Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor.

EWSR1::CREM gene fusions are increasingly being recognized in a diverse number of soft tissue tumors, including well-defined entities such as angiomatoid fibrous histiocytoma or clear cell sarcoma, and other unclassifiable tumors. As a group, EWSR1::CREM fused tumors often demonstrate primitive spindle or epithelioid cells, myxoid stroma, and a broad immunophenotype. Herein we present an unusual case of a child diagnosed with an intranasal malignant myxoid tumor harboring an EWSR1::CREM gene fusion. To the best of our knowledge, this is the first case of intranasal myxoid tumor with this particular fusion. Diagnosis and management of the case is discussed.

Reducing Chemotherapy Waiting Times in the Ambulatory Setting of a Tertiary Cancer Centre Using a Design Thinking Approach.

INTRODUCTION: Chemotherapy is complex. We hypothesized that a design thinking approach could redesign preparatory processes and reduce wait times. METHODS: A multidisciplinary process mapping exercise was undertaken to understand the current processes, followed by proposing and testing solutions. Proposals were selected based on desirability and feasibility. These focused on starting the morning treatments on time and scheduling pre-made regimens in these slots. The primary outcome measure was the time from the appointment to starting treatment. Treatments in the post-intervention study group were compared against a historical control group. RESULTS: The median time to start morning treatment decreased by 46%, from 83 min (with an interquartile range 50-127) in the control group to 45 min (with an interquartile range of 24-81 min) in the study group (p < 0.001). This translated into an overall improvement for the day, with the median time to start treatment decreasing from 77 min (with an interquartile range of 40-120 min) to 47 min (with an interquartile range of 20-79 min) (p < 0.001). Pre-makes increased by 258%, from 908 (28.5%) to 2340 (71.7%) regimens (p < 0.001). The number of patients starting treatment within an hour of their appointment increased from 1688 (32.8%) to 3355 (62.3%, p < 0.001). CONCLUSION: We have shown that a data-driven, design thinking approach can improve waiting times. This can be adapted to improve other processes in an empathetic, sustainable manner.

Analysis of Prognostic Factors For External Auditory Canal Carcinoma: A 22-Year Experience.

OBJECTIVE: This study aimed to investigate the prognostic factors and treatment outcomes in patients with the external auditory canal (EAC) squamous cell carcinoma (SCC). METHODS: All patients diagnosed with EAC SCC and treated with curative intent at a single tertiary institution were retrospectively reviewed over a 22-year period. Treatment modalities included surgery with adjuvant treatment or definitive radiotherapy. The primary endpoints were overall survival (OS) and disease-specific survival (DSS). RESULTS: There were 51 patients in our cohort. The 5-year OS and DSS were 64.0% ± 7.0% and 72.0% ± 7.0% respectively. Patients in the surgical arm and RT arm showed no significant difference in OS, DSS, and LRFS (p = 0.075, 0.062, 0.058 respectively). Compared to other routes of spread within the temporal bone, pattern of posterior disease invasion (involving mastoid/sigmoid sinus) showed poorer OS and DSS on multivariate analysis (hazard ratio, HR4.34 and 5.88; p = 0.006 and 0.009). On multivariate analysis, the following factors were independently prognostic of poorer OS and DSS: Previous radiotherapy (HR 3.29 and 4.81, p = 0.021 and p = 0.029); Presence of facial nerve palsy (HR 3.80 and 7.63, p = 0.013 and p = 0.003); Posterior pattern of invasion (HR4.05 and 3.59, p = 0.013 and p = 0.043). Advanced modified Pittsburgh stage was not predictive of poor OS and DSS (HR1.17 and 1.17 E+5, p = 0.786 and p = 0.961). CONCLUSION: Presence of previous radiotherapy, facial nerve palsy and posterior pattern of disease invasion were independent prognostic factors of poorer survival in patients with EAC SCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:2203-2210, 2023.

Outcomes of second-tier rapid response activations in a tertiary referral hospital: A prospective observational study.

INTRODUCTION: A second-tier rapid response team (RRT) is activated for patients who do not respond to first-tier measures. The premise of a tiered response is that first-tier responses by a ward team may identify and correct early states of deterioration or establish goals of care, thereby reducing unnecessary escalation of care to the RRT. Currently, utilisation and outcomes of tiered RRTs remain poorly described. METHODS: A prospective observational study of adult patients (age ≥18 years) who required RRT activations was conducted from February 2018 to December 2019. RESULTS: There were 951 consecutive RRT activations from 869 patients and 76.0% patients had a National Early Warning Score (NEWS) ≥5 at the time of RRT activation. The majority (79.8%) of patients required RRT interventions that included endotracheal intubation (12.7%), point-of-care ultrasound (17.0%), discussing goals of care (14.7%) and intensive care unit (ICU) admission (24.2%). Approximately 1 in 3 (36.6%) patients died during hospitalisation or within 30 days of RRT activation. In multivariate analysis, age ≥65 years, NEWS ≥7, ICU admission, longer hospitalisation days at RRT activation, Eastern Cooperative Oncology Group performance scores ≥3 (OR [odds ratio] 2.24, 95% CI [confidence interval] 1.45-3.46), metastatic cancer (OR 2.64, 95% CI 1.71-4.08) and haematological cancer (OR 2.78, 95% CI 1.84-4.19) were independently associated with mortality. CONCLUSION: Critical care interventions and escalation of care are common with second-tier RRTs. This supports the need for dedicated teams with specialised critical care services. Poor functional status, metastatic and haematological cancer are significantly associated with mortality, independent of age, NEWS and ICU admission. These factors should be considered during triage and goals of care discussion.

Adaptations of a Tertiary Otorhinolaryngology Head and Neck Surgery Department in Singapore during the COVID-19 Outbreak.

PURPOSE: The novel coronavirus 2019 (COVID-19) outbreak which was first reported in Wuhan, China has been declared a pandemic by the World Health Organization on March 11, 2020. Otorhinolaryngologists deal intimately with pathologies of the head and neck region and upper respiratory tract and have been reported as a vulnerable group of healthcare workers who may be more susceptible to COVID-19 nosocomial infection. METHODS: In this article, we provide a comprehensive overview of the adaptations of Singapore's largest tertiary Otorhinolaryngology department during the COVID-19 outbreak. This was undertaken via an evidence-based approach. The relevant medical literature and evidence underlying our adaptations are highlighted. RESULTS: A four-pronged strategy including (1) personnel segregation, (2) triaging and decantment, (3) use of personal protective equipment and (4) changes in clinical practice was employed. The strategy was bolstered by drawing upon a collective learnt experience from the 2003 Severe Acute Respiratory Syndrome (SARS) outbreak. CONCLUSION: A rigorous framework which can preserve operationality while navigating the heightened risks during this outbreak is critical for every Otorhinolaryngology department. As the pandemic continues to evolve and more scientific reports of this disease are made available, approaches will need to be morphed.

Awake Intubation via an Ambu AuraGain in a Patient With Extreme Obesity: A Case Report.

Severe obesity is associated with increased morbidity and mortality. Airway management is challenging due to difficulties in positioning and airway management and altered pulmonary physiology. We report a case of awake flexible bronchoscopic intubation via an Ambu AuraGain in a patient with a body mass index of 54 kg/m scheduled for elective thyroidectomy. The procedure was well tolerated and easily performed in the full sitting position for optimal airway and ventilation and cardiovascular stability during intubation. To our knowledge, this is the first published report of awake flexible bronchoscopic intubation via an AuraGain, and we discuss our rationale for using this technique.

Use of a trans-tracheal rapid insufflation of oxygen device in a "cannot intubate, cannot oxygenate" scenario in a parturient -a case report.

Background: The trans-tracheal rapid insufflation of oxygen (TRIO) device is less commonly used and is an alternative to trans-tracheal jet ventilation for maintaining oxygenation in a "cannot intubate, cannot oxygenate" (CICO) scenario. Case: We report the successful use of this device to maintain oxygenation after jet ventilator failure in a parturient who presented with the CICO scenario during the procedure for excision of laryngeal papilloma. Conclusions: A stepwise approach to the airway plan and preparation for an event of failure is essential for good materno-fetal outcomes. The TRIO device may result in inadequate ventilation that can lead to hypercarbia and respiratory acidosis. Hence, it should only be used as a temporizing measure before a definitive airway can be secured.

Carotid Artery Involvement in Advanced Recurrent Head and Neck Cancer: A Case Report and Literature Review.

BACKGROUND: Surgical management of advanced head and neck tumors involving the carotid artery remains controversial with compromised survival outcomes and heightened risks of morbidity and mortality. CASE: We describe a case of a 74-year-old lady with previous T1N0M0 left tongue squamous cell carcinoma, who developed a left nodal recurrence encasing the left external carotid artery from the carotid bifurcation. She underwent an extended left radical neck dissection with carotid artery patch plasty and remains well to date, 10 months after surgery. DISCUSSION: Head and neck tumor recurrences are not uncommon, and radical resection of advanced tumors involving the carotid artery is increasingly performed. Various carotid interventions including tumor peeling, ligation, and resection with reconstruction were reported with differences in survival outcomes, morbidity and mortality. CONCLUSION: An aggressive surgical approach to advanced tumors involving the carotid artery may be a viable attempt for survival prolongation but requires proper case selection and has to be weighed against the risks of complications to better optimize patient outcomes.

Outcome predictors in elderly head and neck free flap reconstruction: A retrospective study and systematic review of the current evidence.

Free flap tissue transfer has become the gold standard for reconstruction of composite head and neck defects. We sought to investigate the efficacy and morbidity of these procedures in the elderly. We retrospectively reviewed 245 head and neck free flap procedures (234 patients). Patients were stratified by age group (≥ or <65 years). Univariate and multivariate analyses were used to evaluate the following primary outcomes - free flap survival, postoperative medical and surgical complications and 30-day mortality. We found that free flap success and surgical complication rates were similar between the two age groups. Overall flap success and perioperative mortality rates were 94.3% and 2.1% respectively. Medical complications were significantly more common in the elderly group (p <0.001) and this correlated with comorbidity (OR = 2.81, p = 0.044) and advanced tumour stage (OR = 10.20, p= 0.029). Age was not independently associated with poor outcomes in our cohort. We then performed a systematic review of similar case-control studies worldwide and compared their findings with our results. We conclude that advanced age does not preclude free flap success in head and neck reconstruction. Rather, the presence of comorbidity appears to predict the development of medical complications postoperatively. Elderly patients with low comorbidity scores may be offered free flap reconstruction with less reservation.

Distant metastasis as the sole initial manifestation of well-differentiated thyroid carcinoma.

Thyroid carcinoma usually presents as a neck lump. Distant metastasis as the sole initial manifestation of well-differentiated thyroid carcinoma (WDTC) is rare and little is known about these patients. The aim of this study is to characterize patients who present with distant metastasis as the sole initial manifestation of WDTC. Retrospective review of case records of WDTC seen at the National Cancer Centre Singapore from 2002 to 2015 was performed. Patients with no prior complaint of neck swelling and whose first presentation was distant metastatic WDTC were included. Patient demographics, disease characteristics, radiological imaging, histopathology, types of treatment administered, and survival outcomes were examined. Nineteen out of seven hundred and thirty-two cases fulfilled inclusion criteria. Mean age was 65.4 years. All patients presented with osseous (36.8%), pulmonary (31.6%), cerebral metastases (5.3%), or a combination of two out of three aforementioned sites (26.3%). Follicular thyroid carcinoma was most common (47.4%), followed by papillary (36.8%) and medullary (15.8%). More than two-thirds of patients had multiple metastatic foci. Thirteen out of nineteen patients (68.4%) underwent total thyroidectomy with or without neck dissection and adjuvant RAI, while the rest declined surgery. The mean length of follow-up was 40.1 ± 5.1 months and 5-year disease-specific survival was 48.0 ± 17.2%. Distant metastasis without a history of neck swelling as the initial presentation of WDTC is extremely rare. Osseous metastasis and follicular thyroid carcinoma are the most common metastatic site and etiology, respectively. Disease-specific survival at 5-year post-diagnosis is lower compared to patients with thyroid carcinoma diagnosed with distant metastasis on further work-up.

Salivary fistula: Blue dye testing as part of an algorithm for early diagnosis.

Objective: Orocutaneous and pharyngocutaneous fistula (OPCF) is a debilitating complication of head and neck surgery for squamous cell carcinoma (SCC), resulting in delayed adjuvant treatment and prolonged hospitalization. As yet, there is no established test that can help in prompt and accurate diagnosis of OPCF. This study aims to determine the accuracy of bedside blue dye testing and its role as part of an algorithm for early diagnosis. We also analyze the risk factors predisposing to OPCF. Study Design: Retrospective cohort study from 2012 to 2014. Methods: Patients with head and neck SCC who underwent major resection and reconstruction, at risk of OPCF, were included. Results of blue-dye and video-fluoroscopic swallow-studies (VFSS) testing for OPCF were recorded. For the patients that were noted to develop OPCF, the length of time to diagnosis of fistula and subsequent mode of management were examined. Results: Of the 93 patients in this study, 25 (26.9%) developed OPCF. Advanced T-classification (T3/T4) was the only significant predisposing risk factor (p = 0.013). The sensitivity and specificity of the bedside blue dye testing was found to be 36.4% and 100%, respectively. The test positive patients were diagnosed with OPCF at a median of postoperative day (POD) 9.5 as compared to POD 13 for the test negative patients (p = 0.001). Early diagnosis was associated with faster fistula resolution with treatment. Conclusion: Blue dye testing is a simple bedside test that can assist in the early diagnosis of OPCF in patients, allowing treatment to be instituted earlier with improved outcomes. Level of Evidence: 3.

The Singapore Myeloma Study Group Consensus Guidelines for the management of patients with multiple myeloma.

Multiple myeloma (MM) is an incurable plasma cell neoplasm with an incidence of 100 patients per year in Singapore. Major advances have been made in the diagnosis, risk stratification and treatment of MM in the recent past. The reclassification of a subset of patients with smouldering MM, based on high-risk biomarkers, and the development of the revised international staging system are among the key new developments in diagnosis and staging. The use of novel agent-based treatment has resulted in significant improvements in the survival and quality of life of many patients with MM. Determining the optimal use of proteasome inhibitors, immunomodulators and, more recently, monoclonal antibodies is an area of ongoing investigation. In this guideline, we aim to provide an overview of the management of MM, incorporating the latest developments in diagnosis and treatment.

Patient-specific carbon nanocomposite tracheal prosthesis.

PURPOSE: Surgical removal of the trachea is the current gold standard for treating severe airway carcinoma and stenosis. Resection of 6 cm or more of the trachea requires a replacement graft due to anastomotic tension. The high failure rates of current grafts are attributed to a mismatching of mechanical properties and slow epithelium formation on the inner lumen surface. There is also a current lack of tracheal prostheses that are closely tailored to the patient's anatomy. METHODS: We propose the development of a patient-specific, artificial trachea made of carbon nanotubes and poly-di-methyl-siloxane (CNT-PDMS) composite material. Computational simulations and finite element analysis were used to study the stress behavior of the designed implant in a patient-specific, tracheal model. RESULTS: Finite element studies indicated that the patient-specific carbon nanocomposite prosthesis produced stress distributions that are closer to that of the natural trachea. In vitro studies conducted on the proposed material have demonstrated its biocompatibility and suitability for sustaining tracheal epithelial cell proliferation and differentiation. In vivo studies done in porcine models showed no adverse side effects or breathing difficulties, with complete regeneration of the epithelium in the prosthesis lumen within 2 weeks. CONCLUSIONS: This paper highlights the potential of a patient-specific CNT-PDMS graft as a viable airway replacement in severe tracheal carcinoma.