedures (25 craniotomies, 17 biopsies) performed on 30 patients (17 male, with a median age of 8 at surgery). Of the 25 surgical resections performed, complete resection was achieved in 9 (36%), near-total resection in 10 (40%), and limited debulking in 6 (24%). The predetermined surgical aim was achieved or exceeded in 91.3% of cases. The proportion of craniotomies for which substantial resection was achieved, increased from 37.5 to 94.2% with use of iMRI (p = 0.014). Surgical morbidity was not associated with greater extent of surgical resection. High-grade histology is identified as the only independent significant factor influencing overall survival as calculated by Cox proportional hazards model (p = 0.006). We note a significant change in the rate and extent of attempted resection of paediatric thalamic tumours that has developed over the last 3 decades. Use of iMRI is associated with a significant increase in substantial tumour resection surgeries. This is not associated with any significant level of surgical morbidity. Improvements in pre- and intra-operative imaging alongside better understanding of tumour biology facilitate patient selection and a surgically more aggressive approach in selected cases whilst maintaining safety and avoiding operative morbidity.

Paediatric cerebellar pilocytic astrocytomas (PA) (WHO grade 1) are amongst the most common of childhood brain tumours and are generally amenable to resection, with surgery alone being curative in the majority of cases. There is, however, a lack of consensus regarding the frequency and duration of post-treatment MRI surveillance for these tumours. This is important, as follow-up imaging is a significant use of resources and often associated with patient and family anxiety. We have assessed the utility of MRI surveillance in the detection of cerebellar PA recurrence at our regional paediatric neurosurgical centre. The tumour register at Alder Hey Children’s Hospital was searched to identify all patients diagnosed between 2007 and 2017, with a confirmed histopathological diagnosis of cerebellar PA. Patient demographics, surgical outcome, number of MRI scans and length of follow-up were recorded for each patient. Forty patients met the inclusion criteria. The mean age at diagnosis was 7.8 years (range 2 to 17 years). Complete surgical resection (CR), confirmed by post-operative MRI, was achieved in 36 of the 40 patients, including all 31 cases from 2009 and later for which intraoperative MRI (iMRI) was utilised. There was one case of recurrence after CR (at 2.2 years) out of the 36 cases, whereas all 4 patients with initial partial resections had progressive growth of their tumours and required second surgical interventions. This series confirms the very low likelihood of recurrence for completely resected cerebellar PAs and suggests that in such cases the duration and frequency of surveillance imaging could be limited to a maximum of 2.5 to 3 years of follow-up imaging. This report also indicates improved complete resection rates over time, probably associated with technical advances including the routine in-house use of iMRI in 2009.

This study aimed to investigate the inter-rater reliability and construct validity of the Scale for the Assessment and Rating of Ataxia (SARA) and Brief Ataxia Rating Scale (BARS) in children with posterior fossa tumours. These scales have been developed for adults with genetic ataxias, and the performance of these scales in children with brain tumours has not previously been described.The participants, who had undergone surgical resection for a posterior fossa tumour (inclusion criteria age 4-18 years), were recruited from the neuro-oncology service at a tertiary children's hospital. Children were assessed using the SARA, BARS and Paediatric Evaluation of Disability Index (PEDI) mobility domain, a measure of function. Children were independently rated by two therapists to determine the inter-rater reliability of the SARA and BARS. The construct validity was determined by assessing the correlation between the two scales with the PEDI.Forty-four children were recruited. Inter-rater reliability was good for both scales, demonstrating the strong correlations (SARA, r = 0.94; BARS, r = 0.91) and the good consistency (93 % of SARA and 90 % of BARS paired scores differing by less than 2 points) between two raters. Both ataxia scales demonstrated a strong negative correlation with the mobility domain of the PEDI (SARA, r = -0.77; BARS, r = -0.76), indicating that more severe ataxia was associated with worse mobility. The mean time for completion of the SARA was 4.5 and 2.7 min for the BARS.The SARA and BARS are reliable and valid measures and appear to be of equal value in determining the severity of ataxia in children with posterior fossa tumours.

The purpose of this study is to compare the surgical and imaging outcome in children who underwent brain tumour surgery with intention of complete tumour resection, prior to and following the start of intra-operative MRI (ioMRI) service. ioMRI service for brain tumour resection commenced in October 2009. A cohort of patients operated between June 2007 and September 2009 with a pre-surgical intention of complete tumour resection were selected (Group A). A similar number of consecutive cases were selected from a prospective database of patients undergoing ioMRI (Group B). The demographics, imaging, pathology and surgical outcome of both groups were compared. Thirty-six of 47 cases from Group A met the inclusion criterion and 36 cases were selected from Group B; 7 of the 36 cases in Group A had unequivocal evidence of residual tumour on the post-operative scan; 5 (14 %) of them underwent repeat resection within 6 months post-surgery. In Group B, ioMRI revealed unequivocal evidence of residual tumour in 11 of the 36 cases following initial resection. In 10 of these 11 cases, repeat resections were performed during the same surgical episode and none of these 11 cases required repeat surgery in the following 6 months. Early repeat resection rate was significantly different between both groups (p = 0.003). Following the advent of ioMRI at our institution, the need for repeat resection within 6 months has been prevented in cases where ioMRI revealed unequivocal evidence of residual tumour.

Pineal region tumours are rare. Surgery is associated with high morbidity, and consensus on ideal management pathways remains variable. Patients with pineal region tumours were identified from the neuro-oncology database, and their data were retrospectively reviewed. Data collection included presentation, germ cell markers, tumour size and location, imaging, histology, treatment and control/relapse rates. Twenty-four patients were treated at Alder Hey Children’s Hospital between 1998 and 2010. Median age at diagnosis was 12.7 years. Sixteen patients presented with hydrocephalus. Twelve were successfully treated with endoscopic third ventriculostomy (EVT), and four required shunt insertion during follow-up. Fifteen patients had endoscopic biopsies. Eleven biopsies were performed at the same time as third ventriculostomy, and four patients without significant hydrocephalus had endoscopic image-guided biopsies. Eight patients had stereotactic biopsies (either because of slit ventricles at diagnosis or because of a ventriculoperitoneal shunt already in place), and only one patient had an open biopsy. No morbidity or mortality was associated with the biopsies. Histology showed four pineal parenchymal tumours, nine gliomas and eleven germ cell tumours. Thirteen patients underwent surgical resection. Significant tumour debulking was achieved in all of these patients, with gross total resection in 70 % of cases. There was one intraventricular haemorrhage (full recovery) and one mild hemiparesis. There was no surgical mortality. The mainstay of hydrocephalus management is EVT which provides a safe and reliable route for biopsy. In our experience, craniotomy and tumour resection are effective and safe.

The management of recurrent ependymoma within the paediatric population remains a therapeutic challenge. The options available are varied and patients may have already received prior radio- or chemotherapy. As yet, no consensus exists regarding their optimal treatment. We review the literature and present our contemporary management strategies for this interesting group of patients. Survival following recurrence is poor and those prognostic factors that predispose to recurrence include extent of surgical resection and the timing of administration of adjuvant therapy. The extent of resection at re-operation can confer a survival advantage, without a necessary increase in morbidity. Strategies aimed at improving surgical resection at first diagnosis include improving and centralising post-surgical radiological review, defining what are true residuals, and centralising surgical review of incompletely resected tumours. Re-irradiation can improve survival, and with the use of conformal radiation fields need not necessarily lead to neuropsychological damage. Cisplatin and etoposide remain the most effective chemotherapeutic agents to date and with an increase in the understanding of tumour biology this may improve further. Because of the complex nature of this group of patients, decisions regarding their management require the involvement of a paediatric neurosurgeon, paediatric neuro-oncologist and paediatric radiation oncologist.

A rare case of a large jugular foramen tumour presenting as a posterior fossa mass is described.The patient, a 13-year-old boy presented with symptoms of raised intracranial pressure and on MRI imaging demonstrated the tumour origin within the jugular foramen, with appearances suggestive of schwannoma. He underwent a complete excision as a single-stage procedure thorough a lateral suboccipital craniotomy with minimal morbidity. Histology confirmed the diagnosis of schwannoma (WHO I). Jugular foramen schwannoma is virtually unheard of in the paediatric population.The differential diagnosis of a jugular foramen tumour includes tumours of the glomus jugulare, meningiomas, schwannomas, myxomas and chondrosarcomas. The imaging characteristics are important when considering this differential diagnosis.

Herpes simplex virus (HSV) encephalitis is a rare association with pediatric neurosurgical pathologies.A 13-year-old boy was diagnosed with an inoperable, biopsy-proven pontine grade II astrocytoma. During radiotherapy, he developed status epilepticus controlled by thiopentone with intubation and ventilation. Empiric cefotaxime and aciclovir were given. Lumbar cerebrospinal fluid (CSF) showed a normal white cell count, normal glucose, and a slightly elevated protein level. However, the CSF showed a positive polymerase chain reaction (PCR) for HSV type 1 DNA. Intravenous aciclovir was given for 21 days and foscarnet for 7 days. He was extubated after 4 weeks at which time he was aphasic with spastic diplegia. After 8 weeks, MRI brain scan showed the typical bitemporal pattern of HSV encephalitis. He made slow improvement but died 8 months after diagnosis from tumor progression.HSV encephalitis is a rare but life threatening complication in neurosurgical patients. A low threshold for both investigation with CSF PCR and empirical treatment with intravenous aciclovir is warranted. As in this case, initial microscopic examination of the CSF may be normal. The literature on HSV encephalitis in neurosurgical patients is discussed.

We present a case of a chordoid meningioma, a tumour sub-type which comprises less than 0.5% of all meningiomas. The patient, a 12-year-old Somalian girl with a history of having had a craniotomy for a brain tumour in her native country 11 months previously, presented with seizures. Imaging revealed an isolated left frontal tumour, which at the time was felt to be a residual mass. She underwent a macroscopically complete resection of this tumour. Histology revealed it to be a grade 2 chordoid meningioma. Recurrence with additional local meningeal lesions was detected on follow-up magnetic resonance imaging (MRI) at 2 months post surgery. These were resected and she was treated with adjuvant cranial radiation treatment that resulted in non-progression of the cranial tumour on imaging 6 and 12 months post surgery. However, at 12 months post initial presentation, she re-presented with disseminated spinal disease refractory to salvage radiation treatment and succumbed to her illness. This is the first reported case of cranial radiation treatment being used to treat sub-totally resected recurrent chordoid meningioma. Whilst intra-cranial control of the tumour was achieved with this management, it did not prevent spinal progression. The authors advise a high-surveillance management strategy when treating these lesions and to employ cranial and spinal radiation treatment at the first sign of disease progression.

Meningeal melanocytoma was first described over 30 years ago as a benign tumour derived from melanocytes. Since then, data suggest that its mode of presentation is variable without a clear predilection for any particular site in the neuroaxis. Although classified as a benign tumour, this tumour shows a marked tendency towards reduced survival following subtotal resection and transformation over time in a limited number to malignant melanoma. Incomplete resection of these tumours without postoperative radiotherapy has only a 42% 5-year survival rate. Its classification as a benign tumour should be revised, given the published 5-year survival data.We report a fatal case of meningeal melanocytoma in the cerebello-pontine angle in a 10-year-old child. This case exemplifies the vascular nature of these lesions even with minimal vascular blush on angiography. An updated literature search is presented, the results of which highlight the need for close follow-up and adjuvant treatment following subtotal resection.