Promising response to vemurafenib and cobimetinib treatment for BRAF V600E mutated craniopharyngioma: a case report and literature review.

Craniopharyngiomas are tumors that arise from the remnants of Rathke's pouch along the nasopharynx to the diencephalon. Current standard of care includes maximal surgical resection versus adjuvant radiation if a maximal resection is unfeasible. Pharmacological therapy with MAPK targeted agents is an emerging therapeutic option for tumors with BRAF V600E mutations. We report a 45-year-old male with a strictly third ventricle papillary craniopharyngioma with a BRAF V600E mutation. After initial surgery with subtotal resection, the patient demonstrated durable response to targeted BRAF and MEK inhibitor therapy with vemurafenib and cobimetinib. Our report suggests that targeted therapy may reduce the need for radiation and impact surgical interventions in select cases.

H3G34-mutant diffuse hemispheric glioma with osseous metastases: a case report and literature review.

Aim: H3G34 diffuse hemispheric glioma is a CNS tumor that is difficult to diagnose and treat and accompanied with poor prognosis. It is becoming clear that extra CNS metastasis may present in a subset of patients with H3G34 gliomas, further complicating diagnosis and treatment. Materials & methods: We present a case of a 19-year-old female with a H3G34 mutant diffuse hemispheric glioma with osseous metastases. We then provide a literature review of the most recent understanding of H3G34 mutant malignancies. Conclusion: Given the stress that patients with H3G34 can experience and the poor prognosis, it is imperative to expand our knowledge and ascertain accurate diagnostic methodologies and targeted therapeutic approaches.

Safety and diagnostic efficacy of gadoteridol for magnetic resonance imaging of the brain and spine in children 2 years of age and younger.

BACKGROUND: Neonates and young children require efficacious magnetic resonance imaging (MRI) examinations but are potentially more susceptible to the short- and long-term adverse effects of gadolinium-based contrast agents due to the immaturity of their body functions. OBJECTIVE: To evaluate the acute safety and diagnostic efficacy of gadoteridol (ProHance) for contrast-enhanced MRI of the central nervous system (CNS) in children ≤2 years of age. MATERIALS AND METHODS: One hundred twenty-five children ≤2 years old (including 57 children <6 months old) who underwent contrast-enhanced MRI of the CNS with gadoteridol at 0.1 mmol/kg body weight were retrospectively enrolled at five imaging centers. Safety data were assessed for acute/subacute adverse events in the 48 h following gadoteridol administration and, when available, vital signs, electrocardiogram (ECG) and clinical laboratory values obtained from blood samples taken from 48 h before until 48 h following the MRI exam. The efficacy of gadoteridol-enhanced MRI compared to unenhanced MRI for disease diagnosis was evaluated prospectively by three blinded, unaffiliated readers. RESULTS: Thirteen changes of laboratory values (11 mild, 1 moderate, 1 unspecified) were reported as adverse events in 7 (5.6%) patients. A relationship to gadoteridol was deemed possible though doubtful for two of these adverse events in two patients (1.6%). There were no clinical adverse events, no serious adverse events and no clinically meaningful changes in vital signs or ECG recordings. Accurate differentiation of tumor from non-neoplastic disease, and exact matching of specific MRI-determined diagnoses with on-site final diagnoses, was achieved in significantly more patients by each reader following the evaluation of combined pre- and post-contrast images compared to pre-contrast images alone (84.6-88.0% vs. 70.9-76.9%; P≤0.006 and 67.5-79.5% vs. 47.0-66.7%; P≤0.011, respectively). CONCLUSION: Gadoteridol at 0.1 mmol/kg body weight is safe, well tolerated and effective for contrast-enhanced MRI of the CNS in children ≤2 years of age.

Bilateral external auditory canal masses following repair of ruptured abdominal aortic aneurysm and open decompressive exploratory laparotomy for compartment syndrome: A rare case of spontaneous bilateral otorrhagia.

Very few cases of spontaneous otorrhagia (SO) following nonotolaryngologic surgery have ever been reported in surgical literature and none in radiographic. Of the surgical cases reported, SO occurred in the perioperative period following laparoscopic surgeries in the Trendelenburg position. We report the first case of spontaneous bilateral otorrhagia which presented as bilateral external auditory canal masses following endovascular surgery and open decompressive laparotomy in a 60-year-old male with a prior history of hypertension and smoking. We seek to inform radiologists that SO can present on neck imaging as external auditory canal masses as a complication of nonotolaryngologic surgery away from the imaged field of view.

A Comprehensive Review of Cross-Sectional Imaging of the Nasolacrimal Drainage Apparatus: What Radiologists Need to Know.

OBJECTIVE. The purpose of this study is to provide a comprehensive review of the radiographic anatomy and cross-sectional imaging findings of the full gamut of nasolacrimal drainage apparatus diseases, highlighting imaging findings from the different nasolacrimal drainage apparatus surgeries, posttreatment complications, and potential imaging pitfalls. CONCLUSION. Radiologists play a critical role in guiding the management of nasolacrimal drainage apparatus diseases and should be familiar with the anatomy and characteristic imaging findings of commonly encountered nasolacrimal drainage apparatus abnormalities and surgeries.

Basilar Apex Aneurysms in the Setting of Carotid Artery Stenosis: Case Series and Angiographic Anatomic Study.

BACKGROUND: Intracranial aneurysms (IAs) are life-threatening lesions known within the literature to be found incidentally during routine angiographic workup for carotid artery stenosis (CAS). As IAs are associated with vascular shear stress, it is reasonable to expect that altered flow demands within the anterior circulation, such as with CAS, increase compensatory flow demands via the Circle of Willis (COW) and may induce similar stress at the basilar apex. OBJECTIVE: We present a series of nine unruptured basilar apex aneurysms (BAA) with CAS and a comparative radiographic analysis to BAA without CAS. METHODS: Twenty-three patients with BAA were retrospectively identified using records from 2011 to 2016. CAS by North American Symptomatic Carotid Endarterectomy Trial (NASCET) criteria, morphology of BAA, competency of COW, and anatomic relationships within the posterior circulation were examined independently by a neuroradiologist using angiographic imaging. RESULTS: Nine (39%) of the twenty-three BAA patients had CAS, with six having stenosis ≥50%. Four (67%) of the patients with ≥50% CAS demonstrated aneurysm flow angles contralateral to the side with highest CAS. Additionally, the angle between the basilar artery (BA) trajectory and aneurysm neck was observed to be smaller in patients with ≥50% CAS (61 vs 74 degrees). No significant differences in COW patency, posterior circulation morphology, and degree of stenosis were observed. CONCLUSION: Changes in the cervical carotid arteries may lead to blood flow alterations in the posterior circulation that increase the propensity for BAA formation. Posterior circulation imaging can be considered in CAS patients to screen for BAA.

OUTER RETINAL LAYER THICKNESS AS GOOD VISUAL PREDICTOR IN PATIENTS WITH DIABETIC MACULAR EDEMA.

PURPOSE: To evaluate the relationship between outer retinal layer (ORL) thickness in diabetic macular edema to visual acuity (VA). METHODS: Ninety-five eyes were included in this prospective nonrandomized case-control study. They divided into 30 cases of healthy normal subjects and 65 cases of nonproliferative diabetic retinopathy with diabetic macular edema. Complete ophthalmic examination and spectral domain-optical coherence tomography were done for all cases and analyzed to measure central foveal point thickness (CFT), ORL thickness at fovea, and subfoveal choroidal thickness. RESULTS: The ORL thickness was significantly thinner in diabetic macular edema group (85.3 µm) than controls (99.9 µm) (P = 0.002). There was higher significant correlation between ORL thickness and logMAR VA (r = -0.87, P < 0.001) than correlation between CFT and VA (r = 0.18, P = 0.16). The cutoff point of the relationship between ORL thickness and VA was 88 µm with moderate sensitivity (81%) and high specificity (88%), below which vision is affected. Outer retinal layer thickness is significantly related to subfoveal choroidal thickness but not CFT. CONCLUSION: This study revealed reduction in ORL thickness in diabetic macular edema. There is a higher and stronger correlation between ORL thickness and vision than that between CFT and vision.

Case 227: Endobronchial Carcinoid Tumor with Incidental Metastatic Breast Cancer Detected with Somatostatin Receptor Scintigraphy ((111)In Pentreotide).

HISTORY: A 30-year-old woman with polycystic ovarian syndrome who was undergoing hormone replacement therapy presented with a 6-month history of a nonproductive cough and a 1-day history of hemoptysis (approximately 20 mL). Intravenous contrast material-enhanced (100 mL of Omnipaque 350; GE Healthcare, Princeton, NJ) computed tomographic (CT) pulmonary angiography was performed to evaluate for pulmonary embolism. On the basis of the CT pulmonary angiographic findings, chromogranin A and 5-hydroxyindoleacetic acid levels were measured and were 7 nmol/L (343 µg/L) (high) and 2.9 mg per 24 hours (15.167 µmol/d) (normal), respectively. This patient underwent bronchoscopy and biopsy. After these tests, she was referred for whole-body scintigraphy, which revealed an unexpected finding that was further investigated with fluorine 18 ((18)F) flurodeoxyglucose (FDG) positron emission tomography (PET) and CT.

Image findings of cranial nerve pathology on [18F]-2- deoxy-D-glucose (FDG) positron emission tomography with computerized tomography (PET/CT): a pictorial essay.

This article aims to increase awareness about the utility of (18)F -FDG-PET/CT in the evaluation of cranial nerve (CN) pathology. We discuss the clinical implication of detecting perineural tumor spread, emphasize the primary and secondary (18)F -FDG-PET/CT findings of CN pathology, and illustrate the individual (18)F -FDG-PET/CT CN anatomy and pathology of 11 of the 12 CNs.

Rosai-Dorfman disease in neuroradiology: imaging findings in a series of 10 patients.

OBJECTIVE: Rosai-Dorfman disease is a rare disorder characterized histologically by lymphatic sinus dilatation due to histiocyte proliferation. Our goal was to describe the CT, MRI, and (18)F-FDG (FDG) PET findings in a series of patients with this diagnosis. MATERIALS AND METHODS: We retrospectively reviewed the imaging studies of 10 patients with pathologically confirmed Rosai-Dorfman disease who were treated in our institution between January 2004 and December 2007. RESULTS: We found the following areas of general involvement: three intracranial, seven head and neck, and three spinal, with some patients having more than one site. Specific sites of involvement included the following: intracranial meninges, n = 2; pituitary, n = 2; lacrimal gland, n = 1; paranasal sinus, n = 3; neck lymph nodes, n = 6; salivary gland, n = 3; tonsil, n = 1; skin, n = 1; spinal meninges, n = 2; vertebral body, n = 1; and thymus, n = 1. The MRI characteristics of the involved areas were generally T1 isointense, T2 isointense, diffusion isointense to gray matter, and intensely enhancing with gadolinium chelate contrast agents. CT images generally showed the lesions were hyperdense to gray matter and intensely enhancing. FDG PET showed variable uptake, with nodal and lacrimal disease generally being FDG avid and other sites not. CONCLUSION: Rosai-Dorfman disease has a protean imaging appearance but most frequently presents as neck lymphadenopathy. The disease is frequently multifocal, and a diagnosis in one area should prompt suspicion that other sites may be involved also.

Significant coronary calcification detected using contrast-enhanced computed tomography: is it an indication for further investigation?

BACKGROUND: Evaluate the significance of coronary arteries calcifications detected using contrast-enhanced chest computed tomography (CECCT) scans. METHODS: A total of 145 patients who underwent both CECCT and gated stress myocardial perfusion imaging (MPI) within 2 weeks were included. The chest CT scans were reviewed for the presence of coronary artery calcium (CAC) by 2 experienced blinded readers. The degree of calcifications seen in any visualized area of the major coronary arteries was graded on a scale of 1 to 3 (1 when 0 to 4 small scattered plaques were seen, 2 when 5 or more scattered plaques were seen, and 3 when diffuse contiguous calcification were seen). The grade of CAC was correlated with the presence of MPI abnormalities. Additionally, the locations of the MPI abnormality was correlated with the CAC grade in the culprit coronary artery. RESULTS: Of 580 major coronary arteries evaluated, 79% had grade 1 CAC, 10% had grade 2, 11% had grade 3, and 1% were inevaluable. Of the 145 patients, 33 (23%) had abnormal MPI results. Twenty-three of the patients with abnormal MPI results (70%) had more than 4 calcified plaques (CAC grade of 2 or 3) in one or more of their coronary arteries, whereas 41 of 122 patients with normal MPI results (37%) had similar CAC grades (P=0.001). Seventeen of the 33 patients (52%) who had MPI defects also had significant CAC in the culprit coronary artery, 7 patients (21%) had significant CAC in a different coronary artery from the MPI defect territory, 8 patients (24%) had no significant CAC visualized, and in 1 patient a pacemaker wire interfered with CAC grading in the culprit coronary artery (RCA). The sensitivity, specificity, the positive, and negative predictive values of grade 2 or 3 CAC for an abnormal MPI results were 70%, 63%, 36%, and 88%, respectively. When a subgroup of patients above 60 years old with grade 3 CAC was reanalyzed, the sensitivity, specificity, the positive, and negative predictive values for an abnormal MPI results were 69%, 73%, 41%, and 90%, respectively. CONCLUSION: Multiple diffuse CAC as detected during the interpretation of CECCT scans in combination with advanced age is a significant finding that warrants further investigation for functionally significant CAD.