Carcinomas of the external auditory canal: Management and results: A multicenter REFCOR propensity score matching study.

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.

[Rare cancers of the head and neck on behalf of the REFCOR, part 2]. / Cancers rares de la tête et du cou sous l'égide du REFCOR, partie 2.

Among the 16,000 new cases of malignant tumors of the head and neck diagnosed in France each year, 10% are not conventional squamous cell carcinomas. These so-called rare cancers are distinguished by their presentation and patterns of failure, which is important to recognize in order to offer specific adapted management and maximize the chances of tumor control. These cancers can be rare by their histology as well as their anatomical location when arising from the paranasal sinuses, salivary glands and ear. The management of these heterogeneous rare diseases of complex treatment has considerably been structured over the last 15 years, in particular via the French ENT Cancer Expertise Network (REFCOR) and international networks and registries (EURACAN, etc.). Structuration also favors research with identification of new entities and setting up of specific therapeutic trials. A first article (part 1) discusses the diagnostic and therapeutic specificities of these rare cancers, and develops the recommendations of the REFCOR concerning rare epithelial tumors, i.e., salivary tumors, sinonasal tumors, variants of conventional squamous cell carcinomas, neuroendocrine carcinomas, malignant odontogenic tumors, and ear tumors. This second article (part 2) is focused on non-epithelial tumors (sarcomas, mucosal melanomas, lymphomas, tumors of uncertain or undetermined malignancy) and describes the organization and missions of the REFCOR.

[Rare cancers of the head and neck on behalf of the REFCOR, part 1]. / Cancers rares de la tête et du cou sous l'égide du REFCOR, partie 1.

Among the 16,000 new cases of malignant tumors of the head and neck diagnosed in France each year, 10% are not conventional squamous cell carcinomas. These so-called rare cancers are distinguished by their presentation and patterns of failure, which is important to recognize in order to offer specific adapted management and maximize the chances of tumor control. These cancers can be rare by their histology, which determines their local invasiveness, and their hematogenous/nodal spread. Their diagnosis can be difficult and often requires comprehensive immunohistochemistry and genomic techniques. Expert pathology review is recommended in the cases of undifferentiated tumors, sarcomas and at the slightest diagnostic doubt. These rare cancers can also be rare by their anatomical location when arising from the paranasal sinuses, salivary glands and ear. Their location requires knowledge of their specific extension routes, and may call for a specific surgical technique (skull base endoscopic sinus surgery, extended total parotidectomy, etc.) and adapted radiotherapy to spare healthy organs surrounding the tumor. This article (part 1) discusses the diagnostic and therapeutic specificities of these rare cancers, and develops the recommendations of the French ENT Cancer Expertise Network (REFCOR) concerning rare epithelial tumors, i.e., salivary tumors, sinonasal tumors, variants of conventional squamous cell carcinomas, neuroendocrine carcinomas, malignant odontogenic tumors, and ear tumors. A second article (part 2) is focused on non-epithelial tumors (sarcomas, mucosal melanomas, lymphomas, tumors of uncertain or undetermined malignancy) and describes the organization and missions of the REFCOR.

[A thyroid-like low-grade nasopharyngeal papillary adenocarcinoma]. / Un cas rare d'adénocarcinome papillaire nasopharyngé thyroid-like de bas grade.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma is a rare histopathological entity. Patients usually complain of nasal obstruction and epistaxis. Diagnosis is confirmed on endonasal biopsy using immunohistochemical studies. Surgery is the treatment of choice and this pathology exhibits no metastasizes nor recurrence after treatment.

Mucoepidermoid carcinoma of salivary glands: A French Network of Rare Head and Neck Tumors (REFCOR) prospective study of 292 cases.

BACKGROUND: To describe the characteristics of the largest European study of MEC of salivary glands and to determine the prognostic factors for overall and disease free survival. PATIENTS AND METHODS: Patients with MEC were prospectively included in the Réseau d'Expertise Français sur les Cancers ORL Rares (REFCOR, French Network of Rare Head and Neck Tumors) database between 2009 and 2015. RESULTS: A total of 292 patients were included. Tumors were classified as low grade in 175 cases (60%), intermediate in 39 (13%) and high grade in 78 (27%). Median follow-up was 26 months. The 5-year OS and DFS rates were respectively 83% and 69%. In multivariate analysis, age (p = 0.004), diabetes (p = 0.02) and advanced stage (p = 0.03) were found to have a significant negative impact on OS. Diabetes (p = 0.001), alcohol consumption (p = 0.003) and advanced stage (p = 0.001) were found to have a significant negative impact on DFS. Compare to low grade, high grade tended to have a negative impact on OS (p = 0.05) and had a significant effect on DFS (0.002) while intermediate grade had no significant influence on survival. The surgical treatment had a positive impact on both OS (p = 0.00005) and DFS (p = 0.0005). Postoperative radiotherapy had no impact in multivariate analysis. CONCLUSION: Advanced clinical stage, high grade tumor, high age, the impossibility of carrying out a complete surgical resection, and diabetes are the main prognostic factors in this prospective series of patients with MEC. Such findings open new research perspectives on the influence of these components on initial patient care.

Gold nanoclusters as a contrast agent for image-guided surgery of head and neck tumors.

With the objective to evaluate the potential of ultra-small gold (Au) nanoclusters (NCs) for optical image-guided surgery, we synthesized and characterized AuNCs shelled by zwitterionic or pegylated ligands. The toxicity of the different AuNCs was evaluated on the Head and Neck Squamous Cell Carcinoma (HNSCC) CAL-33 and SQ20B cell lines in vitro. The safer AuNCs were administrated intravenously to mice for the determination of the pharmacokinetic properties. Biodistributions were performed on orthotopic CAL-33 HNSCC-bearing mice. Finally, the AuNCs were used for image-guided surgery, allowing the increase of the survival time vs. control animals, and the number of animals without any local recurrence.

Cricoarytenoid Joint Ankylosis: Classification and Transoral Laser Microsurgical Treatment.

OBJECTIVE: This study aimed to describe a graduated approach for effective transoral mobilization of cricoarytenoid joint ankylosis (CJA) in the context of the Bogdasarian system of classifying posterior glottic web-based stenosis (PGWS). STUDY DESIGN: This is a retrospective cohort study through data from medical records and operative notes. A consecutive series of 23 patients who underwent reconstructive transoral laser microsurgery for PGWS with a significant degree of CJA (Bogdasarian grade III-IV) was included in the study. METHODS: Techniques necessary to remobilize their cricoarytenoid joints were reviewed in the context of the extent of scar tissue found. RESULTS: Arytenoids with CJA were successfully mobilized by resection of the fused portion of the cricoid and arytenoid cartilages achieving respiratory improvements as well as decannulation of tracheostomy-dependent patients. The majority (83%) of patient's voices improved. All patients tolerated a full diet after the procedures. Cases with Bogdasarian grade III PGWS with minor unilateral fixation should be classified as IIIa. If the fixation is severe, the case should be classified as a grade IIIb. Grade IVa would indicate that both sides were mildly to moderately ankylosed, and grade IVb involves ankylosis of both joints with subtotal or complete fusion of at least one; it presents the greatest surgical challenge. CONCLUSION: We provided effective transoral techniques for the re-mobilization of cricoarytenoid joint, along with a classification of CJA that aims to standardize the severity of disease in the context of the existing and widely accepted Bogdasarian scale.

Evaluation of the theranostic properties of gadolinium-based nanoparticles for head and neck cancer.

BACKGROUND: The aim of the study was to evaluate the benefits of the combination of Gadolinium-based nanoparticles AGuIX and radiotherapy on the recurrence free survival after tumor resection in a head and neck animal orthotopic model. METHODS: Human head and neck CAL33 orthotopic tumors were implanted in female NMRI nude mice. The biodistribution of AGuIX was studied by fluorescence imaging. Tumor resection was performed 19 days after tumor implantation. Radiotherapy was performed 23 days after resection (10 Gy), 1 hour after AGuIX IV injection. RESULTS: After systemic administration, AGuIX passively accumulated in the orthotopic tumors. After tumor surgery, the combination of AGuIX with radiotherapy significantly improved the recurrence free survival and the median survival time (196 days) compared to irradiated only mice (75 days). CONCLUSION: This study demonstrated the improvement of the recurrence free survival following combination of AGuIX injection with radiotherapy after Head and neck tumor resection.

Facial nerve tractography: A new tool for the detection of perineural spread in parotid cancers.

OBJECTIVES: To determine whether facial nerve MR tractography is useful in detecting PeriNeural Spread in parotid cancers. METHODS: Forty-five participants were enrolled. Thirty patients with surgically managed parotid tumors (15 malignant, 15 benign) were compared with 15 healthy volunteers. All of them had undergone 3T-MRI with diffusion acquisition and post-processing constrained spherical deconvolution-based tractography. Parameters of diffusion-weighted sequences were b-value 1,000 s/mm2, 32 directions. Two radiologists performed a blinded visual reading of tractographic maps and graded the facial nerve average pathlength and fractional anisotropy (FA). We also compared diagnostic accuracy of tractography with morphological MRI sequences to detect PeriNeural Spread. Non-parametric methods were used. RESULTS: Average pathlength was significantly higher in cases with PeriNeural Spread (39.86 mm [Quartile1: 36.27; Quartile3: 51.19]) versus cases without (16.23 mm [12.90; 24.90]), p<0.001. The threshold above which there was a significant association with PeriNeural Spread was set at 27.36 mm (Se: 100%; Sp: 84%; AUC: 0.96, 95% CI 0.904-1). There were no significant differences in FA between groups. Tractography map visual analyses directly displayed PeriNeural Spread in distal neural ramifications with sensitivity of 75%, versus 50% using morphological sequences. CONCLUSIONS: Tractography could be used to identify facial nerve PeriNeural Spread by parotid cancers. KEY POINTS: • Tractography could detect facial nerve PeriNeural Spread in parotid cancers. • The average pathlength parameter is increased in case of PeriNeural Spread. • Tractography could map PeriNeural Spread more precisely than conventional imaging.

Malnutrition and refeeding syndrome prevention in head and neck cancer patients: from theory to clinical application.

PURPOSE: The goal of this review is to raise awareness about refeeding syndrome (RFS) and to give a comprehensive presentation of recent guidelines and latest scientific data about nutritional management among head and neck cancer (HNC) patients while focusing on RFS prevention. METHODS: A review of literature for nutritional assessment and RFS management was conducted. Electronic searches of Medline, Cochrane, PubMed and Embase databases for articles published in peer-reviewed journals were conducted from February to September 2017 using the keywords: "nutrition assessment", "head and neck cancer", "refeeding syndrome" and "guidelines". Articles, reviews, book references as well as national and international guidelines in English and French were included. RESULTS: The prevalence of malnutrition is high in HNC patients and a large number of them will need artificial nutritional support or refeeding intervention. RFS is characterized by fluid and electrolyte imbalance associated with clinical manifestations induced by rapid refeeding after a period of malnutrition or starvation. Regarding risk factors for malnutrition and RFS, HNC patients are particularly vulnerable. However, RFS remains unrecognized among head and neck surgeons and medical teams. Practical data are summarized to help organizing nutritional assessment and refeeding interventions. It also summarizes preventive measures to reduce RFS incidence and morbidity in HNC population. CONCLUSION: Nutritional assessment and early refeeding interventions are crucial for HNC patients care. As prevention is the key for RFS management, early identification of patients with high risks is crucial and successful nutritional management requires a multidisciplinary approach.

Spontaneous Cerebrospinal Fluid Rhinorrhea: Association with Body Weight and Imaging Data.

Objective Spontaneous cerebrospinal fluid rhinorrhea (SCSFR) might be the only clinical manifestation of idiopathic intracranial hypertension (IIH), which has been historically related to overweight. Our goal was to search for an association between SCSFR and increased body weight on the one hand and SCSFR and imaging findings suggestive of IIH on the other hand. Materials and Methods We retrospectively collected clinical and radiological data of patients operated on endoscopically for SCSFR in our institution from 1993 to 2013. Analyzed factors were body mass index (BMI), extended sphenoid sinus pneumatization on computed tomography, and empty sella and distention of the optic nerve sheath on magnetic resonance imaging. Results There were 15 patients: 8 females/7 males; mean age 50 years. Primary surgical success rate was 86.7%. Regarding body weight, 80% were overweight (BMI ≥ 25) versus 32% in the French general population ( p < 0.001). Among patients with SCSFR, 20% were obese (BMI ≥ 30) versus 15% in French individuals without SCSFR ( p = 0.483). Increased pneumatization of sphenoid sinuses was observed in 92.9 versus 27.5% in the general population ( p < 0.0001). Empty sella was found in 46.2 versus 3% in the general population ( p < 0.00001). Dilation of the optic nerve sheath was observed in 46.2 versus 15% in the general population ( p < 0.01). Conclusion We found statistically significant associations between SCSFR and overweight, increased pneumatization of sphenoid sinuses, empty sella, and dilation of optic nerve sheath, but not with obesity, which did not have any additional impact of CSF leak than did overweight.

Reconstructive transoral laser microsurgery for posterior glottic web with stenosis.

OBJECTIVES/HYPOTHESIS: To demonstrate that reconstructive transoral laser microsurgical (R-TLM) techniques can be used for the treatment of symptomatic laryngeal posterior glottic web-based stenosis (PGWS) in a large cohort of patients utilizing a postcricoid mucosal advancement flap (PCMAF). STUDY DESIGN: Retrospective cohort review. METHODS: A consecutive series of patients with PGWS who underwent R-TLM using a PCMAF were reviewed for outcomes. After laser excision of the PGWS scar and mobilization of fixed cricoarytenoid joints, a PCMAF was raised using microinstruments and a scanning free-beam CO2 laser. The flap was advanced and attached over the scar bed using a technique with multiple novel features that make it easy to adopt. RESULTS: Fifty-two patients were treated. Of the cases, 42.3% had a tracheostomy at presentation with grade II to IV PGWS, and 46% of cases had grade III to IV PGWS. In all cases, R-TLM was the only treatment approach. No open reconstructions were performed. No airway stents were used. Patients without tracheostomy, regardless of the grade of stenosis, did not require a tracheostomy to undergo this operation. All tracheostomy patients were successfully decannulated. All patients without a tracheostomy had significant improvement of their respiratory symptoms on the Dyspnea Index (mean Δ = 14.75, P value <.01). CONCLUSIONS: RTLM using the PCMAF is a feasible, safe, and effective alternative to open approaches for airway reconstruction for PGWS. This novel transoral technique includes a much simpler endoscopic suturing alternative to knot tying among other new features. It is reproducible and reliable for laryngologists familiar with laryngeal microsurgery. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:685-690, 2017.

Schizophyllum commune: an emergent or misdiagnosed fungal pathogen in rhinology?

Schizophyllum commune is a common basidiomycete fungus that is rarely involved in human disease. The medical records of patients operated on for fungal rhinosinusitis (FRS) in two University Hospitals between 2012 and 2014 were reviewed. Within the two-year survey, six female, and notably no male, patients were diagnosed with S. commune rhinosinusitis. Mean age was 44.6 years at diagnosis (30 to 68 years). Mean time between onset of symptoms and diagnosis was 8.5 months (2 to 12 months). All six patients were immunocompetent and had no particular host factor for FRS. S. commune was identified using MALDI-TOF mass spectrometry and identifications were confirmed via DNA sequence analysis. Chronic invasive fungal rhinosinusitis was diagnosed in three of our six patients. Based on histological findings, antifungal treatment was delivered in association with surgery. The basidiomycete fungus S. commune is an emerging cause of rhinosinusitis probably as a direct consequence of the recent technological progress in fungal identification methods (DNA sequencing and MALDI-TOF mass spectrometry).

Near-infrared fluorescence imaging-guided surgery improves recurrence-free survival rate in novel orthotopic animal model of head and neck squamous cell carcinoma.

BACKGROUND: Appropriate animal models are required to test novel therapeutics for head and neck squamous cell carcinoma (HNSCC) such as near-infrared (NIR) imaging-guided surgery. METHODS: We developed an optimized animal model of orthotopic HNSCC (in female athymic NMRI (Naval Medical Research Institute) nude mice) with a prolonged survival time. Resection of the orthotopic tumors was performed 30 days after implantation with or without the aid of a miniaturized clinical grade NIR optical imaging device, after systemic administration of a fluorescent RGD-based probe that targets αv ß3 integrin. RESULTS: NIR optical imaging-guided surgery increased the recurrence-free survival rate by 50% through the detection of fluorescent cancer residues as small as 185 µm; these fragments could remain unidentified if resection was performed exclusively under unaided visual guidance. CONCLUSION: NIR optical imaging-guided surgery showed an improved HNSCC tumor resection quality in our optimized orthotopic animal model. © 2015 Wiley Periodicals, Inc. Head Neck 38: E246-E255, 2016.

Role of near-infrared fluorescence imaging in head and neck cancer surgery: from animal models to humans.

Complete resection of head and neck cancers with negative surgical margins improves the prognosis of the disease and decreases the recurrence rate. Near-infrared fluorescence-guided surgery of head and neck cancer is a rapidly evolving field that represents an invaluable tool for tumor detection and resection. Here, we present a literature review of the principles of near-infrared fluorescence imaging and its use in head and neck cancer surgery. We discuss important studies in both animal models and humans that have been carried out up to this point. We also outline the important fluorescent molecules and devices used in head and neck fluorescence imaging-guided surgery. Although near-infrared fluorescence-guided surgery for head and neck cancers showed efficacy in animal models, its use in humans is limited by the small number of fluorescent probes that are approved for clinical use. However, it is considered as a novel surgical aid that helps delineate tumor margins preoperatively and could spare patients from the added morbidity that is associated with additional surgery or chemoradiation. In addition, it is a useful tool to detect sentinel lymph nodes as well as metastatic lymph nodes.

Rare case study of a primary carcinoma of the petrous bone and a brief literature review.

BACKGROUND: Temporal bone carcinoma is an aggressive tumor with multiple unconfirmed risk factors. METHODS AND RESULTS: Herein, we present a rare case of a primary petrous bone carcinoma in a female patient (65 years old) with an irrelevant medical history. She presented a postauricular swelling that revealed a multilocular osteolytic cystic lesion of the mastoid portion of the temporal bone on the CT scan. The patient underwent resection of the lesion and pathological analysis revealed moderately to well-differentiated squamous cell carcinoma. Postoperative radiotherapy was carried out. Until the present time, the patient shows complete remission on regular clinical and radiological follow-up. CONCLUSION: Although no widely accepted strategy for managing temporal bone tumors exists, a review of the literature showed that surgery with or without radiotherapy is the treatment of choice. Preoperative assessment and accurate staging are vital in ensuring that the treatment is adequate for each disease stage.

Lemierre syndrome: study of 11 cases and literature review.

BACKGROUND: Lemierre syndrome is a rare but serious illness that associates throat infection and thrombosis of the internal jugular vein (IJV) or one of its tributaries with subsequent distant septic emboli. The purpose of our study was to review the pathogenesis, clinical presentation, and treatment of this disease. METHODS: Patients with confirmed Lemierre syndrome were included in our retrospective monocentric study. All patients had bacteriologic analyses as well as radiologic imaging. RESULTS: There were 11 patients in our study (from 1998-2012). Fusobacterium necrophorum was responsible for the infection in 45% of cases. Surgical drainage of pharyngeal, cervical, or mediastinal abscesses was carried out in 8 cases. All patients received broad-spectrum antibiotics. Six patients were admitted to the intensive care unit (ICU). One patient (9%) died. CONCLUSION: Treatment with broad-spectrum antibiotics is the primary choice of treatment of Lemierre syndrome. Surgery is indicated in case of abscess formation.

Sphenoid sinus fungus ball.

The objective of this retrospective study is to present a large series of patients with sphenoid sinus fungus ball (SSFB) and describe clinical manifestations, diagnostic workup, surgical treatment, and eventual complications of this disease. We included patients operated on for this disease over a 14-year period. All patients benefited from mid-to-long-term follow-up. There were 28 patients (18 females, 10 males, mean age 64 years). Main symptoms were posterior rhinorrhea and headache. Less common symptoms were alteration of vision or ocular mobility and cacosmia. Preoperative diagnosis was based on nasal endoscopy and CT scanning. MRI was performed in case of suspicion of a tumor, an intraorbital or intracranial invasion. Treatment consisted in endoscopic transnasal or transethmoidal sphenoidotomy with removal of the fungus ball. Specimens were sent to pathology and mycology to confirm diagnosis. Postoperative complications consisted of two cases of epistaxis and two other cases of bacterial superinfection of the operated sphenoid cavity. No recurrence of the fungus ball was seen after a mean follow-up of 13 months. To conclude, SSFB is a relatively uncommon entity, usually due to Aspergillus infection. Although not invasive, if left untreated, it can lead to long-term serious complications. Preoperative nasal endoscopic examination and CT scan are the standard tools for diagnosis. Endoscopic sphenoidotomy with removal of the fungus ball is the current treatment because it has proven effective and has a low morbidity and recurrence rate.

Descriptive epidemiology of upper aerodigestive tract cancers in France: incidence over 1980-2005 and projection to 2010.

Over the 1998-2002 period, some French Départements have been shown to have the world's highest incidence of upper aerodigestive tract (UADT) cancers in men. The objectives were to describe the changes in UADT cancer incidence in France over the 1980-2005 period, present projections for 2010, and describe the anatomical and histological characteristics of these tumours. The trend of cancer-incidence over 1980-2005 and projection up to 2010 were obtained using age-period-cohort models (data from eleven cancer registries) and incidence/mortality ratios in the area covered by these registries. The description of UADT cancers by anatomical and histological characteristics concerned data collected between 1980 and 2004 in eleven cancer registries. In men, cancer incidence decreased in all cancer sites and the world-standardized incidence rates decreased by 42.9% for lip-oral cavity-pharynx (LOCP) cancers and 50.4% for larynx cancer. In women, the world-standardized incidence rates increased by 48.6% for LOCP cancers and 66.7% for larynx cancer. Incidence increased the most for oropharynx, palate, and hypopharynx cancers. Incidence analysis by one-year cohorts revealed a progressive shift of the incidence peak towards younger and younger generations, with no change as yet in the mean age at diagnosis. In France, the incidence of these cancers is still higher than in other European and North American countries. This urges actions towards reducing the major risk factors for those cancers, namely alcohol and tobacco consumption, especially among young people, and reducing exposure to risk factors due to social inequalities.