Recursive partitioning analysis for disease progression in adult intracranial ependymoma patients

Intracranial ependymomas are rare tumors in adults. Although recent advancements from demographic, clinical, and biological studies provide new perspectives on this rare tumor, they are not yet widely applied in clinical practice. Currently, most ependymoma patients are treated in the same way: via surgical resection with adjuvant radiation therapy. However, it is reasonable to apply more aggressive treatment for high-risk patients. From this point of view, we performed a study to investigate risk grouping for disease progression of intracranial ependymomas in adults. A total of 53 patients were included in this study. Data were extracted for patient and tumor characteristics, extent of resection, progression-free survival (PFS), and overall survival. Prognostic variables from univariate and multivariate survival analyses were included in a recursive partitioning analysis for the hierarchical risk grouping of the estimated PFS. Three risk groups were defined based on the clinical prognostic factors. Survival analysis showed significant differences in mean PFS between the different groups: 160.5 ± 22.1 months in the complete resection group, 100.4 ± 36.8 months in the incomplete-resection and intraventricular-location group, and 23.5 ± 6.9 months in the incomplete-resection and extraventricular-location group (p < 0.001). The risk of disease progression in adult intracranial ependymoma patients could be stratified by degree of resection and tumor location. In clinical practice, this result could provide useful information, such as when “second-look” surgery should be performed or whether small tumors invading the fourth ventricle floor should be resected at the expense of neurological deficit.     

Clinical features and management of five patients with supratentorial subependymoma

Subependymoma is a rare low-grade glioma of the central nervous system that is often asymptomatic and discovered incidentally. If symptomatic, however, its clinical features vary widely. We report and discuss the management of five surgical patients (two males and three females, 27–65 years old). All tumors developed supratentorially around the foramen of Monro, and their diameters ranged from 18 mm to 90 mm. Of the three symptomatic patients, one presented with sudden loss of consciousness despite having only a small tumor, while in contrast, another patient showed only gradual memory disturbance despite having a very large tumor (90 mm diameter). Two patients were asymptomatic but had ventricular dilatation. Gross total resection was achieved via either a transcortical (three patients) or an anterior transcallosal approach (two patients). No recurrence was observed during follow-up. We concluded that as surgery is the only curative treatment for subependymoma, and even a small tumor can present with sudden deterioration, we recommend early and total resection.     

Prognosis by tumor location in adults with intracranial ependymomas

Intracranial ependymomas are rare tumors in adults. Thus, factors affecting prognosis are poorly understood. We performed a study to investigate whether tumor location is an important prognostic factor in adults who undergo surgery for intracranial ependymomas. PubMed was searched to identify studies that reported clinical outcomes in adult patients with intracranial ependymoma. Data were extracted for patient and tumor characteristics, extent of resection, progression-free survival (PFS), and overall survival (OS). Tumors were categorized as supratentorial or infratentorial and extraventricular or intraventricular. Presenting clinical features and tumor characteristics were tabulated. Kaplan–Meier and multivariate Cox regression survival analyses were performed to determine PFS and OS by tumor location. Extent of resection was also analyzed by tumor location. A total of 183 patients were included in the meta-analysis. Patients presented at a mean of 8.2 months with a myriad of clinical features. The mean tumor size was 3.38 cm, and 19.3% of tumors were cystic. Supratentorial tumors were most commonly located in the frontal and parietal lobes, and infratentorial tumors in the fourth ventricle. Supratentorial tumors demonstrated significantly poorer PFS (p < 0.001) and OS (p = 0.003) than infratentorial tumors, despite a higher rate of gross total resection (GTR) for the supratentorial tumors (72.6% versus 42.1%). Extraventricular ependymomas displayed significantly poorer PFS than intraventricular ependymomas (p = 0.009). In summary, supratentorial ependymomas have significantly poorer PFS and OS than their infratentorial counterparts, despite being more conducive to GTR, suggesting increased clinical aggressiveness. Extraventricular location is also associated with significantly poorer PFS than intraventricular location.     

Clinical and imaging features of central neurocytomas

To describe the clinical and imaging characteristics of patients with central neurocytoma (CN), we reviewed data on 27 patients who had histologically confirmed CN and were treated in our institution between 1999 and 2010. Neuro-imaging findings on CT scan (n = 18) and MRI (n = 25) were retrospectively evaluated. There were 15 males and 12 females with a mean age of 29 years (range, 11–46 years). The most frequent presentations included headache (n = 21) and vomiting (n = 6). Tumor sites included bilateral lateral ventricles (n = 10), right lateral ventricle (n = 7), left lateral ventricle (n = 7) and fourth ventricle (n = 3). On MRI, the T1-weighted signal was hypointense in 12 patients and isointense in 13, and the T2-weighted signal was isointense in 8 patients and hyperintense in 15. CT scans/MRI revealed a cystic component in 18 patients. Tumors showed a mild to marked enhancement in 26 patients. Flow voids from tumor vessels on MRI were present in 14 patients, and calcification was noted in six of 18 patients with CT scans. All lateral ventricle tumors were resected through a transcortical or transcallosal approach. Gross total resection was achieved in 19 patients, near total in two and subtotal in six. One patient died of cerebral infarction in the perioperative period. At the last follow up, there were three known clinical recurrences in this series. However, no recurrence was noted in 17 patients who underwent gross total resection with no adjuvant therapy.     

Brain tumors and the area postrema

Brain tumors can rarely present with symptoms mistaken for anorexia nervosa. We report a patient with a long-standing history of anorexia who developed headaches and was found on brain MRI to have a brain tumor in the area of the fourth ventricle. On admission, the patient presented with a 4 month history of headaches and a 10 year history of “anorexia nervosa”. Interestingly, the patient did not endorse the classic sense of an altered self-body image. Her body weight on admission was 37 kg. The patient underwent surgical resection of the tumor. On postoperative day (POD) 1, the patient subjectively reported an increased appetite. On POD 2, we documented that she finished her entire food tray for the first time during her hospital stay. Her peri-operative course was without any complications. She presented for a follow-up clinic visit 2 weeks postoperatively and was noted to have a new body weight of 47 kg (10 kg gain). To our knowledge, this is the first reported occurrence of a sporadic, and third overall occurrence, of a hemangioblastoma that presented with an anorexia nervosa–like syndrome that was ultimately cured with surgical resection. In patients presenting with a history of psychiatric illness, it is important to consider the possibility of underlying, organic pathologies in the central nervous system affecting the relevant neuro-anatomical domains.     

Hurthle cell carcinoma presenting as a single choroid plexus metastasis

Choroid plexus masses represent approximately 0.3–0.8% of intracranial tumors. Herein we present, to our knowledge, the first reported patient with an isolated Hurthle cell papillary thyroid carcinoma metastasis to the choroid plexus of the lateral ventricle. Unresponsive to iodine ablation and refusing surgery, the patient underwent Gamma Knife radiosurgery (Elekta AB, Stockholm, Sweden), receiving 15 Gy to the 50% isodose line. The lesion regressed until 5 years later at which time it was unresponsive to 18 Gy and required surgical resection. Although extraneural metastatic cancers are recognized as potential sources for the single choroid plexus mass, we must consider even the unusual culprit in patients with a history of cancer.     

Medullary hemangioblastoma: 34 patients at a single institution

This study aimed to elucidate the surgical experience of medullary hemangioblastoma (MH) at a single institution. We reviewed 34 consecutive patients with MH operated on between January 2005 and June 2012 in the neurosurgery department of the Beijing Tiantan Hospital. There were 14 men and 20 women. The patients were aged from 17 to 60 years with an average age of 38 years. Tumors were cystic in 12 patients (Type A), and solid in 22 patients. The solid tumors were of a small size in six patients (<3 cm, Type B), large in 12 (3.1–5 cm, Type C), and giant in four (>5 cm, Type D). Radical tumor removal was achieved in all patients. Tracheotomy was performed in 10 patients (one Type B patient, seven Type C, two Type D) postoperatively. Pneumonia secondary to lower cranial nerve palsy occurred in six patients (all Type C). Complications including intracranial infection (n = 5), gastrointestinal bleeding (n = 2), and intracranial hematoma (n = 1) also occurred in this group. Follow-up (range, 2–82 months; mean, 30 months) was available in all patients. At follow-up, 29 patients (85.3%) had a good outcome. Twenty-eight of these (82.4%) had an excellent outcome postoperatively (Karnofsky Performance Status ⩾80). Although transient surgical complications are possible especially for large solid tumors, total surgical resection can be performed with favorable long-term outcomes with meticulous microsurgical technique and understanding of the vascular pattern of the tumor. Postoperative management of MH is as important as the operation.     

Jugular foramen schwannomas: A single institution patient series

Jugular foramen schwannomas are rare. To our knowledge only a small number of series including a large number of patients have been reported. We aimed to analyse the clinical characteristics, surgical approaches, and outcome of patients undergoing treatment for jugular foramen schwannomas via a retrospective analysis of departmental records. Data for 28 patients treated for jugular foramen schwannomas in the Department of Neurosurgery at our tertiary level referral institution between January 2001 and December 2010 were analysed. Most patients were in the fourth decade of life, with the duration of symptoms ranging from 1 month to 13 years. A skull base approach was used in every surgically treated patient. Of the 19 patients for whom radiological follow-up were available, complete tumor excision was achieved in 17 patients. Follow-up ranged from 3 months to 59 months (mean, 32 months). One patient died and three had permanent morbidity in the form of facial nerve palsy. Jugular foramen schwannomas are best treated by total surgical resection. Partial resection is appropriate for tumors with adhesions to the brainstem and in medically unfit patients. Subsequent radiosurgery can be used for small residual tumors.     

Pleomorphic xanthoastrocytoma arising from the suprasellar region: A report of two cases

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic neoplasm that usually arises in children and young adults. Typically, lesions of PXA are superficially located in the cerebral hemispheres. Herein, we report two extremely rare patients with PXA arising from suprasellar regions. One of the patients is a 29-year-old man admitted to our hospital with a history of progressive headache for 1 month. The patient’s brain MRI revealed a large tumor arising from the suprasellar cistern of the third ventricle. The second patient, a 52-year-old woman, presented with progressive dizziness and visual disturbance that had developed over the course of 1 year. The MRI revealed a well-enhanced suprasellar solid mass measuring 1.4 × 1.2 × 1.4 cm. Both patients underwent surgical removal of their tumors, and both patients showed similar microscopic structures and immunohistochemical phenotypes: the tumor cells were pleomorphic with mixtures of spindle-shaped, and multinuclear giant cells. In addition, eosinophilic granular bodies and xanthomatous cells were seen on section. Immunohistochemistry was positive for GFAP, S-100, and CD34, and was negative for IDH 1, CK, and Syn. The Ki-67 proliferation index was less than 1%. Silver impregnation revealed reticulin fibers surrounding the individual tumor cells, and small cell groups. Based on these findings, the two patients were diagnosed with PXA in the suprasellar region. To date, only five such patients have been reported in the literature. PXA should be included in the differential diagnosis for tumors arising in the sellar region.     

Factors associated with survival for patients with glioblastoma with poor pre-operative functional status

Patients with glioblastoma (GB) are known to have poor prognoses, and among these patients, those with poor neurological function have an even poorer prognosis. Consequently, aggressive surgeries and adjuvant therapies are often withheld because of this dismal outlook. The effects of aggressive therapies in this small subset of patients remain unknown. The goal of this study was to evaluate outcomes and factors associated with survival for poor functioning patients who underwent aggressive resection of their GB. Adult patients who underwent surgical resection of an intracranial primary GB at an academic tertiary-care institution between 1997 and 2007 were retrospectively reviewed. Patients with a Karnofsky Performance Scale (KPS) score of ?60 were included. A total of 100 patients with primary GB met the inclusion criteria. The average age (±standard deviation) and KPS score of this cohort were 54 ± 15 years and 53 ± 12, respectively. No patient (0%) experienced perioperative mortality, and 0 (0%), 10 (10%), and 3 (3%) of patients incurred a new or increasing language, motor, and visual deficit, respectively. At last follow-up, 88 (88%) patients died with a median survival of 6.6 months. The factors associated with improved survival were age <65 year (p = 0.005), tumor size >2 cm (p = 0.01), radical tumor resection (p = 0.01), and temozolomide (p = 0.001). This study identifies a subset of patients with poor functional status who may benefit from aggressive surgical resection.     

The impact of stereotactic laser ablation at a typical epilepsy center

PurposeStereotactic laser ablation (SLA) is a novel form of epilepsy surgery for patients with drug-resistant focal epilepsy. We evaluated one hundred consecutive surgeries performed for patients with epilepsy to address the impact of SLA on our therapeutic approach, as well as patient outcomes.MethodsA retrospective, single center analysis of the last one hundred neurosurgeries for epilepsy was performed from 2013 to 2015. Demographics, surgical procedures, and postoperative measures were assessed up to 5 years to compare the effect of SLA on outcome. Confidence intervals (CI) and comparative tests of proportions compared outcomes for SLA and resective surgery. Procedural categorical comparison used Chi-square and Kaplan–Meier curves. Student t-test was utilized for single variables such as age at procedure and seizure onset.ResultsOne hundred surgeries for epilepsy yielded thirty-three SLAs and twenty-one resections with a mean of 21.7-month and 21.3-month follow-up, respectively. The temporal lobe was the most common target for SLA (92.6%) and resection (75%). A discrete lesion was present on brain magnetic resonance imaging (MRI) in 27/32 (84.4%) of SLA patients compared with 7/20 (35%) of resection patients with a normal MRI. Overall, 55–60% of patients became seizure-free (SF). Four of five patients with initial failure to SLA became SF with subsequent resection surgery. Complications were more frequent with resection although SF outcomes did not differ (Chi square; p = 0.79). Stereotactic laser ablation patients were older than those with resections (47.0 years vs. 35.4 years, p = 0.001). The mean length of hospitalization prior to discharge was shorter for SLA (1.18 days) compared with open resection (3.43 days; SD: 3.16 days) (p = 0.0002).ConclusionWe now use SLA as a first line therapy at our center in patients with lesional temporal lobe epilepsy (TLE) before resection. Seizure-free outcome with SLA and resection was similar but with a shorter length of stay. Long-term follow-up is recommended to determine sustained SF status from SLA.     

The glomic artery supply of carotid body tumors and implications for embolization

Carotid body tumors (CBT) are rare neuroendocrine neoplasms that usually present in the third or fourth decades of life and are benign in more than 95% of cases. In the angiographic literature, the arterial supply to carotid body tumors is well documented but is often incomplete, with infrequent mention of the glomic artery, a common arterial feeder described in the anatomic and pathologic literature. Through a review of our neuroendovascular patient database, we identified eight patients with CBT undergoing transarterial embolization followed by resection. Mean patient age was 51.5 years (range 29–82), and all patients were female. Mean tumor size was 91.2 cc (standard deviation [SD] 61.1, median 67.7 cc). After embolization, greater than 90% flow reduction was achieved in 5/8 patients (63%); 60–80% flow reduction was achieved in the remaining patients. Mean operative blood loss was 166 cc (SD 100, median 122 cc) and mean operative time was 252 minutes (SD 134.5, median 155 minutes). Pre-embolization angiography was reviewed to identify a glomic artery, defined as a dominant artery supplying the CBT arising from the region of the carotid bifurcation. In six of eight patients (75%) a glomic artery could be identified, arising from the common carotid artery in 4/6 patients and the external carotid artery in 2/6 patients. Thus, glomic artery to supply to CBT was identified in the majority of patients in this series. Knowledge of its presence and identification as a direct supplier, frequently from the common carotid artery itself, provides an avenue for more thorough preoperative embolization of CBT.     

Arterial fenestrations and their association with cerebral aneurysms

Fenestrations of intracranial arteries and associated aneurysms are rare. The significance of these fenestrations in relation to aneurysms remains unclear. We present four patients with fenestration-associated aneurysms and a comprehensive review of associations with aneurysms and other vascular lesions. A PubMed search of the literature was conducted from 1970–2012 reporting cases of intracranial aneurysms associated with arterial fenestration or duplications. Data were collected on patient presentation, sex, age, aneurysm and fenestration location, aneurysm treatment, and presence of other vascular lesions. We performed a retrospective review of four patients with intracranial fenestrations associated with aneurysms at our institution from 2012–2013. There were 59 cases of fenestrations and associated aneurysms in the literature. Aneurysms were reported as either arising from (n = 50) or adjacent to but distinct from (n = 13) fenestrations. The most common single fenestration location was at the basilar artery (n = 23, 36.5%); however the majority of fenestrations were in the carotid circulation (n = 34, 54.0%). The majority of patients with aneurysms and fenestrations at all locations except those at the anterior communicating artery (70.5%) presented with subarachnoid hemorrhage. Patients with aneurysms arising from a fenestration or adjacent to a fenestration presented with an additional intracranial vascular lesion in 38% and 31% of cases, respectively. The majority of all aneurysms were treated with microsurgical clipping. Aneurysms associated with cerebral arterial fenestrations are most commonly discovered after subarachnoid hemorrhage and are most often located in the carotid circulation. A high index of suspicion must be maintained for an associated vascular lesion if an intracranial fenestration is discovered.     

Paediatric germ cell tumours of the central nervous system: Results and experience from a tertiary-referral paediatric institution in Australia

A retrospective analysis was conducted on consecutive patients with intracranial germ cell tumours diagnosed and treated from 1 January 1997 to 31 December 2007 to assess and determine demographic factors and treatment outcomes of children with these tumours treated in a major paediatric referral hospital in Australia. In this study, intracranial germ cell tumours represented 4.8% of paediatric brain tumours seen. Of the 21 patients identified, 15 (71.4%) were diagnosed with pure germinoma and six (28.6%) with non-germinomatous germ cell tumours (NGGCT) or mixed tumours. One patient received chemotherapy alone, two patients were treated with radiation alone and the remaining 18 received a combination of chemotherapy and radiotherapy. A total of 33 neurosurgical operations were performed with 15 biopsies via open, endoscopic or transphenoidal means; nine open resections; and nine procedures for hydrocephalus comprising seven third ventriculostomies and two ventriculoperitoneal shunts. For patients with pure germinomas, the 5-year disease-free rate (DFS) was 93.3%, and overall survival (OS) rate was 100% compared to NGGCT or mixed tumours (DFS 50%; OS 50%) (DFS p = 0.019, OS p = 0.004). The data presented show that pure germinomas carry a favourable prognosis. The data also support that treatment with induction chemotherapy followed by dose-attenuated radiotherapy is an effective alternative with results comparable to historical controls treated with craniospinal irradiation. Although chemoradiotherapy has become the mainstay of treatment in intracranial germ cell tumours, surgery remains integral to the management of this condition. Surgery remains important in establishing the histological diagnosis, as well as in the treatment of hydrocephalus. Furthermore, debulking procedures may be advocated in NGGCT as they are often resistant to chemotherapy.     

Outcome of children with acetylcholine receptor (AChR) antibody positive juvenile myasthenia gravis following thymectomy

Most evidence supporting the benefit of thymectomy in juvenile myasthenia gravis (JMG) is extrapolated from adult studies, with only little data concerning paediatric populations. Here we evaluate the outcome of children with generalized JMG who underwent thymectomy between 1996 and 2010 at 2 tertiary paediatric neurology referral centres in the United Kingdom. Twenty patients (15 female, 5 male), aged 13 months to 15.5 years (median 10.4 years) at disease onset, were identified. Prior to thymectomy, disease severity was graded as IIb in 3, III in 11, and IV in 6 patients according to the Osserman classification. All demonstrated positive anti-acetylcholine receptor (AChR) antibody titres. All patients received pyridostigmine and 14 received additional steroid therapy. Transternal thymectomy was performed at the age of 2.7–16.6 years (median 11.1 years). At the last follow-up (10 months to 10.9 years, median 2.7 years, after thymectomy), the majority of children demonstrated substantial improvement, although some had required additional immune-modulatory therapies. About one third achieved complete remission. The postoperative morbidity was low. No benefit was observed in one patient with thymoma. We conclude that thymectomy should be considered as a treatment option early in the course of generalised AChR antibody-positive JMG.     

Safety and feasibility of combined coiling and neuroendoscopy for better outcomes in the treatment of severe subarachnoid hemorrhage accompanied by massive intraventricular hemorrhage

Subarachnoid hemorrhage (SAH) with intraventricular hemorrhage (IVH) is associated with poor outcomes. The aim of this study was to evaluate the safety and feasibility of combined coiling and neuroendoscopy for treating severe SAH with massive IVH. Between April 2008 and June 2011, 49 patients with a severe SAH were treated at the Department of Neurosurgery, Fukuoka University, Japan; 10 of these patients had a massive IVH with a ruptured aneurysm. All 10 patients (three men and seven women; mean age, 63.1 ± 8.5 years) were treated with coiling and neuroendoscopic removal of the IVH within 2 days of onset. Coiling was successfully performed at a mean volume embolization ratio of 21.8 ± 5.5%. Neuroendoscopic removal of the IVH reduced the mean Graeb score from 10.5 ± 2.0 to 4.8 ± 2.5 (p = 0.005). All external drains were removed on day 3. No rebleeding or acute hydrocephalus was noted. The Glasgow Outcome Scale scores at discharge indicated two patients with good recovery, three with moderate disability, four in a vegetative state, and one dead. A good modified Rankin Scale (mRS) score (0–2) at least 6 months later (mean follow-up period, 15.4 ± 9.2 months) was observed for five patients (50%), and a poor mRS score (3–6) was observed for the remaining four patients. Neuroendoscopically removing the IVH from all of the ventricles between the lateral and the fourth ventricle and coiling the ruptured aneurysm is a safe, feasible approach for treating severe SAH with massive IVH.     

Surgical treatment of hypermotor seizures originating from the temporal lobe

PurposeTo describe the characteristics of electroclinical manifestations in patients with hypermotor seizures (HMSs) originating from the temporal lobe.MethodsWe retrospectively reviewed the data of patients who underwent surgical treatments for seizure to identify patients with HMSs of temporal origin. We systematically reviewed patient seizure histories, imaging reports, video-EEG monitoring data, operative records and pathological findings.ResultsEight of the 9 patients reported auras. The ictal behavior included marked agitation in 5 patients and mild agitation in 4 patients. All of the 9 patients exhibited stiffness or dystonia of the upper limb or contralateral limbs during ictus. Seven of the 9 patients completed intracranial recording and at least 3 seizures were recorded for each patient. The intracranial recordings showed ictal activity originating from mesial temporal lobe in 6 patients and the lateral temporal lobe in 1 patient. The time interval of ictal propagation from the temporal to frontal lobe was 15.0 ± 8.3 s. While the time interval from EEG origination to the beginning of hypermotor behavior was 21.0 ± 8.1 s. Brain MRIs revealed hippocampal sclerosis in 3, neoplastic lesion in 1, and normal images in the remaining 5 patients. Patients were followed for 1–5 years after the anterior temporal lobectomy; 7 patients remained seizure-free throughout follow-up.ConclusionSome HMSs can originate from the temporal lobe. In carefully selected patients, surgical resection may lead to good outcomes.     

Anaplastic hemangiopericytoma in the frontal horn of the lateral ventricle

Intracranial hemangiopericytomas are uncommon tumors, and their intraventricular occurrence is even rarer. We report a 40-year-old man who presented with raised intracranial pressure. His MRI showed a 3.3 × 3.2 × 3.2 cm heterogeneously enhancing lesion in the left frontal horn obstructing the foramen of Monro and causing hydrocephalus. The tumor was excised through an anterior interhemispheric, transcallosal approach, and histopathology revealed an anaplastic hemangiopericytoma (World Health Organization grade III). To our knowledge this is the first report of this rare pathology being located within the frontal horn of the lateral ventricle.