Faculty Bibliography

The BRAF-V600E mutation is found in 15-20% of pediatric low grade gliomas (PLGG) and result in worse outcome and higher risk of transformation to high grade gliomas (PHGG). Although ongoing trials are assessing the role of BRAF inhibitors (BRAFi) in these children, data are still limited. We aimed to report overall response rates and predictors of outcome in childhood BRAF-V600E gliomas. We collected clinical, imaging and molecular information of patients treated with BRAFi outside trials from centers participating in the PLGG taskforce. Response was calculated by RANO criteria and follow up data were collected for all patients. Sixty-six patients were treated with BRAFi (55 PLGG and 11 PHGG); median follow-up time was 1.5 years (0.1-5y). In PLGG, objective response (tumor reduction of >25%) was observed in 77% compared to 15% in a cohort treated with conventional chemotherapy (pCDKN2A deletion was not associated with lack of response, while specifc enhancing patterns correlated strongly with response to BRAFi. Two-year PFS for the BRAF-V600E PLGG was 74% vs 47% for BRAFi vs chemotherapy, respectively (p=0.02). Our data reveal rapid, dramatic and sustained response of BRAF-V600E PLGG to BRAFi. These are in contrast to BRAF-V600E PHGG and non-enhancing PLGG. Additional molecular analyses are being performed to identify poor responders and emerging mechanisms of resistance in these tumors

BACKGROUND:Awareness of disease status has been identified as a factor in the treatment decision-making process. Women with recurrent ovarian cancer are facing the challenge of making treatment decisions throughout the disease trajectory. It is not understood how women with ovarian cancer perceive their disease and subsequently make treatment decisions. PURPOSE/OBJECTIVE:The purpose of this phenomenological study was to understand the lived experience of women with recurrent ovarian cancer, how they understood their disease and made their treatment decisions. METHODS:A qualitative design with a descriptive phenomenological method was used to conduct 2 in-depth interviews with 12 women (n = 24 interviews). Each interview was ∼60 minutes and was digitally recorded and professionally transcribed. Data collection focused on patients' understanding of their disease and how patients participated in treatment decisions. A modified version of Colaizzi's method of phenomenological reduction guided data analysis. RESULTS:Three themes emerged to describe the phenomenon of being aware of disease status: (1) perceiving recurrent ovarian cancer as a chronic illness, (2) perceived inability to make treatment decisions, and (3) enduring emotional distress. CONCLUSIONS AND IMPLICATIONS/CONCLUSIONS:This study revealed how 12 women conceptualized recurrent ovarian cancer as a chronic disease and their perceived inability to make treatment decisions because of lack of information and professional qualifications, resulting in enduring emotional distress. Future research should replicate the study to confirm the persistence of the themes for racially, ethnically, and religiously diverse patient samples and to improve understanding of awareness of disease status and decision-making processes of patients.

Percutaneous osseointegrated bone conduction auditory devices provide excellent auditory rehabilitation. Device-related complications relate to skin abutment interface and cosmetic concerns, resulting in the development of transcutaneous devices. The Sophono and Baha Attract are safe and considered cosmetically superior to the percutaneous Baha Connect and Ponto. They provide excellent auditory enhancement; however, owing to indirect connectivity between processor and implant, there is on average 5- to 7-db less gain when compared with percutaneous bone-anchored implants. Surgical implantation of either device is usually performed under monitored sedation, in an ambulatory setting, with less than a 1-hour operative time, and minimal complications.

A new category of hearing technology has emerged that comprises devices inserted deep into the ear canal. Although not implanted, they represent an extension of what is expected of a traditional hearing aid. There are advantages to these devices, but they are not suited for all individuals with hearing loss. This category consists of 2 devices currently available in the United States: Lyric (Phonak AG, Stafa, Switzerland) and Earlens (Earlens, Menlo Park, CA, USA).

OBJECTIVES/OBJECTIVE:Adequate treatment of laryngopharyngeal malignancy often incorporates radiation therapy. Structures surrounding laryngopharynx exposed to traditional radiation doses are susceptible to posttreatment toxicity. Among poorly understood sequelae is the rare manifestation of sternoclavicular joint (SCJ) osteoradionecrosis (ORN). METHODS:Three institutional encounters prompted a comprehensive literature search, generating three published case reports. Systematic extraction and analysis (n = 6) of demographics, cancer history, comorbidities, ORN presentation, imaging, and management established the largest series to investigate this pathology. RESULTS:Patients were males (6), 54 to 70 years old, smokers (4), with Hypertension/dyslipidemia, myocardial infarction/coronary artery disease, second primary (2), diabetes mellitus (1), and myelofibrosis(1). Four underwent total laryngectomy, one primary, three as salvage. Five patients had concurrent chemoradiation (≥70 Gy). All patients presented with swollen, tender neck wounds concerning for persistent/recurrent malignancy. Computed tomography (CT) demonstrated bone erosion (5 of 5) and increased bone scan uptake (2 of 2). All responded to surgical exploration with drainage alone (1), sequestrectomy (2), or bone resection with synovectomy (3). Complete healing took 2 months to 3 years. One unrelated patient death occurred before control of ORN was achieved. DISCUSSION/CONCLUSIONS:Given varied patient characteristics, synergistic risk factors exist that alter bone radiation threshold, resulting in irreversible ischemic damage and osteoradionecrosis. Vascular susceptibility and inability to repair may regulate that threshold. Understanding this relationship will facilitate early detection and intervention. CONCLUSION/CONCLUSIONS:Integrating cases of sternoclavicular joint ORN promotes awareness of atypical laryngopharyngeal radiation complications, elucidates contributing factors, educates physicians on presentation and management, and provides a platform for prospective investigation. LEVEL OF EVIDENCE/METHODS:4. Laryngoscope, 2018.

Intralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

BACKGROUND:A current challenge in osteoporosis is identifying patients at risk of bone fracture. PURPOSE/OBJECTIVE:To identify the machine learning classifiers that predict best osteoporotic bone fractures and, from the data, to highlight the imaging features and the anatomical regions that contribute most to prediction performance. STUDY TYPE/METHODS:Prospective (cross-sectional) case-control study. POPULATION/METHODS:. Field Strength/ Sequence: 3D FLASH at 3T. ASSESSMENT/RESULTS:Quantitative MRI outcomes by software algorithms. Mechanical and topological microstructural parameters of the trabecular bone were calculated for five femoral regions, and added to the vector of features together with bone mineral density measurement, fracture risk assessment tool (FRAX) score, and personal characteristics such as age, weight, and height. We fitted 15 classifiers using 200 randomized cross-validation datasets. Statistical Tests: Data: Kolmogorov-Smirnov test for normality. Model Performance: sensitivity, specificity, precision, accuracy, F1-test, receiver operating characteristic curve (ROC). Two-sided t-test, with P < 0.05 for statistical significance. RESULTS:The top three performing classifiers are RUS-boosted trees (in particular, performing best with head data, F1 = 0.64 ± 0.03), the logistic regression and the linear discriminant (both best with trochanteric datasets, F1 = 0.65 ± 0.03 and F1 = 0.67 ± 0.03, respectively). A permutation of these classifiers comprised the best three performers for four out of five anatomical datasets. After averaging across all the anatomical datasets, the score for the best performer, the boosted trees, was F1 = 0.63 ± 0.03 for All-features dataset, F1 = 0.52 ± 0.05 for the no-MRI dataset, and F1 = 0.48 ± 0.06 for the no-FRAX dataset. Data Conclusion: Of many classifiers, the RUS-boosted trees, the logistic regression, and the linear discriminant are best for predicting osteoporotic fracture. Both MRI and FRAX independently add value in identifying osteoporotic fractures. The femoral head, greater trochanter, and inter-trochanter anatomical regions within the proximal femur yielded better F1-scores for the best three classifiers. LEVEL OF EVIDENCE/METHODS:2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018.

Objectives/UNASSIGNED:To perform a needs-based assessment for tracheostomy care education for nonsurgical first responders in the hospital setting and to implement and assess the efficacy of a targeted tracheostomy educational program. Methods/UNASSIGNED:A prospective observational study conducted between October 2017 and May 2018 including emergency medicine (EM) residents, internal medicine (IM) residents, and intensive care unit (ICU) advanced practice providers at 2 tertiary hospitals. Needs-based assessments were conducted, leading to specialty specific curricula. One-hour educational sessions included didactics and case-based simulation. A pre- and posttest objective knowledge quiz and self-assessment were administered, and a posttest was repeated at 6 months. Results/UNASSIGNED:< .05) in mean objective knowledge score was seen across all groups between pre- and postintervention assessments with relative but not significant improvement at 6 months. There were significant increases in comfort level from pre- to postintervention. At 6-month follow-up, comfort level remained significantly increased for the majority of questions for the EM group and for select questions for IM and ICU advanced practice provider groups. Discussion/UNASSIGNED:Nonsurgeons are often first responders to critical airway situations yet receive limited formal education regarding tracheostomy. We demonstrated improvement in knowledge and comfort after a targeted educational module for tracheostomy care and management. Implications for Practice/UNASSIGNED:Although tracheostomy care is multidisciplinary, specialty-specific education may provide a more relevant foundation on which to build skills. Prompt and effective management of tracheostomy emergencies by first responders may improve patient safety and reduce mortality.

Oropharyngeal carcinoma associated with the human papillomavirus is increasing in incidence and represents a unique head and neck disease with favorable treatment outcomes. This review evaluates the evolving role of radiotherapy in regional management with an overall goal of treatment de-escalation in the appropriate patient. Determining the optimal approach and selection factors for treatment de-escalation is under active investigation. Response to induction chemotherapy, refining adverse pathologic factors after a primary surgical approach, decreasing radiation dose with or without chemotherapy in the definitive or adjuvant settings as well as more selective nodal level irradiation all are current strategies for treatment de-escalation. This review details the likely changes in regional radiotherapy management for oropharyngeal carcinoma in the modern human papillomavirus era and discusses future approaches to patient selection with the goal of reducing toxicities while maintaining function preservation and quality of life in group of patients who are younger and healthier than traditional head and neck cancer patients.