Papillary Carcinoma of Stensen's Duct with Intestinal Differentiation.

Stensen's duct carcinoma (StDC) is an extremely rare neoplasm, with fewer than 40 cases reported in the literature. We report a unique case of primary StDC with papillary features and intestinal differentiation of a 74-year-old male. We discuss the radiologic and pathologic correlation along with the differential diagnosis of this rare entity.

Chemotherapy-induced muscle wasting: association with NF-κB and cancer cachexia.

A compounding feature of greater than 50% of all cancers is the high incidence of the cachexia syndrome, a complex metabolic disorder characterized by extreme weight loss due mainly to the gross depletion of skeletal muscle tissue. Although studies into the cause of cancer cachexia has spanned over multiple decades, little is known about the effects of various cancer treatments themselves on cachexia. For example, chemotherapy agents induce side effects such as nausea and anorexia, but these symptoms do not fully account for the changes seen with cancer cachexia. In this study we examine the effects of chemotherapeutic compounds, specifically, cisplatin in the colon-26 adenocarcinoma model of cancer cachexia. We find that although cisplatin is able to reduce tumor burden as expected, muscle wasting in mice nevertheless persists. Strikingly, cisplatin alone was seen to regulate muscle atrophy, which was independent of the commonly implicated ubiquitin proteasome system. Finally, we show that cisplatin is able to induce NF-κB activity in both mouse muscles and myotube cultures, suggesting that an additional side effect of cancer treatment is the regulation of muscle wasting that may be mediated through activation of the NF-κB signaling pathway.

Tablet-based Screening for Hearing Loss: Feasibility of Testing in Nonspecialty Locations.

OBJECTIVE: To determine the feasibility of audiometric screening with tablet-based applications in typical clinic locations: examination room and clinic waiting area. STUDY DESIGN: A randomized prospective study. SETTING: Tertiary referral center. PATIENTS: Participants included 107 adult patients referred for audiometric testing to assess hearing loss. INTERVENTION: Each patient completed standard audiometry testing and one of three tablet-based audiometric applications that included pure-tone air conduction testing. The tablet-based audiometric testing was completed in a quiet examination room and a clinic waiting area using noise-cancellation headphones. A 5-question patient satisfaction survey was completed at the end of the testing. MAIN OUTCOME MEASURE: Thresholds at each frequency were compared with those obtained from tablet-based audiometric applications in a quiet examination room and clinic waiting area. Sensitivity and specificity of each tablet-based audiogram in detecting a hearing loss at each frequency was determined. RESULTS: All three tablet-based audiometric applications were user-friendly for hearing screening. However, one application was shown to be feasible and the most accurate of the three tested with 92% of thresholds within 10 dB of conventional audiometry across all test conditions. This application had a sensitivity of 96 to 100% and specificity of 72 to 85% for identifying a hearing loss in each frequency tested. Variability was noted among applications between testing in a quiet clinic room and testing in the clinic waiting area. Patients showed no preference for either conventional audiometry or the tablet-based device. CONCLUSION: Tablet-based audiometric applications can be used to screen for hearing loss in typical clinic locations. This tool does not replace standard audiometry testing but allows for screening for hearing disorders when appropriate and in settings without access to audiometric equipment.

Association of Compliance With Process-Related Quality Metrics and Improved Survival in Oral Cavity Squamous Cell Carcinoma.

IMPORTANCE: Quality metrics for patients with head and neck cancer are available, but it is unknown whether compliance with these metrics is associated with improved patient survival. OBJECTIVE: To identify whether compliance with various process-related quality metrics is associated with improved survival in patients with oral cavity squamous cell carcinoma who receive definitive surgery with or without adjuvant therapy. DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort study was conducted at a tertiary academic medical center among 192 patients with previously untreated oral cavity squamous cell carcinoma who underwent definitive surgery with or without adjuvant therapy between January 1, 2003, and December 31, 2010. Data analysis was performed from January 26 to August 7, 2015. INTERVENTIONS: Surgery with or without adjuvant therapy. MAIN OUTCOMES AND MEASURES: Compliance with a collection of process-related quality metrics possessing face validity that covered pretreatment evaluation, treatment, and posttreatment surveillance was evaluated. Association between compliance with these quality metrics and overall survival, disease-specific survival, and disease-free survival was calculated using univariable and multivariable Cox proportional hazards analysis. RESULTS: Among 192 patients, compliance with the individual quality metrics ranged from 19.7% to 93.6% (median, 82.8%). No pretreatment or surveillance metrics were associated with improved survival. Compliance with the following treatment-related quality metrics was associated with improved survival: elective neck dissection with lymph node yield of 18 or more, no unplanned surgery within 14 days of the index surgery, no unplanned 30-day readmissions, and referral for adjuvant radiotherapy for pathologic stage III or IV disease. Increased compliance with a "clinical care signature" composed of these 4 metrics was associated with improved overall survival, disease-specific survival, and disease-free survival on univariable analysis (log-rank test; P < .05 for each). On multivariable analysis controlling for pT stage, pN stage, extracapsular spread, margin status, and comorbidity, increased compliance with these 4 metrics was associated with improved overall survival (100% vs ≤50% compliance: adjusted hazard ratio [aHR], 4.2; 95% CI, 2.1-8.5; 100% vs 51%-99% compliance: aHR, 1.7; 95% CI, 1.0-3.1), improved disease-specific survival (100% vs ≤50% compliance: aHR, 3.9; 95% CI, 1.7-9.0; 100% vs 51%-99%: aHR, 1.3; 95% CI, 0.6-2.9), and improved disease-free survival (100% vs ≤50% compliance: aHR, 3.0; 95% CI, 1.5-5.8; 100% vs 51%-99% compliance: aHR, 1.6; 95% CI, 0.9-2.7). CONCLUSIONS AND RELEVANCE: Compliance with a core set of process-related quality metrics was associated with improved survival for patients with surgically managed oral cavity squamous cell carcinoma. Multi-institutional validation of these metrics is warranted.

Localized lymph node light chain amyloidosis.

Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and review the literature of lymph node amyloidosis discussing work-up and risk factors for systemic progression.

Connexin-related (DFNB1) hearing loss: is routine computed tomography imaging necessary?

OBJECTIVES: Determine if routine computed tomography (CT) imaging is necessary in the workup for children with connexin-related (DFNB1) sensorineural hearing loss (SNHL). STUDY DESIGN: Case-control retrospective chart and imaging review. SETTING: Tertiary care otolaryngology practice. SUBJECTS AND METHODS: High-resolution temporal bone CT scans of children (n = 21) with DFNB1 SNHL were compared to age-matched controls with either conductive hearing loss (CHL, n = 33) or a nonsyndromic, non-DFNB1 SNHL (n = 33). Sixteen measurements of cochleo-vestibular structures were recorded. Statistical analysis was performed using a repeated analysis of variance model that controlled for both age and gender. Area under the curve (AUC) and multidimensional AUC (MAUC) analyses were also performed. RESULTS: Overall, no statistically significant differences were found between the 3 experimental groups. In addition, comparisons between the DFNB1 and CHL groups, DFNB1 and non-DFNB1 SNHL groups, and CHL and non-DFNB1 SNHL groups failed to demonstrate any statistically significant differences. AUC and MAUC analyses also failed to detect any significant differences between the 3 groups. CONCLUSIONS: Patients with DFNB1 SNHL do not have significant anatomic differences on temporal bone CT scans when compared to non-DFNB1 SNHL and CHL control groups. Based on the above analysis, it is reasonable to avoid routine CT imaging of the temporal bones in children with known DFNB1 SNHL unless otherwise clinically indicated.

Embedding quality and safety in otolaryngology-head and neck surgery education.

Education in patient safety (PS) and quality improvement (QI) helps both medical students and residents understand the health care environment in the United States, where these concepts are now incorporated into virtually every aspect of patient care. The Accreditation Council of Graduate Medical Education has made PS/QI a mandatory component of resident education, and a number of specialties have published their experiences with incorporating PS/QI into their training programs. In otolaryngology-head and neck surgery, a strong curriculum can be built by teaching residents about the principles of PS/QI through both didactic and experiential learning, and morbidity and mortality and QI conferences can serve as the cornerstone of this curriculum. Understanding the potential challenges in PS/QI education can allow training programs to plan their strategy effectively for successful incorporation into their existing curricula.

Attitudes toward professionalism education in Otolaryngology-Head and Neck Surgery residency programs.

OBJECTIVES/HYPOTHESIS: Resident professionalism development is important for physician training and patient care. Meaningful professionalism curriculum requires collaboration between learners and educators. We aimed to better understand attitudes of Otolaryngology-Head and Neck Surgery (OHNS) program directors (PDs) and residents toward professionalism education. STUDY DESIGN: Prospective survey. METHODS: We identified contemporary methods of professionalism evaluation and education and formulated questions to assess attitudes toward professionalism education. Surveys were electronically distributed to 104 PDs who were asked to forward a separate survey to residents. RESULTS: The resident survey was completed by 110 OHNS residents, and the PD survey was completed by 33 OHNS PDs. The majority of residents (78%) and faculty (84%) believed professionalism could be taught. Role modeling (93%) and morbidity and mortality conference (90%) were the most common methods of education. Faculty questionnaires (100%) and nursing/ancillary staff questionnaires (93%) were the most common methods of resident evaluation. The majority of residents considered faculty mentoring (66%) and small group discussions (56%) to be useful methods for teaching professionalism, whereas only 14% considered formal lectures useful. Residents valued questionnaires by faculty (98%), medical staff (97%), and patients (94%) for professionalism evaluation. CONCLUSION: We are the first to study OHNS residents and PDs attitudes toward professionalism evaluation and education. Residents value mentoring programs and small group sessions rather than formal didactics and value evaluation from multiple sources. Programs should consider incorporating these specific educational and evaluative methods into their professionalism curricula. LEVEL OF EVIDENCE: N/A.

Endoscopic endonasal approaches to infratemporal fossa tumors: a classification system and case series.

OBJECTIVES/HYPOTHESIS: To propose a clinically applicable anatomic classification system describing three progressive endoscopic endonasal approaches (EEAs) to the infratemporal fossa (ITF) and their potential sequelae. Overall feasibility and outcomes of these approaches are presented through a consecutive case series. STUDY DESIGN: Description of classification system for EEAs to the ITF and case series. METHODS: A classification system of EEAs to ITF tumors was created based on the senior author's clinical experience and cadaveric dissection. A retrospective chart review of 21 child and adult patients with primary ITF tumors treated by these approaches from 2008 to 2012 at a tertiary-care academic medical center was conducted. RESULTS: Three progressive EEAs to ITF tumors were defined: 1) a transpterygopalatine fossa approach, 2) a transmedial pterygoid plate approach, and 3) a translateral pterygoid plate approach. Twenty-one patients treated with these approaches were identified consecutively, with a mean age of 44.2 years (range, 11-79 years). Tumors primarily involving the pterygopalatine fossa and not the ITF were excluded. Pathology included three advanced juvenile nasopharyngeal angiofibromas, three adenoid cystic carcinomas, two recurrent inverted papillomas, two trigeminal schwannomas, and 11 other diverse skull base pathologies. No intraoperative or postoperative complications occurred, with a mean follow-up of 21.5 months (range, 1-55 months). Expected potential sequelae such as V2/palatal numbness, Eustachian tube dysfunction, and trismus occurred in 10/21 patients. CONCLUSIONS: EEAs to ITF tumors are technically feasible with low risk of complications for well-selected patients. The proposed classification system is useful for anticipating potential sequelae for each approach.

ERCC1 protein expression is associated with differential survival in oropharyngeal head and neck squamous cell carcinoma.

OBJECTIVE: To investigate ERCC1 protein expression and its relationship to clinical factors and treatment outcomes in patients with head and neck squamous cell carcinoma (HNSCC). DESIGN: Case series. SETTING: Tertiary care academic center. SUBJECTS: One hundred and seventy-six patients diagnosed with HNSCC and treated with intent to cure between 2002 and 2008 were analyzed with respect to clinical data and tumor pathology. MAIN OUTCOME MEASURES: Tissue microarrays were constructed from tumor blocks and immunohistochemical staining for ERCC1 performed. ERCC1 expression status was dichotomized into high and low using the Allred score. Clinical characteristics of patients with high versus low ERCC1 expression were compared. Distributions of overall survival (OS) and progression-free survival (PFS) were analyzed using the Kaplan-Meier method. RESULTS: Of 176 patients, ERCC1 showed baseline nuclear staining in 148 patients (84.1%). Lower staining intensity ERCC1 expression was prominent in parabasal cells in the lower half of the epithelium, while at high staining intensity, ERCC1 expression was present throughout the epithelium. The median H-score was 50. No significant differences in age, gender, smoking status, tumor site, or stage were seen between the high and low ERCC1 expression groups. Expression of ERCC1 stratified by tumor site correlates with OS. Patients with oropharyngeal HNSCC and high ERCC1 expression (H-score > 120) were more likely to survive (P < .01) and remain disease free when compared to non-oropharyngeal squamous cell carcinoma (SCCa) patients with high ERCC1 expression despite treatment modality and human papillomavirus virus (HPV) status. CONCLUSION: Patients with oropharyngeal SCCa and high ERCC1 expression may have better outcomes despite HPV status.

Chromobacterium violaceum necrotizing fasciitis: a case report and review of the literature.

Necrotizing fasciitis is a severe, rapidly progressive infection of the subcutaneous tissue that causes significant destruction. It is rarely encountered in the pediatric population. We describe the case of a 14-year-old boy who was diagnosed with Chromobacterium violaceum necrotizing fasciitis and subsequently found to have autosomal recessive chronic granulomatous disease.

STT3A, C1orf24, TFF3: putative markers for characterization of follicular thyroid neoplasms from fine-needle aspirates.

OBJECTIVES/HYPOTHESIS: The goals of this study were to characterize gene expression using fine-needle aspirates (FNAs) from follicular neoplasms to distinguish follicular adenomas (FAs) from follicular thyroid carcinomas (FTCs) and follicular variant of papillary thyroid carcinomas (FVPTCs); and to use FNA material to distinguish benign from malignant follicular neoplasms. STUDY DESIGN: Retrospective expression analysis of diagnosed follicular neoplasms (level of evidence 2b); prospective cohort of FNA from the operating room after thyroid lobectomy (level of evidence 1b). METHODS: Gene expression analysis via reverse-transcription polymerase chain reaction (rt-PCR) of nine genes previously noted to be differentially expressed in follicular neoplasms was performed on formalin-fixed, paraffin-embedded archived normal thyroid tissue (n = 63) and follicular neoplasms as diagnosed on preoperative FNA: FA (n = 16), FTC (n = 13), FVPTC (n = 24), and papillary thyroid carcinomas (PTCs) (n = 10). All cases were originally read as follicular neoplasms on preoperative FNA. To determine if these results could be translated into fresh tissue, ex vivo FNA was performed on follicular neoplasms (n = 17) in the operating room after thyroidectomy. RESULTS: Quantitative gene analysis detected differential TFF3 expression in FA versus FTC, FVPTC, and PTC (P = .02). Rt-PCR of FNA samples demonstrated that malignant nodules overexpress STT3A as compared with benign disease (P = .046). The combination of STT3A overexpression/Clorf24 underexpression identified malignant disease (P = .03) on FNA samples. CONCLUSIONS: Gene-expression data suggest a difference in expression between STT3A, Clorf24, and TFF3 in FAs versus carcinomas that may be detected from an FNA sample. Findings must be validated from preoperative FNAs in larger numbers.

Transpalatal greater palatine canal injection: Radioanatomic analysis of where to bend the needle for pediatric sinus surgery.

BACKGROUND: The greater palatine canal (GPC) local injection is used to limit posterior bleeding during sinus surgery in adults. Given the potential for causing iatrogenic damage to the intraorbital contents, this procedure is not commonly used in the pediatric population. No studies have described the anatomic development of the GPC during facial growth. By using age-stratified radioanatomic analysis, the dimensions of the GPC and the clinical implications are described for pediatric patients. An age-stratified radioanatomic study was performed. METHODS: High-resolution computed tomography measurements included the thickness of the mucosal plane overlying the GPC, the length of the GPC, and the distance between the base of the pterygopalatine fossa (PPF) and the orbital floor. Mean distance and standard deviation were calculated for each age cohort and compared using the one-way ANOVA test. RESULTS: The GPC length correlated directly with patient age. It varied from 9.14 ± 0.11 mm in the youngest age group (<2 years) to 19.36 ± 2.76 mm in adults (18-64 years). The height of the orbit relative to the hard palate approximated the adult dimensions described in the literature by 12-13 years (49.58 ± 1.72 mm). CONCLUSION: These radioanatomic results suggest that the GPC injection described for adult patients may be safely administered to selected pediatric patients. For patients >12 years old, we recommend bending the needle 45° and inserting it 25 mm. For patients 6-12 years old, the needle should be inserted 20 mm to enter into the PPF. In patients <6 years old, the needle may safely be placed 12 mm into the GPC. Each of these descriptions is based on the minimal distance required to effectively access the PPF but with maximal safety in regard to the orbit. Further clinical correlation of these findings is necessary through future investigation.

Molecular biology of head and neck cancer: risks and pathways.

Patients present with a differential baseline risk of cancer based on normal and expected variations in genes associated with cancer. The baseline risk of developing cancer is acted on throughout life as the genome of different cells interacts with the environment in the form of exposures (eg, toxins, infections). As genetic damage is incurred throughout a lifetime (directly to DNA sequences or to the epigenome), events are set in motion to progressively disrupt normal cellular pathways toward tumorigenesis. This article attempts to characterize broad categories of genetic aberrations and pathways in a manner that might be useful for the clinician to understand the risk of developing cancer, the pathways that are disrupted, and the potential for molecular-based diagnostics.