Bevacizumab in the Treatment of Refractory Brain Edema in High-grade Glioma.

We report the case of a 14-year-old boy with a steroid-dependent refractory tumor whose longstanding dexamethasone treatment was successfully discontinued after a course of bevacizumab. The use of bevacizumab despite the absence of clear evidence of radionecrosis allowed a significant decrease in the amount of the brain edema.

Bilateral Parietal Skull Fractures in Infants Attributable to Accidental Falls.

INTRODUCTION: Multiple skull fractures, including bilateral parietal skull fractures (BPSFs) in infants are considered to be suspicious for abusive head trauma (AHT). The aim of this report is to describe a series of BPSF cases in infants which occurred due to accidental falls. METHODS: We searched our neuroradiology database for BPSF in infants (<1 year old) diagnosed between 2006 and 2019; we reviewed initial presentation, mechanisms of injury, clinical course, head imaging, skeletal survey X-rays, ophthalmology, social work and child abuse physicians (CAP) assessments, and long-term follow-up. "Confirmed accidental BPSF" were strictly defined as having negative skeletal survey and ophthalmology evaluation and a CAP conclusion of accidental injury. RESULTS: Twelve cases of BPSF were found; 3 were confirmed to be accidental, with a mean age at presentation of 3 months. Two infants had single-impact falls, and 1 had a compression injury; all 3 had small intracranial hemorrhages. None had bruises or other injuries, and all remained clinically well. A literature search found 10 similar cases and further biomechanical evidence that these fractures can occur from accidental falls. CONCLUSION: While AHT should be kept in the differential diagnosis whenever BPSFs are seen, these injuries can occur as a result of accidental falls.

New insights into the genomic landscape of meningiomas identified FGFR3 in a subset of patients with favorable prognoses.

Background: With a prevalence of 170 000 adults in the US alone, meningiomas are the most common primary intracranial tumors. The management of skull base meningiomas is challenging due to their complexity and proximity to crucial nearby structures. The identification of oncogenic mutations has provided further insights into the tumorigenesis of meningioma and the possibility of targeted therapy. This study aimed to further investigate the association of mutational profiles with anatomical distribution, histological subtype, WHO grade, and recurrence in patients with meningioma. Methods: Tissue samples were collected from 71 patients diagnosed with meningioma from 2008 to 2016. A total of 51 cases were skull based. Samples were subjected to targeted sequencing using a next generation customized cancer gene panel (n = 66 genes analyzed). Results: We detected genomic alterations (GAs) in 68 tumors, averaging 1.56 ± 1.07 genomic alterations (GAs) per sample. NF2 was the most frequently altered gene (36/71 cases). Interestingly, we identified a number of mutations in non-NF2 genes, including a hotspot TERTp c.-124: G > A mutation that may be related to poor prognosis and FGFR3 mutations that may represent biomarkers of a favorable prognosis as reported in other cancers. Conclusions: We demonstrate that comprehensive genomic profiling in our population can reveal a potential new prognostic biomarkers of skull base meningioma. These mutations can enhance diagnostic accuracy and clinical decision-making. Among our findings were the identification of a TERTp mutation and the first report of FGFR3 mutations that may represent biomarkers for the identification of skull base meningioma patients with a favorable prognosis.

Decompressive Cranioplasty in a Patient with Osteopetrosis.

BACKGROUND: Osteopetrosis is a heterogeneous group of uncommon congenital disorders that causes bony sclerosis and remodeling. Patients who are symptomatic can show significant neurologic consequences with the involvement of cranial nerves and symptoms of increased intracranial pressure (ICP). CASE DESCRIPTION: We report an unusual case of a 26-year-old woman with an autosomal-dominant type of osteopetrosis who presented with headache and severe visual deterioration, both attributed to increased ICP. A hemicranioplasty was preformed, resulting in the resolution of her symptoms of ICP and stabilization of her vision. Postoperative imaging showed expansion of the ventricles and the subarachnoid spaces with an improvement of the associated cerebellar herniation. CONCLUSIONS: In conclusion, in patients with symptomatic osteopetrosis, cranioplasty can be considered as an option to treat high ICP-related symptoms.

Proposed clinical internal carotid artery classification system.

INTRODUCTION: Numerical classification systems for the internal carotid artery (ICA) are available, but modifications have added confusion to the numerical systems. Furthermore, previous classifications may not be applicable uniformly to microsurgical and endoscopic procedures. The purpose of this study was to develop a clinically useful classification system. MATERIALS AND METHODS: We performed cadaver dissections of the ICA in 5 heads (10 sides) and evaluated 648 internal carotid arteries with computed tomography angiography. We identified specific anatomic landmarks to define the beginning and end of each ICA segment. RESULTS: The ICA was classified into eight segments based on the cadaver and imaging findings: (1) Cervical segment; (2) cochlear segment (ascending segment of the ICA in the temporal bone) (relation of the start of this segment to the base of the styloid process: Above, 425 sides [80%]; below, 2 sides [0.4%]; at same level, 107 sides [20%]; P < 0.0001) (relation of cochlea to ICA: Posterior, 501 sides [85%]; posteromedial, 84 sides [14%]; P < 0.0001); (3) petrous segment (horizontal segment of ICA in the temporal bone) starting at the crossing of the eustachian tube superolateral to the ICA turn in all 10 samples; (4) Gasserian-Clival segment (ascending segment of ICA in the cavernous sinus) starting at the petrolingual ligament (PLL) (relation to vidian canal on imaging: At same level, 360 sides [63%]; below, 154 sides [27%]; above, 53 sides [9%]; P < 0.0001); in this segment, the ICA projected medially toward the clivus in 275 sides (52%) or parallel to the clivus with no deviation in 256 sides (48%; P < 0.0001); (5) sellar segment (medial loop of ICA in the cavernous sinus) starting at the takeoff of the meningeal hypophyseal trunk (ICA was medial into the sella in 271 cases [46%], lateral without touching the sella in 127 cases [23%], and abutting the sella in 182 cases [31%]; P < 0.0001); (6) sphenoid segment (lateral loop of ICA within the cavernous sinus) starting at the crossing of the fourth cranial nerve on the lateral aspect of the cavernous ICA and located directly lateral to the sphenoid sinus; (7) ring segment (ICA between the 2 dural rings) starting at the crossing of the third cranial nerve on the lateral aspect of the ICA; (8) cisternal segment starting at the distal dural ring. CONCLUSIONS: The classification may be applied uniformly to all skull base surgical approaches including lateral microsurgical and ventral endoscopic approaches, obviating the need for 2 separate classification systems. The classification allows extrapolation of relevant clinical information because each named segment may indicate potential surgical risk to specific structures.

Monosomal karyotype predicts adverse prognosis in patients diagnosed with chronic myelomonocytic leukemia: a single-institution experience.

INTRODUCTION: Cytogenetic abnormalities have a significant prognostic effect in many hematologic neoplasms. Monosomal karyotype (MK), a newly recognized cytogenetic category, has been reported to be a marker of poor prognosis in patients with myelodysplastic syndromes and myelofibrosis, but its effect in chronic myelomonocytic leukemia (CMML) remains relatively unknown. PATIENTS AND METHODS: A retrospective study of cases diagnosed with CMML found a total of 262 patients. Patient characteristics, cytogenetic data, and survival were analyzed. RESULTS: Cytogenetic analysis found diploidy in 167 patients (64%). Trisomy 8 was the most frequent cytogenetic abnormality at 8% (22), followed by complex karyotype (CK) at 5% (14), (-)7 at 4% (10), and MK at 3% (7, of which 6 [86%] were also CK). Median overall survival was statistically significantly worse in MK-positive cases than in MK-negative cases (MK(+) vs. MK(-)). Patients with MK(+) only or CK(+)MK(+) did worse than any other group. CONCLUSION: MK is a rare entity but can predict statistically significantly shorter overall survival among all other cytogenetic categories.