Large Middle Cranial Fossa Schwannoma: A Rare Presentation of Vestibular Schwannoma.

Schwannomas are benign tumors composed of neoplastic Schwann cells and rarely occur in the central nervous system. Schwannomas account for approximately 8% of intracranial tumors and most commonly originate from cranial nerve VIII at the cerebellopontine angle in the posterior fossa. Herein, we report two cases of vestibular schwannomas extending in the middle fossa. The first case shows a 51-year-old male who presented with a history of mild headaches for one year associated with acute nausea, vomiting, and word-finding difficulties. Imaging revealed a large multicystic contrast-enhancing lesion in the left middle cranial fossa. The middle fossa lesion was resected with pathology indicating a schwannoma. The second case shows a 63-year-old woman who presented with seizures, right-sided hearing loss, and right-sided facial weakness. On MRI, she is found to have a large right middle fossa lesion originating from the right internal auditory canal and consistent with vestibular schwannoma with a 9 mm leftward midline shift. The histopathologic examination of the excised tumor indicated a schwannoma. Schwannomas most commonly occur in the posterior fossa when they present intracranially. However, in rare occurrences, they may present as middle fossa masses with significant intracranial compression.

Gliomas: survival differences between metropolitan and non-metropolitan counties.

BACKGROUND: For gliomas, metropolitan status has not been heavily explored in the context of short-term mortality or long-term observed survival. Larger populations are associated with proximity to academic universities/high-volume hospitals. METHODS: The SEER-18 registry was queried for patients with gliomas. The patients were further classified into two population groups based on rural-urban continuum codes: metropolitan or non-metropolitan. Demographics and clinical factors were compared between both groups. For observed survival, univariate and multivariate analyses occurred with Cox proportional hazards model. RESULTS: The non-metropolitan group constituted approximately 10.8% of all patients. Age at diagnosis of glioma was older for the non-metropolitan group compared to metropolitan group (51.60 years vs. 49.06 years). Relative to the metropolitan group, the non-metropolitan group exhibited a larger proportion of Caucasian, married, grade I and IV gliomas, no surgery, no GTR (for those who had surgery), and temporal/parietal/occipital locations. Other covariates (sex, tumor size, laterality, and radiation status) did not exhibit significant differences in proportions. From analysis of observed survival, independent predictors include population group, as well as age, gender, marital status, tumor location, tumor grade, laterality, GTR, and receipt of radiation. Short-term mortality was 11.68% and 13.04% for Metropolitan and non-metropolitan groups, respectively. Median survival was 15 months and 12 months for Metropolitan and non-metropolitan groups, respectively. CONCLUSIONS: About one-tenth of gliomas are treated at non-metropolitan sites. Key differences exist among patient/glioma characteristics based on metropolitan status. Overall, metropolitan status appears to influence short-term mortality and long-term observed survival for gliomas.

Causes of death in glioblastoma: insights from the SEER database.

BACKGROUND: Glioblastoma (GB) and its variants portend a poor prognosis. The predominant cause of death (COD) is related to the cancer diagnosis, but a significant subset is related to other causes. As GB is a systemic disease requiring systemic treatment, focus regarding all COD provides a comprehensive illustration of the disease. METHODS: The SEER-18 was queried for patients with cranial GB and its variants. Age, gender, race, marital status, tumor characteristics, treatment details, and follow-up data were acquired. The patients were classified into group A (death attributed to this cancer diagnosis) or group B (death attributed to causes other than this cancer diagnosis). RESULTS: From 1973 to 2013, 36,632 deaths (94%) constituted group A, and 2,324 deaths (5.9%) constituted group B. The latter significantly exhibited lower proportions of age <60, Caucasians, married status, frontal/brain stem/ventricle tumor locations, and receipt of radiation. From logistic regression, age >60, male gender, race, not married, tumor location, and no radiation were significant independent predictors for group B. The top known CODs in group B are diseases of heart, pneumonia and influenza, cerebrovascular diseases, accidents and adverse effects, and infections. CONCLUSIONS: CODs not attributed to GB remains a significant subset of all CODs. Many of these, particularly diseases of heart, are frequent comorbidities. Moreover, infection-related CODs after GB diagnosis appear more salient compared to CODs in the general population. Consideration of these CODs, and vigilant treatment aimed at these CODs, may improve overall care for GB patients.

Management and survival trends for adult patients with malignant gliomas in the setting of multiple primary tumors: a population based analysis.

INTRODUCTION: The impact of multiple primary tumors, in the setting of malignant glioma (MG), has not been heavily explored. METHODS: We extracted demographics and clinical data from the SEER-18 registry for adult patients with MGs. The cases were separated based on the sequence of MG diagnosis relative to the other primary tumors: Group (A) One primary only or first primary of multiple primaries and Group (B) second primary or subsequent primary tumor. Incidences, frequencies, and glioma-related survivals were analyzed. RESULTS: Group B constituted 12.8% of new MG. The incidences of group B, relative to those of all new MG, range from 0.14 to 0.18. Compared to group A, group B exhibited an older age. Moreover, group B exhibited a higher proportion of females, Caucasians, smaller tumors, non-operative cases, and those receiving radiation (p < 0.05); the proportion with GTR remained comparable. Multiple groupings (oral cavity, digestive system, respiratory system, skin, breast, genital systems, urinary system, lymphoma) exhibited lower glioma-related observed survival (p < 0.05) compared to Group A. An active diagnosis of "leukemia" appears to confer longer glioma-related survival while a history of "breast" or "digestive system" malignancies portends a shorter glioma-related survival. CONCLUSION: For newly diagnosed MG, a high proportion does have history of extra-CNS primary tumors. Generally, these patients appear to have worse glioma-related observed survival compare to those with malignant glioma as the only primary or the first of multiple primary tumors. Knowledge regarding epidemiology, clinical factors, and observed survival can help guide clinical management/consultation for this subset of patients.

Molecular Mechanism of Inhibition of Acid Ceramidase by Carmofur.

Human acid ceramidase (AC) is a lysosomal cysteine amidase, which has received a great deal of interest in recent years as a potential target for the development of new therapeutics against melanoma and glioblastoma tumors. Despite the strong interest in obtaining structural information, only the structures of the apo-AC enzyme in its zymogen and activated conformations are available. In this work, the crystal structure of AC in complex with the covalent carmofur inhibitor is presented. Carmofur is an antineoplastic drug containing an electrophilic carbonyl reactive group that targets the catalytic cysteine. This novel structural data explains the basis of the AC inhibition, provides insights into the enzymatic properties of the protein, and is a great aid toward the structure-based drug design of potent inhibitors for AC, providing the detailed mechanism, which has eluded the scientific community for more than 30 years, of carmofur's mysterious 5-fluorouracil-independent antitumor activity.

Methotrexate-induced Leukoencephalopathy without Typical Restricted Diffusion on Diffusion-weighted Imaging and the Utility of Magnetic Resonance Spectroscopy to Support the Diagnosis.

Methotrexate (MTX) is a common antimetabolite agent that is widely used today in treating leukemia, lymphoma, and osteosarcoma. Its use has been associated with leukoencephalopathy causing seizures, paralysis, and even coma. To achieve the best possible outcome, it is important to be able to make a prompt diagnosis. Studies reported restricted diffusion on diffusion-weighted imaging (DWI) which is a reliable early sign of acute MTX-induced leukoencephalopathy. However, we report here the first case of MTX-induced leukoencephalopathy without typical restricted diffusion on DWI and the utility magnetic resonance spectroscopy to support this diagnosis in the difficult case such as the one being presented here.

Glioblastoma in the setting of prior lower grade gliomas - insights from SEER database.

INTRODUCTION: Secondary glioblastomas (GBs) constitute a small subset of all GBs and tend to arise after a lower grade glioma. Though knowledge regarding this subset has gained traction in recent years, its definition continues to evolve, complicating its clinical management. Investigation of epidemiology and survival patterns may help provide needed insights. RESULTS: The age at GB diagnosis is significantly lower (46.22 vs 60.25 years) for group B. The distribution among type of GB (glioblastoma, giant cell glioblastoma, or gliosarcoma) was significantly different, with no diagnosis of giant cell GB in Group B. Compared to Group A, Group B exhibited a higher proportion of females, not married, smaller tumors, no GTR, and no radiation (all p < 0.05). GB-related observed survivals were comparable. Cox regression with inclusion of co-variates reveal no significant influence of GB group on observed survival. Regarding group B, mean age was 40.197 for diagnosis of initial lower grade glioma. The most common initial ICD-O-3 pathology was oligodendroglioma, NOS; astrocytoma, NOS; astrocytoma, anaplastic; and mixed glioma. METHODS: The SEER-18 registry was queried for patients with GBs. Patients were further classified into two GB groups: Group A - those with GB as the only primary tumor, and Group B - those with GB as a 2nd primary or subsequent tumor and with history of lower grade gliomas. Demographics and clinical factors were compared between group A and B. Appropriate statistics were employed to calculate incidences and differences among factors and GB-related survivals between the groups. CONCLUSIONS: Overall, Group B develops GBs at an earlier age, but observed survival remains similar to those with GBs as the only primary. Moreover, this subset also exhibit different proportions of the types of GBs, and well as differences in other key clinical factors (namely, gender and tumor size at presentation). Prior treatments for lower grade gliomas likely explain some of the differences noted regarding management course after diagnosis of GB.

Intracranial hemangioblastoma - A SEER-based analysis 2004-2013.

INTRODUCTION: Intracranial hemangioblastoma (HB) is a rare pathology. Limited data exist regarding its epidemiology. METHODS: With the SEER-18 registry database, information from all patients diagnosed with intracranial HB from 2004 to 2013 were extracted, including age, gender, race, marital status, presence of surgery, extent of surgery, receipt of radiation, tumor size, tumor location, and follow-up data. Age-adjusted incidence rates and overall survival (OS). Cox proportional hazards model was employed for both univariate and multivariate analyses. RESULTS: A total of 1307 cases were identified. The overall incidence of intracranial hemangioblastoma is 0.153 per 100,000 person-years [95% confidence interval (CI)=0.145-0.162]. Through univariate analysis, age < 40 [hazard ratio (HR)=0.277, p<0.001], no radiation [HR=0.56, p=0.047], and presence of surgery [HR=0.576, p=0.012] are significant positive prognostic factors. Caucasian race [HR=1.42, p=0.071] and female gender [HR=0.744, p=0.087] exhibit noticeable trends towards positive prognosis. Through multivariate analysis, younger age [HR=1.053, p < 0.01], race [HR=1.916, p<0.01], and presence of surgery [HR=0.463, p<0.01 were significant independent prognostic factors. CONCLUSION: Clinical factors such as younger age, Caucasian race, and presence of surgery are significant independent factors for overall survival in patients with HBs. Though analysis regarding extent of surgery did not produce a meaningful relationship, this may be related to surgical bias / expertise. Moreover, no validation for radiation therapy was identified, but this may be related to short follow up intervals and the variable growth patterns of HBs.

Subependymal Giant Cell Astrocytoma: A Surveillance, Epidemiology, and End Results Program-Based Analysis from 2004 to 2013.

BACKGROUND: Subependymal giant cell astrocytoma (SEGA) is a rare, benign neoplasm predominantly associated with tuberous sclerosis complex. Clinical outcomes have largely been conveyed via small- and medium-sized case series. METHODS: With the Surveillance, Epidemiology, and End Results Program (SEER)-18 registry database, information from all patients diagnosed with SEGA from 2004 to 2013 was obtained (age, sex, race, marital status, tumor size, tumor location, occurrence of surgery, receipt of radiation, and follow-up data). Age-adjusted incidence rates and overall survival (OS) were determined. Cox proportional hazards model was used for both univariate and multivariate analyses. RESULTS: The overall incidence of SEGA within the SEER-18 database is 0.027 per 100,000 person-years (95% confidence interval, 0.024-0.031). A total of 226 cases were identified. For OS, univariate analysis revealed age younger than 18 years (hazard ratio [HR], 0.214; P = 0.004) and occurrence of surgery (HR, 0.328; P = 0.039) were significant positive prognostic factors. Sex, marital status, race, tumor size, tumor location, and receipt of radiation did not exhibit significant relationships. Interestingly, subanalysis for extent of resection to gross total resection did not show benefit. Multivariate analysis revealed that both age younger than 18 years (HR, 0.193; P = 0.002) and occurrence of surgery (HR, 0.286; P = 0.021) remained significant. CONCLUSIONS: Based on our analysis, younger age and occurrence of surgery are significant independent factors associated with better OS. There was no support for radiation.

Mechanism for the Rapid Spontaneous Resolution of an Acute Subdural Hematoma and Transformation into a Subdural Hygroma.

BACKGROUND: Spontaneous resolution of acute subdural hematomas (SDHs), occurring as the result of a traumatic injury, is well reported in the literature and rapidly resolving SDHs have been reported as well. Although the mechanism behind the rapid resolution of a SDH has not been well understood, there are postulated mechanisms for this phenomenon. The aim of this report is to present a patient who experienced the rapid resolution of a traumatic SDH, which was replaced by a subdural hygroma. CASE DESCRIPTION: This 25-year-old man presented to the hospital with an acute left-sided SDH after a head-on motor vehicle collision. At the time of presentation, the patient had an acute SDH with 11 mm of midline shift. The hematoma resolved spontaneously within 48 hours and was replaced by a thin subdural hygroma. CONCLUSIONS: Rapidly resolving SDHs represent a phenomenon that has been well described, although is not entirely understood. The imaging findings of this patient suggest that cerebrospinal fluid washout is a mechanism that promotes acute SDH resolution.

A Novel Application of the Integra Camino Bolt for the Drainage of Chronic Subdural Hematoma: A Technical Note.

Chronic subdural hematoma (cSDH) is a common neurosurgical pathology associated with older age and complicated by antiplatelet/anticoagulant therapies. With improving medical care in an aging population, the incidence of cSDH will likely increase over the next 25 years, placing a burden on health care costs. Consequently, a simple and inexpensive treatment option is desirable. As such, we report a basic, but novel, technique to drain cSDH with an Integra Camino bolt. This technique was employed in two patients, where the significant resolution of cSDH and associated clinical symptoms were observed without complications.

Good Outcomes with the Intraventricular Vancomycin Therapy in a Patient with Ruptured Brain Abscesses.

Brain abscesses are associated with high morbidity and mortality rates. In particular, patients with intraventricular rupture of brain abscess (IVROBA) exhibit mortality rates up to 85%. Treatment options are lacking for IVROBA, once patients become refractory to intravenous antibiotics and surgical drainage. Limited data exist regarding the risks and benefits of intraventricular therapy in such a scenario. We report a patient with IVROBA, who deteriorated while on systemic antibiotics; once intraventricular vancomycin was employed, the patient demonstrated remarkable improvement without perceivable side effects. This case suggests that intraventricular vancomycin may be a safe, effective, and viable option for the treatment of IVROBA, especially for patients becoming refractory to systemic antibiotics.

A Rare Extra-Axial Midline Tentorial Adult Medulloblastoma with Dural-Tail Sign Mimicking a Meningioma.

Medulloblastomas are categorized as the World Health Organization Grade IV neoplasms. Only 33 cases have been reported of extra-axial, mostly in the cerebellar pontine angle and lateral cerebellar hemisphere, medulloblastomas in the current literature. Our study showcases the first case of an extremely rare presentation of an extra-axial midline tentorial adult medulloblastoma with the dural-tail sign mimicking a meningioma. To achieve the best possible outcome, a high index of suspicion for medulloblastoma is critical especially in young patient with an atypical posterior fossa mass as treatment regimens drastically different between a medulloblastoma and a meningioma.

Safe Use of Tissue Plasminogen Activator in Conjunction with the Integra Camino Bolt for the Drainage of Chronic Subdural Hematoma.

Chronic subdural hematoma (CSDH) is a relatively common condition encountered in a neurosurgical practice. There have been increased efforts in creating different treatment regimens for CSDH to improve patients' outcomes, including the addition of tissue plasminogen activator (tPA) in drains to reduce recurrences. Here, we present the first case report of the safe use of tPA in conjunction with an Integra Camino bolt for maximized drainage of CSDH with a successful neurological recovery and the complete resolution of the hematoma.

Dysembryoplastic Neuroectodermal Tumor: An Analysis from the Surveillance, Epidemiology, and End Results Program, 2004-2013.

BACKGROUND: Dysembryoplastic neuroectodermal tumor (DNT) is a rare neoplasm. Though the pathology is commonly considered benign, there have been various reports documenting rapid growth, recurrence/progression, sudden death, and malignant transformation. Most studies have addressed outcomes regarding seizure control, but limited data exist regarding incidence and survival. Consequently, we explore the Surveillance, Epidemiology, and End Results (SEER) database to explore the epidemiology of DNT. METHODS: From the SEER-18 registry database, information from all patients diagnosed with intracranial DNT between 2004 and 2013 was extracted, including age, sex, race, marital status, tumor location, tumor size, receipt of surgery, extent of primary surgery, receipt of radiation, and follow-up data. Age-adjusted incidence rates and overall survival (OS) were calculated. A Cox proportional hazards model was used to assess relationships between various demographic/treatment variables and OS. RESULTS: A total of 381 cases were identified in the SEER-18 database. The incidence of DNT within the large subset of the United States population represented by SEER was 0.033 per 100,000 person-years (95% confidence interval [CI], 0.030-0.037). The median duration of follow-up was 50 months. The median OS was not attained. The 3-, 5-, and 9-year OS was 99.363% (95% CI, 97.428%-99.844%), 97.993% (95% CI, 95.168%-99.174%), and 96.296% (95% CI, 91.834%-98.341%), respectively. Seven of the 381 patients passed at their last follow up. Of all demographic/treatment factors, only receipt of radiation demonstrated a significant relationship with OS (hazard ratio, 0.051; 95% CI, 0.01-0.267; P < 0.01). CONCLUSIONS: Although the prognosis for DNT is generally favorable, the pathology can lead to poor outcomes in rare cases. Common demographic factors, treatment with surgery, and the extent of surgical resection did not show significant associations with OS. In contrast, treatment with radiation was associated with poorer OS.

Intracranial Subependymoma: A SEER Analysis 2004-2013.

BACKGROUND: Subependymomas are rare, slow-growing, benign tumors. Because they are scarce, knowledge relating to survival remains lacking. Consequently, we explore the SEER database to evaluate prognostic and treatment factors associated with intracranial subependymoma. METHODS: With the SEER-18 registry database, information from all patients with intracranial subependymoma diagnosed during 2004-2013 were extracted, including age, sex, race, occurrence of surgery, extent of primary surgery, receipt of radiation, tumor size, and follow-up data. Age-adjusted incidence rates, overall survival, and cause-specific survival were calculated. Cox proportional hazards model was used for both univariate and multivariate analyses. RESULTS: Four hundred sixty-six cases were identified. The overall incidence of intracranial subependymoma is 0.055 per 100,000 person-years (95% confidence interval, 0.05-0.06). Through multivariate analysis, age <40 years (hazard ratio [HR], 0.21; P = 0.03), female sex (HR, 0.34; P = 0.03), location within ventricles or near brainstem (HR, 0.49; P = 0.04), and occurrence of surgery (HR, 0.50; P = 0.02) were significant independent positive prognostic factors. Receipt of radiation did not show a significant relationship. CONCLUSION: Clinical factors such as younger age, female sex, and location within ventricles or near brain stem demonstrated positive relationship with overall survival. For treatment options, surgery remains a mainstay option. No support for radiation therapy was identified.

Patients with Blunt Traumatic Brain Injury: A Role for Computed Tomography Angiography of the Head to Evaluate Nontraumatic Causes?

BACKGROUND: In the setting of trauma, the cause of intracranial hemorrhage (ICH) is frequently attributed to the physical, traumatic event. Caution should still be directed toward nontraumatic (or spontaneous) causes responsible for the trauma, such as hypertension, cerebral amyloid angiopathy, aneurysms, vascular malformation, and hemorrhagic infarcts. The role for immediate computed tomography angiography remains controversial to evaluate for nontraumatic causes. METHODS: A systematic review of the available literature in the Medline PubMed database. RESULTS: In the available literature, only 12 patients with traumatic brain injury underwent computed tomography angiography of the head that either showed a vascular malformation and/or altered clinical management because of concerns of a vascular malformation. The ICH in 11 patients was attributed to rupture of a cerebral aneurysm; the other patient received a diagnostic angiogram that was negative. CONCLUSIONS: ICH in patients with traumatic brain injury seems to be vastly associated with the traumatic event. Only rare cases have been attributed to aneurysmal rupture. None has been associated with arteriovenous malformation. Nevertheless, clinical vigilance remains reasonable, especially in younger patients and those with hemorrhage within the subarachnoid cisterns or sylvian fissure.

Intracranial subdural empyema mimicking a recurrent chronic subdural hematoma.

Intracranial subdural empyema (ISDE) is a life-threatening condition. The risk for ISDE increases in patients that have undergone prior intracranial procedures. The non-specificity in its clinical presentation often makes ISDE difficult to diagnose. Here, we present a rare case of ISDE mimicking a recurrent chronic subdural hematoma, emphasizing the significance of obtaining early magnetic resonance images of the brain for early diagnosis and treatment to achieve the optimal outcome.

Pure tentorial subdural hematoma from rupture of aneurysm along the transmastoid branches of the occipital artery.

BACKGROUND: Pure subdural hematoma (without subarachnoid, intraventricular, or intraparenchymal hemorrhage) due to a ruptured intracranial aneurysm is rare. Most reported cases involve an aneurysm along the internal carotid artery, posterior communicating artery, or middle cerebral artery. No reports have described an aneurysm along the transmastoid branches of the occipital artery. CASE DESCRIPTION: A 70-year-old female presented with sudden-onset, excruciating headaches, associated with dizziness, nausea, and emesis. There was no history of trauma. Computed tomography (CT) head demonstrated a pure tentorial subdural hematoma. Vascular imaging revealed bilateral aneurysms along the transmastoid branches of the intracranial portion of both the occipital arteries. Consequently, these branches were embolized, with no residual filling of the aneurysms. After the procedure, the patient remained neurologically well. The patient was monitored appropriately for vasospasm, and was discharged home 10 days after presentation. CONCLUSION: Rupture of aneurysms along intracranial branches of the occipital artery can lead to pure subdural hematoma along the tentorium.

Glioblastoma Mimicking Meningioma: Report of 2 Cases.

BACKGROUND: Glioblastoma can mimic various pathologies, including arteriovenous malformation, hemorrhage from ischemic stroke, cerebral contusion, metastatic disease, lymphoma, and infection. The literature is limited regarding diagnostic confusion with meningioma. Herein, we present 2 patients that exhibited imaging, including cerebral angiography during preoperative embolization, which was consistent with meningioma, but where final surgical diagnosis revealed glioblastoma. CASE DESCRIPTION: Case 1 was a 57-year-old woman presenting with headache, ataxia, and memory lapses for the past month. Brain magnetic resonance imaging (MRI) demonstrated a heterogeneous-enhancing right temporoparietal mass with broad contact along the right tentorium, cerebrospinal fluid (CSF) cleft sign, and dural tail sign-consistent with meningioma. Patient underwent angiography with successful polyvinyl alcohol foam (PVA) particle embolization of the petrosquamosal branch of the right middle meningeal artery (MMA) and meningeal branch of the right occipital artery, resulting in significant devascularization of the tumor blush. Subsequently, the patient underwent tumor resection, where pathology revealed glioblastoma. Case 2 was a 60-year-old man presenting with right hemiparesis. Brain MRI demonstrated a left parasagittal, heterogeneous-enhancing mass abutting the falx with a dural tail sign-consistent with meningioma. Patient underwent angiography with successful PVA particle embolization of the left MMA, resulting in significant devascularization of the tumor blush. Patient underwent a tumor resection where pathology revealed glioblastoma. CONCLUSIONS: Glioblastoma can mimic meningioma on MRI with dural tail sign, CSF cleft sign, and broad dural contact. Moreover, cerebral angiography can reveal tumor feeders commonly associated with meningioma. These features can contribute to diagnostic confusion. Based on these 2 cases, preoperative embolization of tumor feeders is possible with glioblastoma.