Carol P. Geer

Clinical, histopathologic, and radiographic indicators of malignancy in head and neck paragangliomas.

OBJECTIVE: The goal of this study is to review our series of head and neck paragangliomas to identify factors that may help in predicting malignancy. STUDY DESIGN: Case series with chart review. SETTING: Academic medical center. SUBJECTS AND METHODS: Subjects with head and neck paragangliomas at our institution from 1976 to current were reviewed. In addition to statistical comparisons of epidemiologic factors, pathologic and radiographic characteristics were reviewed. RESULTS: Of the 84 subjects, there were seven malignant paragangliomas (8%). Age was found to be significantly different between the benign and malignant subgroups, with an average age of 54 ± 16 and 40 ± 12 years, respectively (P = 0.02). Pain was a presenting complaint in five patients with benign disease (6%), and five of the seven malignant patients (71%) presented with pain, showing a significant association between pain and disease type (P <0.0001). The odds ratio for a patient with pain having a malignant tumor was 36 (95% CI: 5.5–234). Enlarging neck mass was noted in all cases of malignant disease, but only in 31 percent of cases of benign disease (P < 0.0001). In a secondary analysis of carotid body tumors alone, enlarging neck mass was not found to be significant between benign and malignant disease (P = 0.14). However, pain continued to be significantly different, with 67 percent of malignant lesions demonstrating pain, compared with only 11 percent of benign lesions (P = 0.01). CONCLUSION: This study suggests that pain, a rapidly enlarging neck mass, and younger age are predictive factors of underlying malignancy, which should prompt one to consider an aggressive diagnostic and management approach.

Social Media in Professional Medicine: New Resident Perceptions and Practices

Background For younger generations, unconstrained online social activity is the norm. Little data are available about perceptions among young medical practitioners who enter the professional clinical arena, while the impact of existing social media policy on these perceptions is unclear. Objective The objective of this study was to investigate the existing perceptions about social media and professionalism among new physicians entering in professional clinical practice; and to determine the effects of formal social media instruction and policy on young professionals’ ability to navigate case-based scenarios about online behavior in the context of professional medicine. Methods This was a prospective observational study involving the new resident physicians at a large academic medical center. Medical residents from 9 specialties were invited to participate and answer an anonymous questionnaire about social media in clinical medicine. Data were analyzed using SAS 9.4 (Cary, NC), chi-square or Fisher’s exact test was used as appropriate, and the correct responses were compared between different groups using the Kruskal–Wallis analysis of variance. Results Familiarity with current institutional policy was associated with an average of 2.2 more correct responses (P=.01). Instruction on social media use during medical school was related to correct responses for 2 additional questions (P=.03). On dividing the groups into no policy exposure, single policy exposure, or both exposures, the mean differences were found to be statistically significant (3.5, 7.5, and 9.4, respectively) (P=.03). Conclusions In this study, a number of young physicians demonstrated a casual approach to social media activity in the context of professional medical practice. Several areas of potential educational opportunity and focus were identified: (1) online privacy, (2) maintaining digital professionalism, (3) safeguarding the protected health information of patients, and (4) the impact of existing social media policies. Prior social media instruction and/or familiarity with a social media policy are associated with an improved performance on case-based questions regarding online professionalism. This suggests a correlation between an instruction about online professionalism and more cautious online behavior. Improving the content and delivery of social media policy may assist in preserving institutional priorities, protecting patient information, and safeguarding young professionals from online misadventure.

A unique presentation of a spinal dural arteriovenous fistula exacerbated by steroids

Spinal dural arteriovenous fistula (DAVF) is an acquired vascular malformation of the spinal cord that presents as a congestive myelopathy resulting from venous hypertension, edema, and ischemia within the cord. Acute clinical exacerbations have been demonstrated in a variety of clinical settings. We report a unique presentation of a 45-year-old male with progressive paraplegia that acutely worsened following three independent treatments with oral and intravenous steroid administration. Spinal angiogram revealed a spinal DAVF at L3 and the patient underwent successful surgical repair. This report highlights the clinical presentation of spinal DAVF and emphasizes the unique and important potential relationship between steroid administration and clinical deterioration.

Case report: atypical presentation of jugular foramen mass

INTRODUCTION Jugular foramen lesions are often associated with pathology of adjacent structures due to either compression or direct invasion. Common presenting symptoms include pulsatile tinnitus, a neck mass, hearing loss, and cranial nerve palsies, leading to changes in taste, vocal cord paralysis, dysphagia, and sternocleidomastoid/trapezius weakness (A. Hakuba, K. Hashi, K. Fujitani, et al., Jugular foramen neurinomas. Surg Neurol 1979; 11:83-94). This patient was found to have a jugular foramen mass after presenting with the unusual constellation of visual changes and headache. CASE PRESENTATION A jugular foramen mass in a young woman was discovered after presenting with visual changes and headache; the patient was found to have papilledema on initial examination. Otologic and head and neck examination were normal. Subsequent imaging demonstrated a mass at the right jugular foramen with compression of this structure; a contralateral transverse sinus stenosis was also seen. This latter abnormality (along with obstruction of the jugular foramen) impeded venous drainage leading to papilledema and visual changes. DISCUSSION In a patient presenting with papilledema and severe headache with an associated jugular foramen mass, a multidisciplinary approach benefits the patient with input from interventional neuroradiology, neurosurgery, and neuro-ophthalmology. Venous outflow was compromised through the left stenotic transverse sinus, and the normal outflow on the right side through the jugular bulb was impeded by the tumor; obstructions of both led to symptomatic impeded venous outflow. This compromise in venous outflow led to an increase in superior sagittal sinus pressure, with subsequent increase in intracranial pressure and resultant papilledema. In an attempt to increase blood flow, an angioplasty was performed on the patients affected transverse sinus. In addition, symptomatology consistent with pseudotumor cerebri prompted the use of acetazolamide for medical management. After both therapies, the patients symptoms dramatically improved and were stable. The tumor has also remained stable, with no immediate need for surgical resection, stereotactic radiation, or consideration of an intraluminal transverse sinus stent placement or shunting. CONCLUSION The unique presentation of a jugular foramen mass in a young woman leading to papilledema highlights the need for high clinical suspicion of potential etiologies necessary for diagnosis. Despite the benign nature of her disease process, an unusual constellation of anatomical factors lead to the need for acute intervention.

Longitudinal Evaluation of Neurobehavioral Outcomes After Carotid Revascularization

BACKGROUND Carotid revascularization, including carotid endarterectomy (CEA) and carotid angioplasty and stenting (CAS), is performed for stroke risk reduction but may also impact cognitive function. Cognitive outcomes observed after carotid revascularization have been inconsistent, and mechanistic relationships with procedural factors are poorly understood. To further explore associations between carotid revascularization and cognitive outcomes, a prospective longitudinal evaluation was conducted of patients undergoing elective CEA or CAS for hemodynamically significant carotid stenosis. METHODS Patients undergoing primary carotid artery revascularization for hemodynamically significant stenosis were evaluated with neurologic and neuropsychological testing at baseline and at 1 and 6 months after revascularization. A subgroup of patients was also studied with baseline and postoperative magnetic resonance imaging (MRI). Outcomes included neurologic or neuropsychological deficits and imaging findings (including quantitative assessment of cerebral blood flow). RESULTS Sixteen patients underwent carotid revascularization with both preoperative and postoperative neurologic and neuropsychological testing; preoperative and postoperative MRIs were also performed on eight patients. Five of 16 treated carotid lesions (31%) were considered symptomatic, and severity of carotid stenosis was 60-79% for 6 of 16 lesions and 80% or more in all others. A single perioperative neurologic deficit was identified; all other patients (15/16) had no abnormalities detected by neurologic examination. Neuropsychological testing identified new postoperative deficits in 3 patients (19%), among whom 2 had a normal neurologic examination at all time points, whereas 1 had clinical evidence of stroke. Quantitative analysis of mean cerebral blood flow revealed postrevascularization increases for both gray matter (48.6 ± 13.9 mL per 100 g/min vs 75.3 ± 70.8 mL per 100 g/min) and white matter (31.8 ± 10.6 mL per 100 g/min vs 55.2 ± 30.1 mL per 100 g/min)(P = 0.04). New postoperative MRI foci of restricted diffusion were identified in 2 patients, both of whom had no neurologic or neuropsychological deficit. Among patients with postoperative neuropsychological deficits, MRI revealed globally increased cerebral perfusion without new postoperative abnormalities in 2 of 3. CONCLUSIONS The relationship between carotid revascularization and cognitive function is complex, and cognitive deficits may occur in the presence of increased cerebral perfusion without detectable embolization.

Pearls & Oy-sters: The critical role of histopathology in diagnosing cancer-associated necrotizing CNS vasculitis

Objective To highlight the importance of a broad differential and histopathologic confirmation in patients with newly diagnosed cancer with brain lesions atypical for CNS metastasis. Methods We report 2 cases of biopsy-proven CNS vasculitis in patients undergoing treatment for a newly diagnosed nonmetastatic cancer. Comprehensive medical record review was performed to identify the clinical presentation, representative neuroimaging, histopathologic features, and response to treatment. Results Patient 1 presented 1 month into induction therapy of malignant vaginal squamous cell carcinoma (stage 3, T2N1M0) with acute episodic left-sided hemiparesis due to seizure activity progressing to severe encephalopathy. Imaging revealed a right frontoparietal lesion while systemic workup was unrevealing. Biopsy demonstrated necrotizing vasculitis. Patient 2 presented 6 months after diagnosis of right breast invasive ductal carcinoma (stage IIa, T2N0M0, estrogen receptor–positive, progesterone receptor–positive, human epidermal growth factor receptor–2 positive) with subacute bifrontal headaches with associated phonophobia. Imaging showed hyperintense lesions involving the right temporoparietal region and systemic workup was unrevealing. Brain biopsy showed a necrotizing vasculitis. Patient 1 was treated with methyprednisolone and plasmapheresis and patient 2 was treated with prednisone. Both patients showed complete resolution of symptoms shortly after treatment and improvement on imaging. Conclusions These cases highlight the importance of comprehensive evaluation of new brain lesions in patients with nonmetastatic solid tumors. Characteristics of new brain lesions in patients with cancer that should raise suspicion of diagnoses other than brain metastasis include (1) primary malignancy without regional or distant metastasis, (2) imaging without discrete mass-like enhancement, and (3) cortically based location of lesions not at the gray–white matter junction.

Radiation dose reduction during neuroendovascular procedures

Aim To describe the impact of steps towards reduction of procedural doses of radiation during neuroendovascular procedures. Methods Phantom exposures under controlled circumstances were performed using a Rando-Alderson adult-sized head phantom. Customized imaging protocols were devised for pediatric and adult imaging and implemented in clinical use. Outcome data for estimated skin doses (ESD) and dose–area product (DAP) following pediatric and adult diagnostic and interventional procedures over 4.5 years were analyzed retrospectively. Results Dose estimates were reduced by 50% or more after introduction of customized imaging protocols in association with modification of personnel behavior compared with doses recorded with previously used vendor-recommended protocols. Discussion Substantial reductions in radiation use during neuroendovascular procedures can be achieved through a combination of equipment modification and operator behavior.

Surgical Considerations in the Optimal Management of Patients with Malignant Brain Tumors

Opinion statementAdvances in technology are revolutionizing medicine and the limits of what we can offer to our patients. In neurosurgery, technology continues to reduce morbidity, increase surgical accuracy, facilitate tissue acquisition, and promote novel techniques for prolonging survival in patients with neuro-oncologic disease. Surgery has been the backbone of glioma diagnosis and treatment by providing adequate, high quality material for precise histologic diagnosis, and genomic characterization in the setting of significant intratumoral heterogeneity, thus allowing personalized treatment selection in the clinic. The ability to obtain and accurately measure the maximal extent of resection in glioma surgery also remains a central role of the neurosurgeon in managing this cancer. To meet these goals, today’s operating room has transformed from the traditional operating table and anesthesia machine to include neuronavigation instrumentation, intraoperative computed tomography, and magnetic resonance imaging scanners, advanced surgical microscopes fitted with fluorescent light filters, and electrocorticography machines. While surgeons, oncologists, and radiation oncologists all play unique critical roles in the care of patients with malignant gliomas, familiarity with developing techniques in complimentary subspecialties can enhance coordination of patient care, research productivity, professional interactions, and patient confidence and comfort with the physician team. Herein, we provide a summary of the advances in the field of neurosurgical oncology which allow more precise and optimal surgical resection for patients with malignant gliomas.