Imaging-based stratification of adult gliomas prognosticates survival and correlates with the 2021 WHO classification.

BACKGROUND: Because of the lack of global accessibility, delay, and cost-effectiveness of genetic testing, there is a clinical need for an imaging-based stratification of gliomas that can prognosticate survival and correlate with the 2021-WHO classification. METHODS: In this retrospective study, adult primary glioma patients with pre-surgery/pre-treatment MRI brain images having T2, FLAIR, T1, T1 post-contrast, DWI sequences, and survival information were included in TCIA training-dataset (n = 275) and independent validation-dataset (n = 200). A flowchart for imaging-based stratification of adult gliomas(IBGS) was created in consensus by three authors to encompass all adult glioma types. Diagnostic features used were T2-FLAIR mismatch sign, central necrosis with peripheral enhancement, diffusion restriction, and continuous cortex sign. Roman numerals (I, II, and III) denote IBGS types. Two independent teams of three and two radiologists, blinded to genetic, histology, and survival information, manually read MRI into three types based on the flowchart. Overall survival-analysis was done using age-adjusted Cox-regression analysis, which provided both hazard-ratio (HR) and area-under-curve (AUC) for each stratification system(IBGS and 2021-WHO). The sensitivity and specificity of each IBSG type were analyzed with cross-table to identify the corresponding 2021-WHO genotype. RESULTS: Imaging-based stratification was statistically significant in predicting survival in both datasets with good inter-observer agreement (age-adjusted Cox-regression, AUC > 0.5, k > 0.6, p < 0.001). IBGS type-I, type-II, and type-III gliomas had good specificity in identifying IDHmut 1p19q-codel oligodendroglioma (training - 97%, validation - 85%); IDHmut 1p19q non-codel astrocytoma (training - 80%, validation - 85.9%); and IDHwt glioblastoma (training - 76.5%, validation- 87.3%) respectively (p-value < 0.01). CONCLUSIONS: Imaging-based stratification of adult diffuse gliomas predicted patient survival and correlated well with 2021-WHO glioma classification.

MRI features predict tumor grade in isocitrate dehydrogenase (IDH)-mutant astrocytoma and oligodendroglioma.

PURPOSE: Nearly all literature for predicting tumor grade in astrocytoma and oligodendroglioma pre-dates the molecular classification system. We investigated the association between contrast enhancement, ADC, and rCBV with tumor grade separately for IDH-mutant astrocytomas and molecularly-defined oligodendrogliomas. METHODS: For this retrospective study, 44 patients with IDH-mutant astrocytomas (WHO grades II, III, or IV) and 39 patients with oligodendrogliomas (IDH-mutant and 1p/19q codeleted) (WHO grade II or III) were enrolled. Two readers independently assessed preoperative MRI for contrast enhancement, ADC, and rCBV. Inter-reader agreement was calculated, and statistical associations between MRI metrics and WHO grade were determined per reader. RESULTS: For IDH-mutant astrocytomas, both readers found a stepwise positive association between contrast enhancement and WHO grade (Reader A: OR 7.79 [1.97, 30.80], p = 0.003; Reader B: OR 6.62 [1.70, 25.82], p = 0.006); both readers found that ADC was negatively associated with WHO grade (Reader A: OR 0.74 [0.61, 0.90], p = 0.002); Reader B: OR 0.80 [0.66, 0.96], p = 0.017), and both readers found that rCBV was positively associated with WHO grade (Reader A: OR 2.33 [1.35, 4.00], p = 0.002; Reader B: OR 2.13 [1.30, 3.57], p = 0.003). For oligodendrogliomas, both readers found a positive association between contrast enhancement and WHO grade (Reader A: OR 15.33 [2.56, 91.95], p = 0.003; Reader B: OR 20.00 [2.19, 182.45], p = 0.008), but neither reader found an association between ADC or rCBV and WHO grade. CONCLUSIONS: Contrast enhancement predicts WHO grade for IDH-mutant astrocytomas and oligodendrogliomas. ADC and rCBV predict WHO grade for IDH-mutant astrocytomas, but not for oligodendrogliomas.

Third-trimester in utero fetal brain diffusion tensor imaging fiber tractography: a prospective longitudinal characterization of normal white matter tract development.

BACKGROUND: White matter is responsible for inter-neuronal connections throughout the brain that are a driving force in cognitive development. Diffusion tensor imaging (DTI) fiber tractography has been used to evaluate white matter development in the fetal brain; however, longitudinal studies of DTI fiber tractography to assess white matter development in the third trimester are lacking. OBJECTIVE: To characterize in utero longitudinal changes in the fetal brain DTI fiber tracts of normal third-trimester fetuses. MATERIALS AND METHODS: For this single-center prospective longitudinal observational pilot study, we recruited 28 pregnant females with normal third-trimester pregnancies who had routine prenatal ultrasound. MRI of the in utero fetal brain was performed with a Siemens 1.5-tesla (T) Espree scanner at 31 weeks, 33 weeks and 36 weeks of gestation, with 14 DTI tractography parameters quantified in 7 brain regions using DTI-studio version 2.4 (Johns Hopkins University, Baltimore, MD; n=98 measurements). We used multilevel mixed models to examine the relationship between longitudinal changes in DTI measurements and between 98 DTI measurements at 31 weeks and 4 routine fetal brain anatomical biometrics (n=392 assessments). RESULTS: We observed statistically significant decreases in radial diffusivity and apparent diffusion coefficient in 13 of 14 brain regions from 31 weeks to 36 weeks of gestation (P<0.001 for all regions except the genu of the corpus callosum). Significant decreases in radial diffusivity from weeks 33 to 36 and weeks 31 to 36 were seen in the corticospinal tracts, centrum semiovale, posterior limb of the internal capsule, and crus cerebri (P<0.001 for all). When considering all possible combinations of DTI fiber tract measurements and the routine morphological fetal brain biometrics, only 6% (24/392) had a significant association (P<0.05), indicating relative independence of the DTI fiber tract measurements from anatomical biometrics. CONCLUSION: In utero longitudinal changes in fetal brain DTI fiber tractography are quantifiable in normal third-trimester fetuses and are largely independent of morphological brain changes.

Magnetic Resonance Perfusion Changes of Arteriovenous Malformations Treated with Stereotactic Radiosurgery.

BACKGROUND: The latency period from stereotactic radiosurgery (SRS) to obliteration of arteriovenous malformations (AVM) requires continuous imaging surveillance. Magnetic resonance (MR) perfusion is promising for noninvasive monitoring of AVMs after SRS. We studied longitudinal MR perfusion changes of brain AVMs treated with SRS. METHODS: Consecutive patients treated for brain AVMs using SRS who had MR perfusion imaging studies performed before and at least once after SRS were studied. We estimated ipsilateral/contralateral brain hemisphere ratios of MR perfusion indexes, including regional cerebral blood flow (rCBF) and relative cerebral blood volume (rCBV), in the AVM nidus, perinidal region, and remote anterior and posterior brain regions. RESULTS: Eleven patients (6 women; median age, 21 years) underwent SRS (median prescription dose, 18 Gy; range, 12-20 Gy) for brain AVMs (median Spetzler-Martin grade 2 and median volume 4.6 mL). Before the SRS, rCBV and rCBF ratios were significantly higher in the AVM nidus compared with other investigated brain regions (P < 0.001). Median time from SRS to the first and last post-SRS MR perfusion studies was 8 and 35 months, respectively. There was a statically significant decrease of rCBV (P = 0.043) and rCBF (P = 0.036) ratios in the AVM nidus, but not other brain regions, during post-SRS follow-up. CONCLUSIONS: There is a gradual decrease of rCBV and rCBF in the AVM nidus after SRS. MR perfusion imaging is promising for monitoring of hemodynamic changes of AVMs after SRS. Larger studies investigating clinical value of MR perfusion imaging for AVMs after SRS are warranted.

Atypical Teratoid Rhabdoid Tumor of the Cauda Equina in a Child: Report of a Very Unusual Case.

Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive malignant primitive neoplasms that commonly occur in children younger than 2 years of age. The prognosis is generally dismal with a median survival time of <1 year. The majority of AT/RT occur in the posterior fossa and less frequently the supratentorium. Primary pediatric spinal AT/RT are exceedingly rare and only 15 cases have been reported to date. Here we report a very unusual case of primary spinal AT/RT extensively involving the spinal cord from T11 down to the cauda equina. In this patient, the tumor was highly aggressive and resulted in extensive dissemination into the nerve roots and paraspinal soft tissue rapidly resulting in the patient's death 1 month after diagnosis. to the best of our knowledge, this degree of involvement of the spine by a primary AT/RT has not been described before.

Alternative to General Anesthesia for a Stat Cesarean Delivery in a Patient with a Large Arteriovenous Malformation Involving the Cervicomedullary Junction in Active Labor.

A 20-year-old G1P0 patient at 38 weeks and 1 day of gestation was admitted for emergency cesarean delivery. Her past medical history was positive for cervicomedullary arteriovenous malformation (AVM) that ruptured three years before. Spontaneous vaginal delivery was contraindicated by neurosurgery. Aiming for cardiovascular stability and immediate reduction of sympathetic activity, a combined spinal epidural was successfully placed. An uneventful cesarean section was performed. The patient was transferred to the intensive care unit neurologically intact and discharged home after 8 days. This report describes an unusual anesthetic management of a patient with a large AVM in active labor.

Atrial Fibrillation, Brain Volumes, and Subclinical Cerebrovascular Disease (from the Atherosclerosis Risk in Communities Neurocognitive Study [ARIC-NCS]).

The aim of the present study was to investigate the association between atrial fibrillation (AF) and total and regional brain volumes among participants in the community-based Atherosclerosis Risk in Communities Neurocognitive study (ARIC-NCS). A total of 1,930 participants (130 with AF) with a mean age of 76.3 ± 5.2, who underwent 3T brain MRI scans in 2011 to 2013 were included. Prevalent AF was ascertained from study ECGs and hospital discharge codes. Brain volumes were measured using FreeSurfer image analysis software. Markers of subclinical cerebrovascular disease included lobar microhemorrhages, subcortical microhemorrhages, cortical infarcts, subcortical infarcts, lacunar infarcts, and volume of white matter hyperintensities. Linear regression models were used to assess the associations between AF status and brain volumes. In adjusted analyses, AF was not associated with markers of subclinical cerebrovascular disease. However, AF was associated with smaller regional brain volumes (including temporal, occipital, and parietal lobes; deep gray matter; Alzheimer disease signature region; and hippocampus [all p <0.05]) after controlling for demographics, cardiovascular risk factors, prevalent cardiovascular disease, and markers of subclinical cerebrovascular disease. Subgroup analysis revealed a significant interaction between AF and total brain volume with respect to age (p = 0.02), with associations between AF and smaller brain volumes being stronger for older individuals. In conclusion, AF was associated with smaller brain volumes, and the association was stronger among older individuals. This finding may be related to the longer exposure period of the older population to AF or the possibility that older people are more susceptible to the effects of AF on brain volume.

Carotid Stent Fracture from Stylocarotid Syndrome.

Eagle syndrome is a rare condition resulting from elongation of the temporal styloid process or calcification of its associated stylohyoid ligaments. Although usually presenting with pain or odynophagia, Eagle syndrome has been reported to cause a multitude of neurologic symptoms or vascular complications, some of which can be life-threatening. We present a case in which an endovascularly placed internal carotid artery stent in close proximity to a calcified stylohyoid ligament resulted in stent fracture with subsequent stent and vessel occlusion. We review and discuss the presentation, diagnosis, etiology, complications and treatment options of the syndrome.

An unusual cause of acute headache: subarachnoid free air secondary to spontaneous bronchopleurodurosubarachnoid fistula from a Pancoast tumor.

Pneumocephalus and pneumorrhachis are related to transgression of the barriers to the central nervous system. We present a patient with a Pancoast tumor treated with palliative chemoradiation who developed symptomatic spinal and intracranial air caused by spontaneous bronchopleurodurosubarachnoid fistula secondary to direct tumor invasion into the thecal sac.

Nasopharyngeal dendritic cell sarcoma, not otherwise specified, in a 34-year-old man.

Dendritic cell sarcoma, not otherwise specified (NOS), is an entity that is poorly defined because of its rarity and poorly understood pathogenesis. It is characterized by positive immunohistochemical staining for S-100 and CD1a, along with an absence of cytoplasmic Birbeck granules on electron microscopy. We report the case of a surgically inaccessible nasopharyngeal dendritic cell sarcoma, NOS, in a 34-year-old man. Treatment with chemotherapy along with adjuvant radiation therapy was successful in decreasing the size of the nasopharyngeal mass, and the patient remained free of any evidence of recurrence nearly 5 years after treatment.