Expanding the Spectrum of Sarcoma with an Internal Tandem Duplication of BCOR: A Non-Pediatric Nasosinusal Case.

INTRODUCTION: Undifferentiated small round-cell sarcomas with BCL6 corepressor (BCOR) alterations, such as an internal tandem duplication (ITD) within exon 15, are typically described as a pediatric group of Ewing-like small round-cell sarcomas. CASE PRESENTATION: In contrast to this notion, we report the case of a 71-year-old woman with a nasosinusal sarcoma featuring a BCOR ITD. To the best of our knowledge, this presence had not been previously documented in a sarcoma of the nasal and sinus cavities in an elderly patient. The identified duplication shares a similar minimal critical region as described in clear-cell sarcomas of the kidney in children. This alteration, located within the PCGF1 binding domain, is believed to disrupt the activity of PRC1.1. CONCLUSION: This case underscores the need for in-depth research into the molecular biology of these rare tumors and explores potential alternative treatment options. The patient achieved remission after two cycles of doxorubicin and cyclophosphamide chemotherapy, highlighting the promise of potential therapeutic options for BCOR ITD sarcomas.

Prospective Evaluation of Aesthetic and Functional Outcomes following Video-Assisted Rhino-Septoplasty.

BACKGROUND: Conservative techniques have been developed in the field of rhinoplasty, focusing on limited tissue resection while emphasizing the reshaping of nasal structural elements. OBJECTIVES: We aimed to prospectively evaluate aesthetic and functional outcomes following Video-Assisted Rhino-Septoplasty (VARS) using validated tools. METHODS: Patients' self-reported outcomes were assessed before and 6 months after surgery. Aesthetic evaluations used FACE-Q Rhinoplasty modules (FQRM) Satisfaction with Nose and Nostrils. Functional evaluations were performed with NOSE scores. T-tests were used to determine the significance of the change in scores before and after surgery. p value < 0.05 was considered statistically significant. RESULTS: Fifty patients were included (sex ratio = 0.16, mean age = 30 y.o., 78% primary cases). All patients had associated septoplasties, and 32 had inferior turbinate reductions. Mean FQRM Nose scores were 28.1 ± 16.3 before vs. 83.4 ± 17.2/100 after surgery. Mean FQRM Nostrils scores were 61.5 ± 28.6 before vs. 85 ± 21/100 after surgery. Mean NOSE scores were 49.5 ± 36.3 before vs. 14.8 ± 16.6/100 after surgery. All p <0.001. We found no correlation between FQRM Nose and NOSE scores after surgery (ρ = -0.1553, IC95% (-0.41;0.12), p = 0.28). CONCLUSION: Our study showed that VARS is an effective technique, yielding high patient satisfaction in both aesthetic and functional outcomes. LEVEL OF EVIDENCE II: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

Surgical management of chronic rhinosinusitis with nasal polyps under local anaesthesia: indications and results.

Objective: Our objective was to specify the indications and duration of effectiveness of Awake Patient Polyp Surgery (APPS) in Chronic Rhinosinusitis with Nasal Polyps (CRSwNP). Secondary objectives were to evaluate complications and Patient-Reported Experience (PREMs) and Outcome Measures (PROMs). Methods: We collected information regarding sex, age, comorbidities and treatments. Duration of effectiveness was the duration of non-recurrence defined by the time between APPS and a new treatment. Nasal Polyp Score (NPS) and Visual Analogic Scales (VAS, from 0/10 to 10/10) for nasal obstruction and olfactory disorders were assessed preoperatively and one month after surgery. PREMs were evaluated using a new tool: the APPS score. Results: Seventy-five patients were enrolled (SR = 3.1, mean age = 60.9 ± 12.3 years). 60% of patients had a previous history of sinus surgery, 90% had stage 4 NPS and more than 60% had overuse of systemic corticosteroids. Mean time of non-recurrence was 31.3 ± 2.3 months. We found a significant improvement (all p < 0.001) for NPS (3.8 ± 0.4 vs 1.5 ± 0.6), VAS obstruction (9.5 ± 1.6 vs 0.9 ± 1.7) and VAS olfactory disorders (4.9 ± 0.2 vs 3.8 ± 1.7). Mean APPS score was 46.3 ± 5.5/50. Conclusions: APPS is a safe and efficient procedure in the management of CRSwNP.

Patient Satisfaction After Non-surgical Rhinoplasty Using Hyaluronic Acid: A Literature Review.

BACKGROUND: Few studies have focused on assessing patient satisfaction after non-surgical rhinoplasty using hyaluronic acid (HA). The main objective of this study was to perform a systematic review assessing patient satisfaction after non-surgical rhinoplasty using HA. METHODS: We conducted a systematic review using the PRISMA model. All articles assessing patient satisfaction using self-questionnaires or scales (visual analogic scales, Likert scale) after non-surgical rhinoplasty were included. We also analyzed injection protocols and complications. The criteria for considering studies for the review were based on the population, intervention, comparison, outcome, timing and setting (PICOTS) framework. RESULTS: A total of 674 patients were included in eight studies. Mean age was 29.5 years old (min = 19, max = 67, sex ratio = 0.20). Rates of "satisfied" or "very satisfied" patients were 100% immediately after injection (n = 404/404 patients), 98.2% in the weeks following injection (n = 628/639 patients), 84.7% 3 months after injection (n = 333/393 patients) and 96.4% one year after injection (n = 60/62 patients). Duration of HA effectiveness ranged between 8 and 14 months. One local infection (0.001%) but no skin necrosis and no blindness were reported. Most of authors used less than 1 mL of HA per procedure. CONCLUSIONS: Provided precautions for use and indications are observed, and presuming good anatomical knowledge, non-surgical rhinoplasty is an interesting procedure associated with high satisfaction rates. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Cross-cultural adaptation into French and validation of the SCAR-Q questionnaire.

PURPOSE: Most questionnaires designed to evaluate patient-reported outcomes regarding scarring are available in English. The objective was to generate a validated French version of the SCAR-Q questionnaire. METHODS: The SCAR-Q questionnaire (including Appearance, Symptom and Psychological impact scales) was translated into French using a translation-back-translation process in accordance with international guidelines (ISPOR and WHO). For validation, two hundred patients consulting in our tertiary center completed the questionnaire. We tested scale reliability (Cronbach's α), floor/ceiling effects and item redundancy (inter-item correlations). Structural validity was tested using confirmatory factor analysis (CFA) with the robust weighted least squares (WLSMV) estimator and Delta parameterization. Model fit was examined using the root mean square error of approximation (RMSEA), the comparative fit index (CFI) and the Tucker-Lewis index (TLI). Correlations between scales and scale repeatability were tested (Spearman coefficient, Intra-class-coefficient). RESULTS: Four steps were required to obtain a translation consistent with the original version. Two hundred patients completed the questionnaire for validation. Internal consistency analysis found Cronbach's alphas > 0.7 for all scales (0.90 < α < 0.97). No floor or ceiling effect was found for all items (max = 85%). A ceiling effect was observed for all scales. Appearance and psychosocial impact scale items showed redundancy, with many inter-item correlations above 0.7. The CFA of the original structure displayed a reasonable fit, with RMSEA = 0.065, CFI = 0.974 and TLI = 0.972. Scales were positively correlated (0.45 < ρ < 0.65; p < 0.001). Test-retest intra-class correlation coefficients ranged from 0.94 to 0.99 for all scales. CONCLUSION: A French version of the SCAR-Q questionnaire is validated, ready for use.

French validation of the FACE-Q Rhinoplasty module.

OBJECTIVES: The aim of this study was to translate the FACE-Q "Rhinoplasty" module into French and validate its use for the French-speaking population. DESIGN: "Satisfaction with the nose" and "Satisfaction with nostrils" questionnaires were used and translated. SETTINGS: We were granted permission to use the FACE-Q rhinoplasty module by the development team. These two questionnaires constitute the rhinoplasty module of the FACE-Q questionnaire. ISPOR and WHO recommendations were used to complete the translation process from English into French in six steps. MAIN OUTCOME MEASUREMENT: Our goal was to obtain, not a literal translation, but rather a translation of the idea or concept. RESULTS: Each step allowed us to make changes to achieve a conceptual translation equivalent to the original version. CONCLUSION: The FACE-Q questionnaire is a reference in the field of cosmetic surgery outcome evaluation. The use of two validated translation recommendations, with a six-step translation-back translation process, leads to a French version corresponding to the original. This version is usable in a French-speaking population.

Human Polyomavirus-6 Infecting Lymph Nodes of a Patient With an Angiolymphoid Hyperplasia With Eosinophilia or Kimura Disease.

Human polyomavirus 6 (HPyV6) is most often detected at the skin surface of healthy individuals. Here, we demonstrate for the first time that HPyV6 also infects internal tissues. We provide direct evidence of HPyV6 infecting a lymph node of a patient with an angiolymphoid hyperplasia with eosinophilia or Kimura disease.

The role of radiation therapy in pediatric mucoepidermoid carcinomas of the salivary glands.

OBJECTIVE: To investigate the role of radiation therapy in rare salivary gland pediatric mucoepidermoid carcinoma (MEC). STUDY DESIGN: A French multicenter retrospective study (level of evidence 4) of children/adolescents treated for MEC between 1980 and 2010 was conducted. RESULTS: Median age of the 38 patients was 14 years. Parotid subsite, low-grade, and early primary stage tumors were encountered in 81%, 82%, and 68% of cases, respectively. All except 1 patient were treated by tumoral surgical excision, and 53% by neck dissection (80% of high grades). Postoperative radiation therapy and chemotherapy were performed in 29% and 11% of cases. With a median 62-month follow-up, overall survival and local control rates were 95% and 84%, respectively. There was 1 nodal relapse. Lower grade and early stage tumors had better survival. Postoperative radiation therapy and chemotherapy were associated with similar local rates. Patients with or without prior cancer had similar outcomes. CONCLUSIONS: Pediatric salivary gland MEC carries a good prognosis. Low-intermediate grade, early-stage tumors should be treated with surgery alone. Neck dissection should be performed in high-grade tumors. Radiation therapy should be proposed for high grade and/or advanced primary stage MEC. For high-grade tumors without massive neck involvement, irradiation volumes may be limited to the primary area, given the risk of long-term side effects of radiation therapy in children. Pediatric MEC as second cancers retain a similar prognosis. Long-term follow-up is needed to assess late side effects and second cancers.