Clinical considerations and surgical approaches for low-grade gliomas in deep hemispheric locations: thalamic lesions.

BACKGROUND: Tumors with epicenter in the thalamus occur in about 4 % of pediatric brain tumors. The histological diagnosis is mainly gliomas. Among them, low-grade glioma (LGG) constituted of a significant entity of the tumors (Cuccia et al., Childs Nerv Syst 13:514-521, 1997; Puget et al., J Neurosurg 106:354-362, 2007; Bernstein et al., J Neurosurg 61:649-656, 1984; Bilginer et al., Childs Nerv Syst 30:1493-1498, 2014). Since Kelly's report in 1989, >90 % resection of thalamic tumors were achieved in reported series (Ozek and Ture, Childs Nerv Syst 18:450-6, 2002; Villarejo et al., Childs Nerv Syst 10:111-114, 1994; Moshel et al., Neurosurgery 61:66-75, 2007; Albright, J Neurosurg 100(5 Suppl Pediatrics): 468-472, 2004; Kelly, Neurosurgery 25:185-195, 1989; Drake et al., Neurosurgery 29: 27-33, 1991). MATERIALS AND METHODS: Sixty-nine cases of thalamic tumors in children were retrospectively reviewed. There were 25 cases of LGGs. We analyzed our experience and correlated it with reported series. RESULTS: Summing up of 4 reported series and the present series, there were 267 cases of thalamic tumors in children. Among these tumors, 107 (40.1 %) were LGGs and 91 (34.1 %) were low-grade astrocytomas (LGAs). In the present series, all of the 25 LGGs were LGAs that consisted of 11 pilocytic astrocytomas (PAs) and 14 diffuse astrocytomas (DAs). Six cases received biopsy sampling only. The remaining 19 cases received different degrees of surgical resection via several approaches. Radical (>90 %) resection was achieved better in PAs comparing with DAs. There was no operative mortality. Two patients had increased neurological deficits. In a mean follow-up period of 11.9 years, three patients died of tumor progression and one patient died of anaplastic change. The 5- and 10-year overall survival (OS) was 87.1 and 87.1 %, respectively. CONCLUSION: Thalamic LGGs are mainly LGAs and are indolent. The rate of >90 % resection was relatively low in the present series. By applying contemporary diagnostic MRI studies, surgical facilities, and appropriate approaches in selective cases, we may try maximum neuroprotective radical (>90 %) resection.

Prognosis of glioblastoma with faint MGMT methylation-specific PCR product.

Methylation-specific polymerase chain reaction (MSP) for the promoter methylation status of O(6)-methylguanine-DNA-methyltranferase (MGMT) gene theoretically provides a positive or negative result. However, the faint MSP product is difficult to interpret. The aim of this study was to evaluate the significance of faint MSP product in glioblastoma (GBM). Critical concentrations of methylated control DNA, i.e., 100, 1, 0.5 and 0 % were run parallel with 116 newly diagnosed GBMs in order to standardize the interpretation and to distinguish positive (+), equivocal (±), and negative (-; unmethylated) results. Cases with the faint MSP product and its intensity between those of 1 and 0.5 % DNA controls were considered equivocal (±). MGMT methylation quantifications were also determined by quantitative real-time MSP (qMSP) and pyrosequencing (PSQ), and protein expression was detected by immunohistochemistry. There were significant correlations between MSP and all the aforementioned studies. The concordance rates between the MSP+ and qMSP+ cases, as well as the MSP- and qMSP- cases were 100 %, and the MSP± cases comprised 76.5 % of qMSP+ cases and 23.5 % of qMSP- cases. PSQ study showed that heterogeneous methylation was more frequently encountered in the MSP± cases. Multivariate analyses disclosed that although the overall survival of the MSP± cases was indistinct from that of the MSP+ cases, its progression free survival was significantly worse and was indistinct from that of the MSP- cases. In conclusion, GBMs with faint MGMT MSP products should be distinguished from MSP+ cases as their behaviors were different.

Factors affecting survival of medulloblastoma in children: the changing concept of management.

Medulloblastoma (MB) is a type of malignant tumor arising only in the cerebellum that was first defined by Cushing and Bailey in 1920s. In this review paper, we trace the evolution of risk stratification and the correlated changing concept of management in the past years. Outcome analysis of the hospital series of the Taipei Veterans General Hospital, Cheng Hsin General Hospital, and Taipei Medical University Hospital was performed to correlate prognostic indicators with reported studies. The purpose is to provide clues for age-specific and risk-adjusted optimal, effective, but beneficial and protective treatment strategies of these tumors in children.

Simulation and evaluation of increased imaging service capacity at the MRI department using reduced coil-setting times.

The wait times for patients from their appointments to receiving magnetic resonance imaging (MRI) are usually long. To reduce this wait time, the present study proposed that service time wastage could be reduced by adjusting MRI examination scheduling by prioritizing patients who require examinations involving the same type of coil. This approach can reduce patient wait times and thereby maximize MRI departments' service times. To simulate an MRI department's action workflow, 2,447 MRI examination logs containing the deidentified information of patients and radiation technologists from the MRI department of a medical center were used, and a hybrid simulation model that combined discrete-event and agent-based simulations was developed. The experiment was conducted in two stages. In the first stage, the service time was increased by adjusting the examination schedule and thereby reducing the number of coil changes. In the second stage, the maximum number of additional patients that could be examined daily was determined. The average number of coil changes per day for the four MRI scanners of the aforementioned medical center was reduced by approximately 27. Thus, the MRI department gained 97.17 min/d, which enabled them to examine three additional patients per month. Consequently, the net monthly income of the hospital increased from US$17,067 to US$30,196, and the patient wait times for MRI examinations requiring the use of flexible torso and head, shoulder, 8-inch head, and torso MRI coils were shortened by 6 d and 23 h, 2 d and 15 h, 2 d and 9 h, and 16 h, respectively. Adjusting MRI examination scheduling by prioritizing patients that require the use of the same coil could reduce the coil-setting time, increase the daily number of patients who are examined, increase the net income of the MRI department, and shorten patient wait times for MRI examinations. Minimizing the operating times of specific examinations to maximize the number of services provided per day does not require additional personnel or resources. The results of the experimental simulations can be used as a reference by radiology department managers designing scheduling rules for examination appointments.

Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit.

BACKGROUND: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)-characterized as tumors mimicking malignant orbital lymphoma (OL)-responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. METHODS: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. RESULTS: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions-involving both lacrimal glands on CT scans-higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674-1.000, p = 0.005] vs. 0.766 [95% CI: 0.615-0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518-0.997] vs. 0.75 [95% CI: 0.509-0.913]), higher specificity (0.813 [95% CI: 0.544-0.960] vs. 0.778 [95% CI: 0.578-0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). CONCLUSIONS: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

Radiation carotid blowout syndrome in nasopharyngeal carcinoma: angiographic features and endovascular management.

OBJECTIVE: To report clinical manifestations, angiographic features, and outcomes of endovascular management in 14 patients with 15 radiation carotid blowout syndromes of nasopharyngeal carcinomas. STUDY DESIGN AND SUBJECTS: Retrospective chart review of 14 patients with nasopharyngeal carcinomas (mean age 49 years) with 15 radiation carotid blowout syndromes who had undergone endovascular embolization to manage oronasal bleeding in the past 10 years. RESULTS: Average radiation dose to affected carotid artery was 73 gray units (latent period: 33 months). Radiation carotid blowout syndrome was detected in internal (n = 10), external (n = 4), or common carotid artery (n = 1). Detachable balloons were used in 11 affecting arteries for vascular occlusion; 4 were treated by liquid adhesives or coil. Endovascular treatment was successful in all 15 radiation carotid blowout syndromes with cessation of hemorrhage. One patient had hemiparesis after embolization. Mean clinical follow-up was 21 months. CONCLUSION: Radiation carotid blowout syndrome in nasopharyngeal carcinoma may occur in various periods or arteries. Endovascular embolization provides both safe and effective management.

Prenatal diagnosis of pulmonary sequestration by ultrasound and magnetic resonance imaging.

A 36-year-old multigravida, G2P1, underwent routine ultrasound scan at 22+1 weeks of gestation, which revealed a single normally growing fetus with left intrathoracic mass and left displacement of the cardiac apex. The left intrathoracic wedge-shaped hyperechogenic mass, measuring 32 x 25 mm in size, was situated at the lower portion of the left lung. A combination of color and power Doppler ultrasound allowed visualization of a vessel arising from the descending aorta, which supplied the mass. The diagnosis of extralobar pulmonary sequestration was made. Magnetic resonance imaging (MRI) was also performed and revealed a well-defined mass with homogeneous high-signal intensity when compared with normal lung tissue in the left upper lung field, which was compatible with pulmonary sequestration. The pulmonary mass was followed up by color and power Doppler every 2 weeks. The peak velocity of 11.85 cm/sec and the diameter of the feeding artery of 1.19 mm gradually decreased and disappeared 8 weeks later. The intrathoracic mass disappeared 10 weeks later at 32+1 gestational weeks. Repeat MRI also revealed spontaneous regression of the mass in favor of resorption of sequestration. The fetus was delivered at 38+1 gestational weeks. A male newborn weighing 2,520 g was spontaneously delivered with an Apgar score of 8 at 1 minute and 9 at 5 minutes. In our patient, it is suggested that progressive decreases in the peak velocity of the feeding vessel heralded the spontaneous regression of pulmonary sequestration not associated with hydrops/hydrothorax.

Paradoxical carbon dioxide embolism during pneumoperitoneum in laparoscopic surgery for a huge renal angiomyolipoma.

We present a case of paradoxical gas embolism during CO2 insufflation in laparoscopic nephrectomy for a huge renal angiomyolipoma. Paradoxical CO2 embolism in the left heart chambers without demonstrable intracardiac right-to-left shunt was detected by transesophageal echocardiography (TEE). The surgical procedure was stopped immediately, but the patient recovered with mild neurologic deficit. We speculate that rapid pneumoperitoneum introduction pushed CO2 into the abnormal vasculature of the angiomyolipoma, which communicates with the systemic vascular system, causing pseudoaneurysm formation. Follow-up abdominal computed tomography showed a new pseudoaneurysm inside the tumor. If intracardiac right-to-left shunt is excluded for the reason of paradoxical gas existence, there remains extracardiac right-to-left shunt, with transpulmonary passage of the venous emboli being the most likely mechanism. In fact, the cause of paradoxical gas embolism in this case remains unknown. Therefore, laparoscopic surgery for huge angiomyolipoma should be performed with extreme caution; an open procedure may be considered as an alternative.

Computed tomography and magnetic resonance imaging findings of intraorbital granular cell tumor (Abrikossoff's tumor): a case report.

BACKGROUND: Granular cell tumors are rare neoplasms which can occur in any part of the body. Granular cell tumors of the orbit account for only 3 % of all granular cell tumor cases. Computed tomography and magnetic resonance imaging of the orbit have proven useful for diagnosing orbital tumors. However, the rarity of intraorbital granular cell tumors poses a significant diagnostic challenge for both clinicians and radiologists. CASE PRESENTATION: We report a case of a 37-year-old Chinese woman with a rare intraocular granular cell tumor of her right eye presenting with diplopia, proptosis, and restriction of ocular movement. Preoperative orbital computed tomography and magnetic resonance imaging with contrast enhancement revealed an enhancing solid, ovoid, well-demarcated, retrobulbar nodule. In addition, magnetic resonance imaging features included an intraorbital tumor which was isointense relative to gray matter on T1-weighted imaging and hypointense on T2-weighted imaging. No diffusion restriction of water was noted on either axial diffusion-weighted images or apparent diffusion coefficient maps. Both computed tomography and magnetic resonance imaging features suggested an intraorbital hemangioma. However, postoperative pathology (together with immunohistochemistry) identified an intraorbital granular cell tumor. CONCLUSIONS: When intraorbital T2 hypointensity and free diffusion of water are observed on magnetic resonance imaging, a granular cell tumor should be included in the differential diagnosis of an intraocular tumor.

Gamma Knife Radiosurgery for Atypical and Anaplastic Meningiomas.

BACKGROUND: Atypical and anaplastic meningiomas have much higher recurrence rates after surgical resection compared with benign meningiomas, but the role of adjuvant radiosurgery remains unclear. This study was undertaken to evaluate the outcomes of gamma knife radiosurgery for patients with atypical and anaplastic meningiomas. METHODS: In this retrospective analysis of a prospectively maintained database, 46 patients with histologically proven atypical or anaplastic meningiomas by current World Health Organization (WHO) criteria underwent postoperative Gamma Knife radiosurgery between 1993 and 2013. The median follow-up period was 32.6 months. The median tumor volume and margin dose were 11.7 mL (range, 2-53 mL) and 13.1 Gy (range, 12.0-16.5 Gy), respectively. RESULTS: Local control at 3 and 5 years was 50.6% and 32.1%, respectively. Gender (P = 0.013) and marginal dose less than or equal to 13 Gy (P = 0.049) were associated with the local control. The 3- and 5-year overall survival for patients with WHO grade II was 97.1% and 88.3%, respectively, compared with 66.7% and 66.7% for patients with WHO grade III meningiomas. Radiation therapy before Gamma Knife radiosurgery (GKRS; P = 0.018) and tumor grade (P = 0.019) were the factors associated with a worse overall survival rate. Fourteen patients (30.4%) developed adverse radiation effects after GKRS treatment, and all were Radiation Therapy Oncology Group grade I. CONCLUSIONS: Postoperative GKRS treatment for patients with atypical and anaplastic meningioma is challenging. More aggressive treatment, including of safely maximizing the extent of surgical resection and using a higher margin dose (>13Gy), should be applied to achieve better local control.

Gamma knife surgery for cavernous hemangiomas: an analysis of 125 patients.

OBJECT: The authors sought to determine the value of gamma knife surgery (GKS) in the treatment of cavernous hemangiomas (CHs). METHODS: Between 1993 and 2002, a total of 125 patients with symptomatic CHs were treated with GKS. Ninety-seven patients presented with bleeding and 45 of these had at least two bleeding episodes. Thirteen patients presented with seizures combined with hemorrhage, and 15 patients presented with seizures alone. The mean margin dose of radiation was 12.1 Gy and the mean follow-up time was 5.4 years. In the 112 patients who had bled the number of rebleeds after GKS was 32. These rebleeds were defined both clinically and based on magnetic resonance imaging for an annual rebleeding rate of 32 episodes/492 patient-years or 6.5%. Twenty-three of the 32 rebleeding episodes occurred within 2 years after GKS. Nine episodes occurred after 2 years; thus, the annual rebleeding rate after GKS was 10.3% for the first 2 years and 3.3% thereafter (p = 0.0038). In the 45 patients with at least two bleeding episodes before GKS, the rebleeding rate dropped from 29.2% (55 episodes/188 patient-years) before treatment to 5% (10 episodes/197 patient-years) after treatment (p < 0.0001). Among the 28 patients who presented with seizures, 15 (53%) had good outcomes (Engel Grades I and II). In this study of 125 patients, symptomatic radiation-induced complications developed in only three patients. CONCLUSIONS: Gamma knife surgery can effectively reduce the rebleeding rate after the first symptomatic hemorrhage in patients with CH. In addition, GKS may be useful in reducing the severity of seizures in patients with CH.

Contrast enhancement patterns of acute spinal epidural hematomas: a report of two cases.

The MR finding of an acute spinal epidural hematoma (SEH) can mimic epidural neoplastic or inflammatory lesions, because acute SEH appears hypointense on T1-weighted images and hyperintense on T2-weighted images. We report on two cases of acute SEH with unusual contrast enhancement patterns on MR images. Contrast enhancement can be an MR finding of acute SEH, especially in bleeding diathesis, which indicates an active lesion that needs early diagnosis and management.

Stereotactic radiosurgery for the treatment of dural arteriovenous fistulas involving the transverse-sigmoid sinus.

OBJECT: The aim of this study was to assess the efficacy and safety of radiosurgery for the treatment of dural arteriovenous fistulas (DAVFs) located in the region of the transverse-sigmoid sinus. METHODS: A series of 20 patients with DAVFs located in the transverse-sigmoid sinus, who were treated with gamma knife surgery between June 1995 and June 2000, was evaluated. According to the Cognard classification, the DAVF was Type I in four patients. Type IIa in seven, Type IIb in two, and combined Type IIa+b in seven. Nine patients had previously been treated with surgery and/or embolization, whereas 11 patients underwent radiosurgery alone. Radiosurgery was performed using multiple-isocenter irradiation of the delineated DAVF nidus. The target volume ranged from 1.7 to 40.7 cm3. The margin dose delivered to the nidus ranged from 16.5 to 19 Gy at a 50 to 70% isodose level. Nineteen patients were available for follow-up review, the duration of which ranged from 6 to 58 months (median 19 months). Of the 19 patients, 14 (74%) were cured of their symptoms. At follow up, magnetic resonance imaging and/or angiography demonstrated complete obliteration of the DAVF in 11 patients (58%), subtotal obliteration (95% reduction of the nidus) in three (16%), and partial obliteration in another five (26%). There was no neurological complication related to the treatment. One patient experienced a recurrence of the DAVF 18 months after angiographic confirmation of total obliteration, and underwent a second course of radiosurgery. CONCLUSIONS: Stereotactic radiosurgery provides a safe and effective option for the treatment of DAVFs involving the transverse and sigmoid sinuses. For some aggressive DAVFs with extensive retrograde cortical venous drainage, however, a combination of endovascular embolization and surgery may be necessary.

Intracranial dural arteriovenous fistulas: natural history and rationale for treatment with stereotactic radiosurgery.

Dural arteriovenous fistulas (DAVFs) are abnormal arteriovenous communications within the dura. The symptoms depend on their location and the pattern of the venous drainage. Patients with cavernous sinus DAVFs often present with ocular manifestations such as exophthalmos, chemosis and diplopia. Patients with transverse or sigmoid sinus DAVFs frequently experience headache and tinnitus on the affected side. DAVFs with anterograde sinus or cortical venous drainage (CVD) have been clinically regarded as benign, whereas DAVFs with retrograde CVD are considered aggressive in behavior. Similar to other cerebral arteriovenous malformations, DAVFs can hemorrhage, with an estimated annual risk of approximately 1.8%. The recommended therapeutic intervention for a DAVF is dependent on the anticipated natural history of the lesion. Management options include surgical resection, embolization and radiosurgery. Radiosurgical treatment has been used for DAVFs in various locations including the anterior cranial fossa, cavernous sinus, transverse/sigmoid sinus, superior sagittal sinus and tentorium. We present an update on 321 DAVF patients treated at the Taipei Veterans General Hospital using Gamma Knife radiosurgery. The prescribed mean margin dose was 17.2 Gy. In our series, 98% of patients had a stable or improved clinical condition after radiosurgery. Stereotactic radiosurgery using the Gamma Knife is a safe and effective alternative for the treatment of DAVFs.

Exclusion of histiocytes/endothelial cells and using endothelial cells as internal reference are crucial for interpretation of MGMT immunohistochemistry in glioblastoma.

We evaluated the predictive value of O6-methylguanine-DNA methyltransferase (MGMT) protein expression and MGMT promoter methylation status in glioblastomas (GBM) treated with temozolomide (TMZ) in a Taiwan medical center. Protein expression by immunohistochemical analysis (IHC) and MGMT promoter methylation detected by methylation-specific polymerase chain reaction (MSP) were performed in a series of 107 newly diagnosed GBMs. We used endothelial cells as an internal reference for IHC staining because the staining intensities of the MGMT-expressing cells in different specimens varied considerably; a positive result was defined as the staining intensity of the majority of tumor cells similar to that of the adjacent endothelial cells. Immunostainings for microglial/endothelial markers were included as part of the MGMT IHC evaluation, and in cases that were difficult to interpret, double-labeling helped to clarify the nature of reactive cells. The MGMT protein expression was reversely associated with MGMT promoter methylation status in 83.7% of cases (MSP/IHC and MSP/IHC; Pearson r=-0.644, P<0.001). Twenty-two of 24 (91.7%) IHC tumors did not respond to TMZ treatment. Combining MSP and IHC results, all the 15 MSP/IHC GBMs were TMZ resistant. The MGMT status detected by either IHC or MSP was significantly correlated with the TMZ treatment response (both P<0.001) and survival of GBM patients (both P<0.05).

Prenatal diagnosis of schizencephaly with septo-optic dysplasia by ultrasound and magnetic resonance imaging.

A 28-year-old woman, gravida 1, para 0, was referred to our fetal diagnosis unit at 28(+2) gestational weeks because no fetal movements were detected. On 2-D ultrasonography, the cephalic axial view showed multiple hypoechoic spaces in the fetal brain, both cerebral cortex and occipital lobe showed bilateral defects, and the septum pellucidum was absent. Multiple irregularly shaped cystic lesions connected with subarachnoid spaces were observed by three-dimensional ultrasonography in surface rendering mode. Septo-optic dysplasia with dysgenesis of corpus callosum was confirmed by prenatal magnetic resonance imaging (MRI). The fetus was complicated with cleft schizencephaly involving bilateral frontal, parietal and occipital lobes, which can often lead to learning disability, epilepsy and cerebral palsy after birth. The flaccid mobility of all four extremities of the fetus, demonstrated prenatally by real-time ultrasound and functional MRI, forecast the risk of postnatal spastic quadriplegia.

Primary pediatric brain tumors: statistics of Taipei VGH, Taiwan (1975-2004).

BACKGROUND: The purpose of the current study was to investigate a hospital series of 986 cases of primary pediatric brain tumors in Taiwan. METHODS: The authors reviewed the database of primary pediatric brain tumors in patients < 18 years of age collected in Taipei Veterans General Hospital (Taipei VGH) from 1975 to May 2004. Age and gender distribution, location, and classification of brain tumors were analyzed. Intracranial tumors with diagnostic imaging were included. Nontumoral lesions, cysts, and vascular malformations were excluded. RESULTS: The mean age of these 986 patients was 7.8 years, and the male to female ratio was 1.4:1. Supratentorial (including pineal-quadrigeminal) located tumors (58.3%) was predominant to infratentorial tumors (41.1%). In these series, 886 patients had either histologic diagnosis (842 patients) or clinical diagnosis (44 patients). The most common 5 categories of tumors were astrocytic tumors (31.1%), germ cell tumors (14.0%), medulloblastomas (13.3%), craniopharyngiomas (8.3%), and ependymal tumors (5.8%). Atypical teratoid/rhabdoid tumors (AT/RTs), a rare but highly malignant tumor, were 2.1%. The high incidence of primary intracranial germ cell tumors correlated with reported series from Japan and Korea. For the remaining 100 patients without diagnostic classifications, the majority were most likely astrocytic tumors in brain stem. CONCLUSIONS: The authors analyzed a large hospital series of primary brain tumors in children. Both histologically verified and unverified tumors were recruited to avoid selective bias. Although it was not a study of a population-based brain tumor registry, it could still be representative of primary pediatric brain tumors in Taiwan.