Advances and residual knowledge gaps in the neck management of head and neck squamous cell carcinoma patients with advanced nodal disease undergoing definitive (chemo)radiotherapy for their primary.

PURPOSE: Substantial changes have been made in the neck management of patients with head and neck squamous cell carcinomas (HNSCC) in the past century. These have been fostered by changes in cancer epidemiology and technological progress in imaging, surgery, or radiotherapy, as well as disruptive concepts in oncology. We aimed to review changes in nodal management, with a focus on HNSCC patients with nodal involvement (cN+) undergoing (chemo)radiotherapy. METHODS: A narrative review was conducted to review current advances and address knowledge gaps in the multidisciplinary management of the cN+ neck in the context of (chemo)radiotherapy. RESULTS: Metastatic neck nodes are associated with poorer prognosis and poorer response to radiotherapy, and have therefore been systematically treated by surgery. Radical neck dissection (ND) has gradually evolved toward more personalized and less morbid approaches, i.e., from functional to selective ND. Omission of ND has been made feasible by use of positron-emission tomography/computed tomography to monitor the radiation response in cN+ patients. Human papillomavirus-driven oropharyngeal cancers and their cystic nodes have shown dramatically better prognosis than tobacco-related cancers, justifying a specific prognostic classification (AJCC) creation. Finally, considering the role of lymph nodes in anti-tumor immunity, de-escalation of ND and prophylactic nodal irradiation in combination are intense areas of investigation. However, the management of bulky cN3 disease remains an issue, as aggressive multidisciplinary strategies or innovative combined treatments have not yet significantly improved their prognosis. CONCLUSION: Personalized neck management is an increasingly important aspect of the overall therapeutic strategies in cN+ HNSCC.

Outcomes of comprehensive geriatric assessment and surgical management in head and neck cancers of the elderly: an observational study.

AIM: The aims of this study were to assess the survival benefit of optimal vs suboptimal management in elderly patients presenting with upper aerodigestive tract (UADT) cancer for which surgery was the standard of care, and determine if comprehensive geriatric assessment (CGA) was a prognostic factor for survival. MATERIALS AND METHODS: This single-center retrospective cohort study was conducted from January 2014 to December 2018. Included patients were aged 70 or older at the time of diagnosis, and presented with UADT cancer with a theoretical indication for curative-intent surgery according to international guidelines. RESULTS: A total of 188 patients were included, with a median age of 78 years. Treatment included surgery in 67.6% of cases and was considered optimal in 60.6% of patients. The overall 3-year survival was 55.2%, and was significantly better in case of optimal vs suboptimal treatment (74.5% vs 25.8%, p < 0.001). In univariate analysis, factors associated with a significantly improved 3-year survival included surgery (p < 0.001), age < 80 years, performance status < 2 and G8 score > 14. In multivariate analysis, CGA was associated with a better survival. CONCLUSIONS: In patients aged over 70 presenting with UADT cancer for which the standard of care is surgery, an optimal management is associated with better overall survival. Receiving a CGA seems to provide a survival benefit in patients with a G8 score ≤ 14, through an optimization of the care pathway before and after the cancer treatment.

Oncological outcomes and prognostic factors of squamous cell carcinoma of the upper gingiva and hard palate: a retrospective study.

PURPOSE: Despite sharing the same staging system as oral cavity cancers, upper gingiva and hard palate (UGHP) squamous cell carcinoma (SCC) have several features that make them a different entity. We aimed to analyze oncological outcomes and adverse prognostic factors of UGHP SCC, and assess an alternate T classification specific to UGHP SCC. METHODS: Retrospective bicentric study including all patients treated by surgery for a UGHP SCC between 2006 and 2021. RESULTS: We included 123 patients with a median age of 75 years. After a median follow-up of 45 months, the 5-year overall survival (OS), disease-free survival (DFS) and local control (LC) were 57.3%, 52.7% and 74.7%, respectively. Perineural invasion, tumor size, bone invasion, pT classification and pN classification were statistically associated with poorer OS, DFS and LC on univariate analysis. On multivariable analysis, the following variable were statistically associated with a poorer OS: past history of HN radiotherapy (p = 0.018), age > 70 years (p = 0.005), perineural invasions (p = 0.019) and bone invasion (p = 0.030). Median survivals after isolated local recurrence were 17.7 and 3 months in case of surgical and non-surgical treatment, respectively (p = 0.066). The alternate classification allowed better patient distribution among T-categories, however without improving prognostication. CONCLUSION: There is a broad variety of clinical and pathological factors influencing prognosis of SCC of the UGHP. A comprehensive knowledge of their prognostic factors may pave the way towards a specific and more appropriate classification for these tumors.

Adjuvant brachytherapy for oral squamous cell carcinomas: a single-center experience comparing low-dose and pulsed-dose-rate techniques.

OBJECTIVE: This study aims to assess the outcomes of adjuvant interstitial brachytherapy (BT) to the tumor bed for oral cavity squamous cell carcinoma (SCC), and to compare the oncological outcomes and toxicity profile of low-dose-rate (LDR) and pulsed-dose-rate (PDR) BT. DESIGN: This retrospective single-center study included all patients who underwent postoperative LDR- or PDR-BT to the tumor bed as the sole adjuvant treatment for an oral tongue or floor of the mouth SCC between January 2000 and December 2020. RESULTS: A total of 79 patients were eligible for this study. The cohort was divided into an LDR group (n = 38) and a PDR group (n = 41). The median time interval between surgery and brachytherapy was 55 days. Median delivered total dose was 55 Gy and median hospital stay was 5 days. Five patients (8.3%) experienced grade 3-4 early toxicity, 2 in the LDR group and 3 in the PDR group. Late toxicities were present in 28 patients (44.4%) and were dominated by grade 1-2 residual pain and dysesthesia, without a statistical difference between the groups. After a median follow-up of 65.1 months, 5­year local control (LC), disease-free survival (DFS), and overall survival (OS) for the whole cohort were 76.3% (95% CI = 63.4-85.1), 61.6% (95% CI = 49.0-72.0), and 71.4% (95% CI = 58.6-80.8), respectively. CONCLUSION: Adjuvant BT after excision of oral cavity SCC provides satisfactory oncological outcomes along with good tolerance. In our study, PDR-BT showed similar oncological and functional results to LDR-BT in this indication.

Rigid versus flexible endoscopy for managing ingested foreign bodies-can we improve pathways?

PURPOSE: This study aims to evaluate management pathways, outcomes and safety of rigid endoscopy (RE) and flexible endoscopy (FE) for the treatment of impacted foreign bodies of the upper gastrointestinal tract (UGIT) in adults. METHODS: Retrospective study, included all patients undergoing RE or FE for impacted UGIT foreign body over an 11-year-period. RESULTS: A total of 144 patients were included (95 FE and 49 RE). FE were performed under local anaesthetic or sedation, and RE under GA. Success rate of FE and RE were 95.8% and 95.9% respectively. During FE an intra-procedural biopsy was performed in 45/95 (47.3%); with 26/95(27.4%) identifying mucosal pathology. Complications was significantly higher in patients having RE (40.8% versus 6.3%, p = .001). CONCLUSION: FE and RE are effective for the therapeutic management of impacted UGIT foreign bodies. However, FE can be performed under LA and was associated with fewer complications, favouring FE where possible as a first line option.

A single centre's experience of 23 cases of total rhinectomy for the treatment of squamous cell carcinoma involving the nasal vestibule.

PURPOSE: This study aims to analyse the oncological outcomes of total rhinectomy (TR) for squamous cell carcinomas (SCCs) involving the nasal vestibule, and to identify prognostic factors for disease recurrence. METHODS: A retrospective single-centre study was conducted between September 2003 and February 2021 including all patients who underwent a TR for a SCC involving the nasal vestibule. RESULTS: 23 patients were included in the study. Tumours originated from the anterior septum (n = 12), vestibule (n = 8) or skin (n = 3). Six TRs (26.1%) were salvage procedures, after primary radiotherapy or partial rhinectomy. Seven patients had a concurrent neck dissection and 17 patients (73.9%) received adjuvant treatment (14 patients had radiotherapy and 3 had chemoradiotherapy). After a median follow-up of 32 months, six patients (26.1%) presented with tumour recurrence. Three patients (13%) had nodal-only recurrence. The estimated 5-year overall survival, disease-free survival and disease-specific survival were 67.5%, 66.3% and 80.7%, respectively. Positive excision margins were a predictive factor for tumour recurrence (p = 0.0401). CONCLUSION: For SCCs involving the nasal vestibule that are not amenable to limited surgical resection, TR along with adjuvant radiotherapy provide good oncological outcomes and should be considered the main treatment option.

Functional and oncological outcomes of salvage transoral robotic surgery: a comparative study.

PURPOSE: Transoral robotic surgery (TORS) as a first-line therapy has been well-documented but evidence is missing regarding salvage therapy. The aim of this study is to compare the oncological and functional outcomes of TORS as a primary and salvage therapy. METHODS: This retrospective monocentric study included 74 patients operated by a single surgeon and sorted out into two groups: primary treatment (PT) or Salvage treatment (ST) in case of previous history of radiation therapy. Patients were further stratified by tumour location: larynx and pharynx (lST vs lPT and pST vs pPT). RESULTS: Forty-eight patients were included in PT group (64.9%) and 26 in ST group (35.1%). ST patients had more frequent cTis/T1 tumours (57.7% vs 29.2%, p = 0.0164) and no clinical lymph disease (3.8% vs 37.5%, p = 0.0016). Tracheostomy was more often performed in the ST group (57.7% vs 16.7%, p = 0.0003) and the lST subgroup (88.9% vs 9.1%, p < 0.0001). Gastric feeding tube placement was more frequent in the ST group (76.9% vs 33.3%, p = 0.0003), the pST subgroup (64.7% vs 15.4%, p = 0.0009) and the lST subgroup (100% vs 54.5%, p = 0.0297). We observed a trend for more postoperative complications in the ST group (69.2% vs 47.9%, p = 0.0783). The overall survival was lower in the ST group (p = 0.0004), and in the pST subgroup (p < 0.0001). The disease-free survival rate was lower in the ST group (p = 0.0001), the pST subgroup (p = 0.0002) and the lST subgroup (p = 0.0328). CONCLUSION: This study confirms that survival and functional outcomes after salvage TORS are worse than in first line surgery.

Airway management during transoral robotic surgery for head and neck cancers: a French GETTEC group survey.

PURPOSE: The aim of the survey was to define the indications for preventive tracheostomy in transoral robotic surgery (TORS) for head and neck cancers. METHODS: From October 2019 to January 2020, an online questionnaire was e-mailed to French surgical ENT teams with considerable experience of the TORS procedure (Gettec group). A descriptive analysis of the answers was performed. RESULTS: Eighteen French surgical teams answered the questionnaire. For 77.8% of the surgical teams, a past history of radiotherapy with residual edema was an indication for prophylactic tracheostomy, and for 88.9%, > 75 mg of antiplatelet medication or anticoagulation treatment was an indication. CONCLUSION: Early preventive tracheostomy during TORS can protect airway from uncommon but potentially life-threatening complications, such as transoral hemorrhage or airway edema. We recommend it in high-risk situations, such as a past history of radiotherapy or antiplatelet therapy associated with large resections. Further studies are needed to establish evidence-based recommendations.

Contribution of narrow band imaging in delineation of laryngopharyngeal superficial cancer spread: a prospective study.

PURPOSE: The aim of this study was to assess the performance of Narrow Band Imaging (NBI) added to White Light (WL) in the delineation of laryngopharyngeal superficial cancer spread during office-based transnasal flexible endoscopy. METHODS: This bi-centric prospective study was conducted between October 2014 and December 2017. We included consecutive patients with laryngopharyngeal malignant tumors. Transnasal flexible endoscopy was performed by two endoscopists who were blinded to each other's assessments and who examined each patient independently. The first endoscopist only performed a WL examination, while the second endoscopist carried out both WL and NBI. The extent of tumor involvement was reported based on predefined anatomical sub-units. Biopsies in NBI + /WL- sub-units were subsequently performed during panendoscopy. RESULTS: Eighty-four patients were included in the study. A total of 72 NBI + /WL- sub-units were sampled in 38 patients, and 37 of the biopsies were positive (51.4%): 16 for invasive carcinoma, 17 for high-grade dysplasia/carcinoma in situ and 4 for low-grade dysplasia. Ultimately, 26.2% of patients had at least one positive biopsy in an NBI + /WL- sub-unit and, therefore, a better tumor delineation. The clinical T stage was upgraded in 4.8% of cases examined. CONCLUSION: Adding NBI to WL imaging during transnasal flexible endoscopy in patients presenting with laryngopharyngeal pre-malignant or malignant lesions improves the delineation of superficial cancer spread, thereby leading to better adapted treatments. Clinicaltrials.gov registration number: NCT02035735.

Multicentre evaluation of the interest in planned surgery for mandibular reconstruction with fibula free flap: a retrospective cohort study.

PURPOSE: The aim of this study was to determine the impact and cost-effectiveness of virtual surgical planning during fibula free flap mandibular reconstruction on peri- and postoperative data. METHODS: We conducted a retrospective cohort study from January 2012 to December 2016 in four French university centres. RESULTS: Three hundred fibula free flaps for mandibular reconstruction were performed in 294 patients. Surgeries were planned in 29.7% of cases (n = 89). There was no significant difference in the rate of negative-margins excision, median length of hospital stay, operative time, and early complications between planned and non-planned surgeries. Morphological analysis revealed a higher rate of centred occlusion in planned patients (satisfactory alignment of interincisal points: Planned 65.5% vs Non-Planned 33.3%, p = 0.006). CONCLUSION: In mandibular reconstruction by fibula free flap, the additional cost generated by virtual surgical planning does not seem to be balanced by savings resulting from a shorter operative course, a reduced hospital stay, or a reduction in postoperative complications. However, virtual surgical planning may provide a higher rate of centred occlusion. Long-term benefits should be assessed by further studies.

Circumferential pharyngectomy in laryngectomised patient and pharyngeal reconstruction by tubed free flap.

BACKGROUND: Metachronous tumour in head and neck cancer patients is a frequent situation with a generally poor prognosis, often improving in case of surgical treatment. METHOD: This article presents the surgical technique for early stage hypopharyngeal metachronous tumour resection and pharyngeal reconstruction by tubed free flap in laryngectomised patient. CONCLUSION: Selected laryngectomised patients presenting with early stage hypopharyngeal metachronous tumours can benefit from a safe carcinologic resection and reconstruction.

Cutaneous Squamous Cell Carcinoma Tumour Size is Associated with Sentinel Lymph Node Metastasis in a Cohort of 69 Patients.

Ten to fifty percent of high-risk cutaneous squamous cell carcinoma may potentially metastasize. However, the concept of sentinel lymph node biopsy remains controversial for cutaneous squamous cell carcinoma. The aim of this study was to identify prognostic factors associated with sentinel lymph node positivity. A bicentric retrospective analysis was conducted between January 2006 and January 2018. All patients undergoing sentinel lymph node biopsy for high-risk cutaneous squamous cell carcinoma were included, based on the criteria of the prognostic classification of the French Society of Dermatology. Seventy-four patients were included. Five (6.8%) procedures failed. Of the 69 patients assessed, the positive sentinel lymph node biopsy rate was 11.6% (n = 8) with a false negative rate of 5.7% (n = 4). The positivity of sentinel lymph node biopsy was associated with tumour size (p = 0.0194). Sentinel lymph node biopsy is an effective staging procedure for clinically N0 high-risk cutaneous squamous cell carcinoma, with an acceptable morbidity. To date, 2 risk factors of sentinel lymph node positivity have been identified with statistical significance: tumour size and poor tumour differentiation.

Videofluoroscopic swallowing study to detect pharyngeal leak after total (pharyngo-) laryngectomy: Retrospective assessment of a single-institution protocol.

BACKGROUND: We aimed to assess the effectiveness of a single-institution protocol of videofluoroscopic swallowing study (VFSS) for the detection of pharyngeal leak (PL) and its usefulness to mitigate evolution into subsequent pharyngocutaneous fistula (PCF) after total (pharyngo-) laryngectomy (TL). METHODS: This retrospective single-center study was conducted between February 2014 and December 2022. We included all patients who underwent TL and performed a VFSS between Day 7 and Day 14 postoperatively to detect a subclinical PL. RESULTS: Two-hundred and forty-eight patients met the inclusion criteria. Among the 186 patients (75%) with a negative VFSS, 11 patients (5.9%) developed a secondary PCF after oral intake resumption (false negative of VFSS). Among the 62 patients (25%) with a positive VFSS, the occurrence of a PCF was avoided in 59.7% of cases. CONCLUSION: This study showed a good effectiveness of VFSS in the detection of PL after TL, alongside a usefulness to mitigate evolution into subsequent PCF.

Prognostic implications of mucosal and deep margin distances according to T-status in oral tongue squamous cell carcinoma: A single-center retrospective study.

OBJECTIVE: To elucidate the prognostic implications of mucosal and deep margin distances in oral tongue squamous cell carcinoma (OTSCC), and to assess a different margin cut-off value in T1-T2 versus T3-T4 tumors. METHODS: This single-center retrospective study included 223 patients who received surgery for a primary OTSCC between January 2017 and December 2021. RESULTS: Multivariable analysis showed that deep margin distance ≥3 mm in T1-T2 tumors and ≥5 mm in T3-T4 tumors was significantly associated with better RFS and OS. Mucosal and deep margin distances were globally clinically useful for 2-year RFS prediction of T1-T2 tumors, for which deep margins seemed to have more clinical utility than mucosal margins. The influence of margin distances on 2-year RFS seemed greater for T1-T2 tumors than T3-T4 tumors. CONCLUSION: Mucosal and deep margin distances were associated with OS and RFS in OTSCC. Shorter deep margin distances may be aimed for in T1-T2 versus T3-T4 tumors.

Squamous Cell Carcinoma of the Nasal Vestibule: A Multi-Centric Observational Cohort Study.

OBJECTIVES: Squamous cell carcinoma of the nasal vestibule (NV-SCC) is a rare but challenging entity, due to the complex anatomy of the region. Consensus on the best treatment strategy is still lacking, as well as a dedicated staging system. Our aim was to analyze oncological outcomes of surgically treated patients and to investigate possible prognostic factors. METHODS: We performed a retrospective multi-centric observational study including six Academic Hospitals over a 10-year period, including only patients who underwent upfront surgery for primary NV-SCC. Patients were staged according to all currently available staging systems. The Kaplan-Meier method was used to compute overall, disease-free, and disease-specific survival. Logistic regression models were used to correlate between survival outcomes and clinical and pathological variables. RESULTS: Seventy-one patients with a median follow-up of 38 months were included in the study. Partial and total rhinectomy were the most commonly performed procedures, respectively, in 49.3% and 25.4% of cases. Neck dissection was performed on 31% of patients, and 45.1% of them underwent adjuvant radiotherapy. Three years overall, disease-specific and disease-free survival were, respectively, 86.5%, 90.3%, and 74.2%. None of the currently available staging systems were able to effectively stratify survival outcomes. Factors predicting lower overall survival on multivariate analysis were age (p = 0.021) and perineural invasion (p = 0.059), whereas disease-free survival was negatively affected by age (p = 0.033) and lymphovascular invasion (p = 0.019). CONCLUSION: Currently available staging systems cannot stratify prognosis for patients who underwent surgery for NV-SCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:2634-2645, 2024.

Transoral Robotic Surgery for Head and Neck Cancer: Advances and Residual Knowledge Gaps.

Minimally invasive surgery is a growing field in surgical oncology. After acquiring its first Food and Drug Administration approval in 2009 for T1-T2 malignancies of the oral cavity, oropharynx, and larynx, transoral robotic surgery (TORS) has gained popularity thanks to its wristed instruments and magnified three-dimensional view, enhancing surgical comfort in remote-access areas. Its indications are expanding in the treatment of head and neck cancer, i.e., resection of tumors of the larynx, hypopharynx, or parapharyngeal space. However, this expansion must remain cautious and based on high-level evidence, in order to guarantee safety and oncological outcomes which are comparable to conventional approaches. This narrative review assesses the current role of TORS in head and neck cancer from an evidence-based perspective, and then identifies what knowledge gaps remain to be addressed.

Evolution of the Incidence of Oral Cavity Cancers in the Elderly from 1990 to 2018.

OBJECTIVES: To describe the evolution of the incidence of oral cavity cancers (OCC) among elderly patients in France between 1990 and 2018 and to compare it to the incidence of other cancers sharing the same main risk factors. MATERIAL AND METHODS: The incidence of cancers in mainland France from 1990 to 2018 was estimated from incidence data observed in every cancer registry of the Francim network. Incidence was modeled by a 2-dimensional penalized spline of age and year of diagnosis, associated with a random effect corresponding to the registry. The elderly population was divided into two groups: 70-79 years old and ≥80 years old. RESULTS: There was a 72% increase in the number of OCC cases in women over 70 years of age between the periods 1990-1999 and 2010-2018. As for men, there was a stabilization in the number of cases (+2%). Over the same period, for laryngeal and hypopharyngeal cancers, there was a decrease in incidence in elderly men and an increase in elderly women, although less marked than for OCC. CONCLUSIONS: Since the 1990s, the incidence of OCC has been increasing in elderly subjects in France, particularly in women. Population aging and growth or alcohol and tobacco consumption alone do not seem to explain this increase, which is not observed in the same proportions for other upper aerodigestive tract cancer subsites sharing the same main risk factors.

Should an elective contralateral neck dissection be performed in midline-reaching squamous cell carcinomas of the oral cavity and oropharynx?

OBJECTIVE: to compare the rate of occult contralateral neck metastases (OCNM) in oral and oropharyngeal squamous cell carcinomas (SCC) reaching or crossing the midline and to identify risk factors for OCNM. MATERIALS AND METHODS: we conducted a single-center retrospective study of oral and oropharyngeal SCC with contralateral cN0 neck. The cohort was divided into a midline-reaching (MR; approaching the midline from up to 10 mm) group and a midline-crossing (MC; exceeding the midline by up to 10 mm) group. Clinical N-status was assessed by a radiologist specializing in head and neck imaging. All patients underwent contralateral elective neck dissection (END). RESULTS: A total of 98 patients were included in this study, 59 in the MR group and 39 in the MC group. OCNM were present in 17.3% of patients, 20.3% in the MR group and 12.8% in the MC group (p = 0.336). In multivariable analysis, MR/MC status as well as distance from the midline (in mm) were not identified as risk factors for OCNM. Conversely, oropharyngeal primary and clinical N-status above N1 were significantly associated with a higher risk of OCNM, with odds ratios (OR) of 3.98 (95% CI = 1.08-14.60; p = 0.037) and 3.41 (95% CI = 1.07-10.85; p = 0.038) respectively. CONCLUSION: in patients with oral and oropharyngeal SCC extending close to or beyond the midline, tumor origin and clinical N-status should carry the most weight when dictating the indications for contralateral END, rather than the midline involvement in itself.

[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.