Characteristics and Outcomes in Head and Neck Sarcomatoid Squamous Cell Carcinoma: The Cleveland Clinic Experience.

OBJECTIVES: To analyze characteristics, treatment outcomes, and prognostic factors of sarcomatoid squamous cell carcinoma of the head and neck. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care center. SUBJECTS AND METHODS: Fifty-five patients were treated for sarcomatoid squamous cell carcinoma of the head and neck between 1996 and 2018. Data collection included clinical history, tumor characteristics, pathology, treatment modality, and outcomes. Mean follow up was 17.1 months. Cox univariate analysis was used to evaluate for associations with locoregional recurrence, distant metastasis, and overall survival. RESULTS: Most patients were white males with a smoking history and median age 66 years (range 41-92) at diagnosis. Twenty-two percent had prior head and neck radiation. Tumor site was most frequently oral cavity (41.8%), followed by larynx (29.1%), and oropharynx (16.4%). Half presented with early T stage disease (15.5% T0, 12.7% T1, 30.9% T2) and the remainder with late stage disease (16.4% T3, 34.5% T4). Locoregional recurrence rate was 60.0%, metastatic recurrence was rate 21.8%, with median time to recurrence of 4 months and mean overall survival of 20 months. Presence of lymphovascular space invasion was statistically associated with locoregional recurrence (P = .018, HR 3.55 [95% CI 1.24, 10.14]) and poorer overall survival (P = .015, HR 2.92 [95% CI 1.23, 4.80]). Treatment with multimodality therapy was associated with decreased locoregional recurrence (P = .039, HR 0.39 [95% CI 0.16, 0.95]) but did not impact overall survival. CONCLUSION: Sarcomatoid squamous cell carcinoma remains a rare and aggressive disease variant with high recurrence rates and high mortality. High risk features such as lymphovascular space may indicate the need for more aggressive therapy.

Pediatric ear foreign body retrieval: A comparison across specialties.

OBJECTIVES: To analyze what characteristics of patients and/or ear foreign bodies should prompt referral to otolaryngology with the goal to maximize successful removal and minimize complications. METHODS: This was a retrospective chart review of pediatric patients who presented for ear foreign body removal from January to December 2016 at a tertiary hospital center. Data collection included successful removal, major or minor complications, use of general or conscious sedation, use of otic or oral antibiotics, age of patient, comorbid behavioral disorders, and foreign body characteristics. Major complications included tympanic membrane perforation or ossicular damage. Minor complications included injury of the canal wall. Patients with retained or extruded tympanostomy tubes were excluded. RESULTS: Of 275 patients aged 1-18 years, 16% presented initially to otolaryngology (ENT), 48.4% presented to the emergency department (ED), 21.8% presented to a pediatrician, and 13.8% presented to ENT after prior attempts at removal. Rate of successful retrieval by ENT was significantly higher than by PCP (95.4% vs 75.0%) or by ENT after prior failed attempts (65.8%), but not when compared to removal in the ED (85.7%). Retrieval by ENT after prior failed attempts had the highest rate of minor complications (26.3% vs 2.3-6.0%). There were two tympanic membrane perforations that were noted by ENT after prior failed attempts. Rate of operative intervention was significantly higher in patients who presented to ENT after prior attempts compared to patients who presented initially to ENT (34.2 vs 4.6%, p = 0.001). Of the patients who presented to ENT after previous failed attempts, there was a higher rate of complications and operative intervention if the patient was age 5 or under or the foreign body was difficult to grasp. CONCLUSIONS: Pediatric ear foreign body presentation is common. Repeated attempts at removal are associated with higher rates of minor complications and operative intervention. Early consultation to otolaryngology should be considered if the foreign body is deemed difficult to grasp such as a bead or stone, especially if the patient is age five or younger.

Electroconvulsive therapy for depression in a patient with an Inspire hypoglossal nerve stimulator device for obstructive sleep apnea: A case report.

OUTCOME OBJECTIVES: METHODS: This is a case report of a patient who underwent placement and initiation of a hypoglossal nerve stimulator device in the context of receiving electroconvulsive therapy for bipolar depression between February and September 2016. To our knowledge, this has not yet been reported in the literature. Outcome measurements included successful device activation and successful device use throughout electroconvulsive therapy. RESULTS: The patient underwent successful device implantation, activation, and use without disruption throughout electroconvulsive therapy sessions. No special device deactivation was required during electroconvulsive therapy sessions. CONCLUSION: Obstructive sleep apnea is a common disorder that causes significant reduction in quality of life and is an independent risk factor for multiple comorbidities. Electroconvulsive therapy is an established treatment for medication-refractory depression with minimal risk in most patient populations. This is the first report in the literature of a patient undergoing ECT for bipolar depression with recent activation of Inspire hypoglossal nerve stimulator who had no disruption in the function of his implanted device.

Use of External Distractors and the Role of Imaging Prior to Mandibular Distraction in Infants With Isolated Pierre Robin Sequence and Stickler Syndrome.

IMPORTANCE: Computed tomographic (CT) scans are often obtained before mandibular distraction osteogenesis in patients with isolated Pierre Robin sequence. There is concern regarding the risk of radiation exposure from CT in children. OBJECTIVE: To evaluate whether preoperative CT is necessary for adequate airway, feeding, and aesthetic outcomes following mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence. DESIGN, SETTING, AND PARTICIPANTS: In a retrospective review of medical records, infants who underwent mandibular distraction between January 1, 1998, and September 30, 2014, at 2 tertiary children's hospitals were identified using procedure codes. Data analysis was conducted December 1, 2014, to March 31, 2015. Fifty-two patients fit the inclusion criteria of isolated Pierre Robin sequence or Stickler syndrome, of being younger than 9 months at the time of distraction, and of use of external distractors. Forty-two of these infants did not receive preoperative CT imaging. EXPOSURE: Mandibular distraction osteogenesis for isolated Pierre Robin sequence or Stickler syndrome. MAIN OUTCOME MEASURES: Number of infants who were able to avoid tracheostomy or achieve decannulation, who were able to avoid placement or achieve removal of a gastrostomy tube, and in whom there were no intraoperative complications, no open-bite deformity, no malocclusion, no asymmetry, and no postoperative complications. RESULTS: In comparison with the 10 infants who underwent preoperative CT, all 42 of the infants (100%) who did not receive preoperative CT imaging successfully avoided tracheostomy or achieved decannulation (P = .04) and 40 patients (95%) did not require placement of a gastrostomy tube or were able to undergo gastrostomy tube removal postoperatively (P < .001). There were no significant differences between the CT and non-CT groups in the other 5 outcome measures. Two patients (5%) required postoperative gastrostomy tube placement, 2 patients (5%) had minor intraoperative complications that might have been anticipated with CT, 2 patients (5%) demonstrated malocclusion, and 1 infant (2%) had asymmetry at the end of the distraction phase. CONCLUSIONS AND RELEVANCE: This series suggests that the absence of preoperative CT does not compromise functional or aesthetic outcomes in mandibular distraction with external distraction devices in infants with isolated Pierre Robin sequence or Stickler syndrome. This finding has implications for cost containment and reduction of radiation exposure to a vulnerable population. LEVEL OF EVIDENCE: 4.

Genetic instability triggered by G-quadruplex interacting Phen-DC compounds in Saccharomyces cerevisiae.

G-quadruplexes are nucleic acid secondary structures for which many biological roles have been proposed but whose existence in vivo has remained elusive. To assess their formation, highly specific G-quadruplex ligands are needed. Here, we tested Phen-DC(3) and Phen-DC(6), two recently released ligands of the bisquinolinium class. In vitro, both compounds exhibit high affinity for the G4 formed by the human minisatellite CEB1 and inhibit efficiently their unwinding by the yeast Pif1 helicase. In vivo, both compounds rapidly induced recombination-dependent rearrangements of CEB1 inserted in the Saccharomyces cerevisiae genome, but did not affect the stability of other tandem repeats lacking G-quadruplex forming sequences. The rearrangements yielded simple-deletion, double-deletion or complex reshuffling of the polymorphic motif units, mimicking the phenotype of the Pif1 inactivation. Treatment of Pif1-deficient cells with the Phen-DC compounds further increased CEB1 instability, revealing additional G4 formation per cell. In sharp contrast, the commonly used N-methyl-mesoporphyrin IX G-quadruplex ligand did not affect CEB1 stability. Altogether, these results demonstrate that the Phen-DC bisquinolinium compounds are potent molecular tools for probing the formation of G-quadruplexes in vivo, interfere with their processing and elucidate their biological roles.