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Background and Objectives: Salivary gland Secretory Carcinoma (SC), characterized by Skalova in 2010 is a rare tumor studied within the REFCOR (French Network of experts on Rare Head and Neck Cancers). We conducted a prospective multicentric cohort study of 108 SC cases in the REFCOR database up to July 2021, analyzing diagnostic, therapeutic, and survival data., Methods: Data was collected prospectively from diagnosis to the last update. Each patient had two histological readings including one by a REFCORpath pathologist, and all cases underwent molecular testing to confirm diagnosis. Statistical analyses were performed using R software., Results: MRI was not contributive to malignancy diagnosis. After 2 histological readings, 79 % of patients were diagnosed, with 21 % requiring molecular testing to confirm diagnosis. Surgical treatment typically involved tumor excision and lymph node dissection. The tumor exhibited low lymph node involvement, with 95 % of patients being cN0, and no nodal metastases post-dissection. Five-year overall survival and recurrence-free survival were 91.4 % {95 % CI (0.84-1)} and 89 % {95 % CI (0.81; 0.98)} respectively, indicating a favorable prognosis., Conclusions: SC is a rare and newly recognized tumor, with generally favorable outcomes. Our cohort, among the largest to date, provides valuable insights. Future research should refine treatment guidelines., (Copyright © 2024. Published by Elsevier Ltd.)

Objectives: The management of the facial nerve (FN) is a major issue in parotid cancer, especially when there is no preoperative facial palsy and FN invasion is discovered intraoperatively. The aim of this study was to assess the impact of FN resection in patients with parotid cancer abutting the FN, without pretreatment facial palsy, using a propensity score matching., Materials and Methods: Data from all patients treated between 2009 and 2020 for a primary parotid cancer abutting or invading the FN but without pretreatment facial palsy were extracted from the national multicentric REFCOR database. Three different definitions of tumors abutting the FN were used for sensitivity analyses, in a retrospective setting. Propensity score matching was used to assess the impact of FN resection on disease-free survival (DFS), overall survival (OS) and locoregional recurrence-free survival (LRRFS)., Results: A total of 163 patients with parotid cancer abutting or invading the FN without pretreatment facial palsy were included. Among them, 99 patients (61 %) underwent FN resection. After overlap weighting and multiple imputation, no benefit of FN resection over preservation was found in terms of OS (HR = 1.21, p = 0.6), DFS (HR = 0.88, p = 0.5) and LRRFS (HR = 0.99, p = 1). Sensitivity analyses revealed similar results, and no significant efficacy was found in the subgroup analyses., Conclusion: In this retrospective study with propensity score analysis, FN resection did not improve survival outcomes in patients without preoperative facial palsy treated surgically for a primary parotid cancer abutting the FN. In line with recent guidelines, the results of this study suggest that FN preservation should be considered whenever possible in this specific group of patients., Competing Interests: Declaration of competing interest The authors have declared no conflict of interest., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: Autofluorescence spectroscopy is effective for noninvasive detection but underutilized in tissue with various pathology analyses. This study evaluates whether AFS can be used to discriminate between different types of laryngeal lesions in view of assisting in vocal fold surgery and preoperative investigations., Methods: A total of 1308 spectra were recorded from 29 vocal fold samples obtained from 23 patients. Multiclass analysis was performed on the spectral data, categorizing lesions into normal, benign, dysplastic, or carcinoma., Results: Through an appropriate selection of spectral components and a cascading classification approach based on artificial neural networks, a classification rate of 97% was achieved for each lesion class, compared to 52% using autofluorescence intensity., Conclusions: The ex vivo study demonstrates the effectiveness of AFS combined with multivariate analysis for accurate classification of vocal fold lesions. Comprehensive analysis of spectral data significantly improves classification accuracy, such as distinguishing malignant from precancerous or benign lesions., (© 2024 Wiley Periodicals LLC.)

Objectives: To analyse prognostic factors and survival outcomes of malignant tumors of the external auditory canal, to investigate the role of regional surgery, and adjuvant radiotherapy in early stages and to investigate the role of surgery in operable T4 stage., Setting: A retrospective analysis was conducted on all patients prospectively included in the national database of the French Expertize Network for Rare ENT Cancers (REFCOR) from January 2000 to December 2016., Participants: 103 patients from 19 reference centers were included. A propensity score matching analysis was applied to enable comparisons between treatments., Main Outcomes and Measures: Event-free survival, overall survival and factors of poor prognosis of the cohort were described. The interest of local and regional surgery and postoperative radiotherapy were evaluated., Results: The factors of poor prognosis on event-free survival were immunosuppression (p = 0.002), Karnofsky status less than 90% (p = 0.02), body mass index less than 19 Kg / m2 (p = 0.0009), peripheric facial palsy (p = 0.0016), and positive margin (p = 0.0006). In early stages, locoregional surgery was associated with an increase in event-free survival (p = 0.003, HR = 0.21) versus local surgery alone, while postoperative radiotherapy was not associated with an increase in event-free survival (p = 0.86, HR = 0.91) or overall (p = 0.86, HR = 0.91). In locally advanced stages, locoregional surgery followed by radiotherapy was associated with an increase in event-free survival (p = 0.03, HR = 0.39) and overall (p = 0.02, HR = 0.34) versus chemoradiotherapy alone., Conclusion and Relevance: Regional surgery is recommended for early stages of cancers of the external auditory canal. In operable cases, locoregional surgery followed by radiotherapy is recommended., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction: Laryngeal chondrosarcoma (LCS) is a rare tumor of slow evolution whose treatment is poorly codified. For a long time, a radical treatment by total laryngectomy (TL) was proposed. More recent studies tend to propose a conservative surgical approach of the larynx. The objective of this study was to compare the overall survival (OS) of total laryngectomized patients (TL+) versus non-laryngectomized patients (TL-). The secondary objectives were to analyse the reoperation free survival (RFS), the total laryngectomy free survival (TLFS) and to identify the preoperative factors leading surgeons to propose TL., Materials and Methods: A retrospective analysis of prospectively collected incident cases from the REFCOR and NetSarc-ResOs multicenter databases between March 1997 and June 2021 was conducted. A propensity score matching analysis was performed to compare the OS of TL+ and TL-patients., Results: 74 patients were included. After propensity score, the 5-year OS of TL+ and TL-patients was comparable (100 %, p = 1). The 5-year RFS rate was 69.2 % (95 % CI [57.5-83.4]) and the 5-year TLFS was 61.7 % (95 % CI [50.4-75.5]). Cricoid involvement greater than 50 % (HR 3.58; IC 95 % [1.61-7.92] p < 0.001), an ASA score of 3 or 4 (HR 5.07; IC 95 % [1.64-15.67] p = 0.009) and involvement of several cartilages (HR 5.26; IC 95 % [1.17-23.6] p = 0.04) are prognostic factors for TL. Dyspnea caused by the tumour is a prognostic factor for reoperation (HR 2.59; IC 95 % [1.04-6.45] p = 0.03)., Conclusion: These results demonstrate that conservative treatment should be considered as first-line treatment for laryngeal chondrosarcoma., Competing Interests: Declaration of competing interest None., (© 2023 Published by Elsevier Ltd.)

Background: Acinic cell carcinomas (AciCCs) are malignant tumours of the salivary glands. The aim of this work was to analyse data from the national REFCOR multicenter cohort (i) to investigate the prognostic factors influencing survival outcomes in AciCC, (ii) to assess the impact on survival of postoperative radiotherapy (RT) in patients treated for AciCC without high-grade transformation and (iii) to explore the prognostic impact of prophylactic neck dissection (ND) in patients treated for AciCC of the major salivary glands., Patients and Methods: Data from all the patients treated for salivary AciCC between 2009 and 2020 were extracted from the REFCOR database. Survival outcomes and prognostic factors influencing Disease-Free Survival (DFS) and Overall Survival (OS) were investigated using univariate and multivariate analyses. Propensity score matching was used to assess the impact of postoperative RT and prophylactic ND on DFS., Results: A total of 187 patients were included. After a median follow-up of 53 months, their 5-year OS and DFS rates were 92.8% and 76.2%, respectively. In multivariate analysis, male sex, older age, higher T and N status, and high grade were independently associated with a worse DFS. In the subpopulation analysed after propensity score matching, patients with cN0 AciCC without high-grade transformation who were treated by surgery and RT did not have an improved DFS compared to patients who were treated by surgery alone (hazard ratio (HR) = 0.87, p = 0.8). Factors associated with nodal invasion were T3-T4 status and intermediate/high histological grade. After propensity score matching, prophylactic ND was associated with a trend toward a better DFS (HR = 0.46, p = 0.16)., Conclusions: These results suggest that (i) long-term follow-up (>5 years) should be considered in patients with AciCC, (ii) treatment by surgery alone could be an option in selected cN0 patients with AciCC without high-grade transformation and (iii) prophylactic ND may be considered preferentially in patients with T3-T4 status and/or intermediate/high histological grade., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Objectives: Close margin is a frequent situation after parotidectomy. The need for systematic prophylactic revision surgery is a question that arises regularly for malignant tumors, as it exposes to a high risk of facial palsy, while oncological benefits are unclear., Study Design: retrospective study., Setting: Multicentric., Subjects and Methods: We included all patients operated for systematic revision surgery in case of close margins after parotidectomy for a malignant tumor and analyzed the rate of tumor residue and its risk factors., Results: A tumor residue was identified in 43.5% of 23 cases, but none in case of initial complete excision with supra-millimetric margins. Invaded lymph nodes were identified in 6 cases, but none in case of low-grade tumors., Conclusions: Systematic revision seems mandatory in case of infra-millimetric margins and high-grade tumors or positive lymph node; further studies are needed to confirm whether it can be spared for T1-T2/N0 low-grade tumors, with close margins but complete initial excision.

Objectives: Human papillomavirus (HPV) is a risk factor for head and neck squamous cell carcinoma (HNSCC), which is currently increasing worldwide. We evaluated the prevalence of HPV DNA and p16 expression in HNSCC patients age <45 years compared with patients aged ≥45 years., Methods: Thirty-nine patients aged <45 years who presented at Besançon University Hospital with HNSCC since 2005 were included in this retrospective study. HPV DNA was detected by HPV genotyping and p16 expression was determined by immunohistochemistry using paraffin-embedded tissues. A matched-group of 38 patients aged ≥45 years from Besançon University Hospital was included., Results: The overall prevalence of HPV infection was 11.7%. HPV16 was the only genotype detected in 4/39 and 5/38 patients, and p16 was expressed in 6/39 and 4/38 patients aged <45 years and ≥45 years, respectively., Conclusions: HPV-positivity and p16 expression were similar in both age groups. The results suggest that p16 immunohistochemistry may provide a prognosis biomarker for all HNSCCs, not only oropharyngeal cancers, and this should be addressed in large clinical trials.

Background: Patterns of nodal involvement in adenoid cystic carcinoma (ACC) of the head and neck have not been sufficiently assessed to guide a decision of prophylactic neck dissection (ND). The objective of this study is to analyse the influence of ND on event-free survival (EFS) for patients with cN0 ACC., Patients and Methods: A multicentre prospective study was conducted between 2009 and 2018. Patients presenting cN0 non-metastatic ACC on any site, and who received surgery on the tumour, were included. EFS was the main judgement criterion. A comparative survival analysis between the groups that received a ND versus those that did not was performed, using a propensity score. Analyses were carried out using the R software., Results: Between 2009 and 2018, 322 patients with cN0 ACC were included, out of which 58% were female. The average age was 53 years. Tumours were in minor salivary glands in 58% of cases, and 52% had T3/T4 stages. ND was performed on 46% of patients. Out of them, seven had histological lymph node invasion, out of which six had tumour infiltration in the mucosa of oral cavity. After propensity score, the median EFS for N0 patients with ND was 72 months (95% Confidence Interval (CI) [48-81]), compared to 73 months (95% CI [52-85]) for patients without ND (HR = 1.33; 95% CI [0.82-2.16]; p = 0.2)., Conclusion: ND of cN0 patients does not provide any benefit on EFS, which suggests that its application on such patients is not necessary., Competing Interests: Conflict of interest statement The authors have declared no conflict of interest., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Adenoid cystic carcinoma (ACC) accounts for 1% of malignant head and neck tumours [1] and 10% of salivary glands malignant tumours. The main objective of our study is to investigate the prognostic factors influencing the event-free survival (EFS) of patients with ACC., Patients and Methods: A multicentre prospective study was conducted from 2009 to 2018. All 470 patients with ACC whose survival data appear in the REFCOR database were included in the study. The main judgement criterion was EFS. Both a bivariate survival analysis using log-rank test and a multivariate using Cox model were performed using the R software., Results: Average age was 55 years. Females accounted for 59.4% of the cohort. The body mass index (BMI) was normal in 86% of cases. Tumours were located in minor salivary glands in 60% of cases. T3/T4 stages represented 58%; 89% of patients were cN0. histological grade III was observed on 21% of patients. The EFS and overall 5-year survival rates were 50% and 85%, respectively. After adjustment, the most significant pejorative prognostic factors were age ≥65 years (hazard ratio [HR] = 1.67), BMI<16.5 (HR = 2.62), and lymph node invasion cN (HR = 2.08)., Conclusion: Age, BMI and N stage are the three main clinical prognostic factors determining EFS identified in this prospective series of patients with ACC. Such findings open new research perspectives on the influence of these components on initial patient care., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: The incidence of cancer during pregnancy is low but is slightly increasing. Data on incidence and etiology of head and neck (HN) cancers in pregnant women are rare. We evaluated the frequency, tumor type, associated factors, and specific biomarkers in HN cancers occurring in pregnant (and peripartum) women., Methods: A systematic literature search was performed on PubMed, for any HN tumor site occurring in pregnant women., Results: Sixty cases of HN cancers occurring during pregnancy were identified. Most of them were oral cavity cancers. Relationships with oncogenic viruses, hormonal disturbance, and shift in maternal immunity profile were identified., Conclusion: Carcinogenesis of HN cancers in pregnant women may be led by different cancer type-specific hallmarks. Relevance of these etiological factors with respect to treatments and birth control recommendations is being investigated by the REFCOR in an ambispective study., (© 2019 Wiley Periodicals, Inc.)

Background: The purposes of this study were to describe the characteristics of a prospective multicenter series of patients with salivary duct carcinoma and to investigate prognostic factors., Methods: Patients included for salivary duct carcinoma between 2009 and 2016 in the Réseau d'Expertise Français des Cancers ORL Rares (REFCOR) database were selected. Immunohistochemical analyses were performed., Results: Sixty-one patients were included in this study. The primary site was the parotid gland in 90% of the cases. Fifty-seven percent of the tumors were stage IV, 65% of patients had lymph node involvement, and 10% had metastases. Tumors showed androgen receptor (89%) and human epidermal growth factor receptor 2 (HER2)/neu (36%). Ninety-four percent of patients underwent surgery and 86% had postoperative radiotherapy. Six patients were treated with targeted therapies. The 3-year overall survival (OS) was 74% and the 3-year disease-free survival (DFS) was 44%. Tumor stages III to IV reduced DFS (hazard ratio [HR] 4.3; P = .04). The N2/3 class reduced distant metastasis-free survival (HR 7.3; P = .007)., Conclusion: Salivary duct carcinoma prognosis is poor and is correlated with tumor stage and lymph node classification. Androgen receptor and HER2/neu should be tested as they offer the possibility of targeted therapies., (© 2018 Wiley Periodicals, Inc.)

Primitive tumors of pterygopalatine fossa (PPF) are often benign tumors or extension of a malignant sinonasal tract. Primitive tumors may rarely occur in PPF.The authors present a 71-year-old woman with a 6-month history of left cranial nerve palsy. Magnetic resonance imaging and positron emission tomography-computed tomography showed an enhancing isolated lesion at the PPF. A transmaxillary biopsy was performed, leading to diagnosis of primitive squamous cell carcinoma. The patient underwent radiotherapy treatment.Primitive tumors of PPF are rare and diagnosis may be difficult. Endoscopic access for diagnosis can be performed. Squamous cell carcinoma occurring in PPF is associated with poor prognosis.

Introduction: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome., Case Report: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica., Conclusion: Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Introduction: Reconstruction of the soft palate after oncologic resection remains a surgical challenge. Speech and swallowing problems are the consequences of velopharyngeal incompetence following soft palate resection. Free tissue transfer like radial forearm flaps can be used in larger defects for complex reconstruction. The conformation of the flap in order to be closer to the shape of the soft palate improves the functional outcome. In the same way, we describe an original "double-arched" flap design., Methods: A double arch of the exact length of the soft palate tumor resection is designed. After suturing, the flap spontaneously formed a double arch of the exact dimensions of the resected piece., Results: The patient achieved good functional recovery without any surgical complications., Conclusion: The original "double-arched" forearm flap design allows a tailored reconstruction with exactly the same shape and dimensions, preserving the functional requirements of speech and deglutition., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

NK cells are critical for innate immunity-mediated protection. The main roles of NK cells rely on their cytotoxic functions or depend on the tuning of Th1 adaptive immunity by IFN-γ. However, the precise influence of inflammatory cytokines on NK cell and CD4 T lymphocyte interactions was never investigated. In this study, we provide evidence that IL-21, a cytokine produced during chronic inflammation or infectious diseases, promotes the differentiation of a specific subset of NK cells coexpressing CD86 and HLA-DR and lacking NKp44. More importantly, IL-21-propagated HLA-DR(+) NK cells produce macrophage migration inhibitory factor and provide costimulatory signaling during naive CD4(+) T cell priming inducing the differentiation of uncommitted central memory T cells. Central memory T cells expanded in the presence of HLA-DR(+) NK cells are CXCR3(+)CCR6(-)CCR4(-)CXCR5(-) and produce IL-2, as well as low levels of TNF-α. Costimulation of CD4(+) T cells by HLA-DR(+) NK cells prevents the acquisition of effector memory phenotype induced by IL-2. Moreover, we identified this population of NK HLA-DR(+) macrophage migration inhibitory factor(+) cells in inflammatory human appendix. Collectively, these results demonstrate a novel function for IL-21 in tuning NK and CD4(+) T cell interactions promoting a specific expansion of central memory lymphocytes., (Copyright © 2016 by The American Association of Immunologists, Inc.)