A Spindle Cell Tumour that Took us for a Spin!: A Case Report and Short Review of Management of Head and Neck Desmoid Fibromatosis.

Head and neck desmoid fibromatosis is a rare type of benign but locally aggressive tumour that has varied presentations and is difficult to manage with a high chance of causing morbidity to the patient. This report highlights the importance of proper diagnosis and surgical planning before embarking on a strenuous surgical resection.

Ultrasound-guided wire localisation: a GPS for hidden head and neck tumours? A case series.

Objectives: Ultrasound-guided wire (USGW) localisation for small non-palpable tumours before a revision head and neck surgery is an attractive pre-operative option to facilitate tumour identification and decrease potential complications. We describe five cases of pre-operative USGW localisation of non-palpable head and neck lesions to facilitate surgical localisation and resection. Methods: All patients undergoing pre-operative USGW localisation for non-palpable tumours of the head and neck region at London Health and Sciences Center, London, Ontario, Canada, were included. All the USGW localisations were performed by the same interventional radiologist, and the surgeries were performed by fellowship trained head and neck surgeons. Results: Five patients were included. All patients were undergoing revision surgery for recurrent or persistent disease. All successfully underwent a pre-operative USGW localisation of the non-palpable lesion before revision surgery. All lesions were localised intra-operatively with no peri-operative complications. Conclusions: USGW localisation is a safe and effective pre-operative technique for the identification of small non-palpable head and neck tumours.

Regulation of epigenetic modifications in the head and neck tumour microenvironment.

Head and neck tumours are common malignancies that are associated with high mortality. The low rate of early diagnosis and the high rates of local recurrence and distant metastasis are the main reasons for treatment failure. Recent studies have established that the tumour microenvironment (TME) can affect the proliferation and metastasis of head and neck tumours via several mechanisms, including altered expressions of certain genes and cytokines. Increasing evidence has shown that epigenetic modifications, such as DNA methylation, histone modification, RNA modification, and non-coding RNAs, can regulate the head and neck TME and thereby influence tumour development. Epigenetic modifications can regulate the expression of different genes and subsequently alter the TME to affect the progression of head and neck tumours. In addition, the cell components in the TME are regulated by epigenetic modifications, which, in turn, affect the behaviour of head and neck tumour cells. In this review, we have discussed the functions of epigenetic modifications in the head and neck TME. We have further examined the roles of such modifications in the malignancy and metastasis of head and neck tumours.

Factors associated with diagnosis delay in head and neck cancer.

INTRODUCTION AND OBJECTIVES: Tumour stage is an important prognostic factor in head and neck tumours. Many tumours are diagnosed in advanced stages despite almost universal healthcare and their being symptomatic. This paper seeks to determine the diagnostic delay in head and neck tumours in our health department, to analyse factors associated with delay and if it is associated with diagnosis in advanced stages. METHODS: Retrospective study of 137 patients with head and neck cancer diagnosed from 2016-2018. Patient delay, delay in primary health care, delay in secondary health care, diagnostic delay and possible associated factors (smoking, location, stage, …) were evaluated. RESULTS: Many patients (44.5%) were diagnosed in advanced stages. The median patient delay was 30 days. The median referral to otorhinolaryngology was 3.5 days. If the referral was made by another specialist (p = .008), the patients were under previous treatment (P=.000) and the tumours were in initial stages (P=.038) this delay was greater. The median from the first visit to otorhinolaryngology was 15 days, higher in regular referrals (43%) (P=.000). The median diagnostic delay was 12 days, higher in surgical biopsies (P=.000). The median professional delay was 58.5 days and total delay was 118.5 days. CONCLUSIONS: Many head and neck tumours are diagnosed in advanced stages. A relationship was not found between diagnosis in advanced stages and diagnostic delay. However, steps must be taken to reduce these excessive delays.

Factores asociados a la demora diagnóstica en tumores de cabeza y cuello / Factors associated with diagnosis delay in head and neck cancer

Introducción y objetivos: El estadio tumoral al diagnóstico es clave en el pronóstico del cáncer de cabeza y cuello. Pese a un sistema sanitario casi universal y ser tumores generalmente sintomáticos, una gran proporción de tumores son diagnosticados en estadios avanzados. El objetivo es conocer el tiempo que se tarda en diagnosticar los tumores de cabeza y cuello en nuestro departamento de salud, analizar si existen factores asociados a su retraso y si este se asocia al diagnóstico en estadios avanzados. Métodos: Estudio retrospectivo de 137 pacientes con cáncer de cabeza y cuello diagnosticados de 2016-2018. Se evaluó la demora del paciente en la búsqueda de atención médica, en la prestación de la atención y en la obtención del diagnóstico, así como la existencia de factores asociados (tabaquismo, localización, estadio, etc.). Resultados: El 44,5% de los pacientes se diagnosticaron en estadios avanzados. Los pacientes tardaron en consultar una mediana de 30 días desde el inicio de los síntomas. Se tardó en derivar a Otorrinolaringología una mediana de 3,5 días. Se evidenció mayor retraso cuando la derivación la hacía otro especialista (p=0,008), si recibieron tratamiento previo (antibiótico, antiinflamatorios no esteroideos, etc.) (p=0,000) y en tumores en estadios iniciales (p=0,038). En la consulta de Otorrinolaringología fueron valorados 15 días después de la derivación. Este tiempo fue mayor en el 43% de los tumores que fueron remitidos de forma ordinaria (p=0,000). Se obtuvo el diagnóstico en 12 días desde la primera visita a Otorrinolaringología, mayor cuando se tomó la biopsia en quirófano (p=0,000). La mediana de retraso médico fue de 58,5 días y el retraso total 118,5 días. Conclusiones: Muchos tumores de cabeza y cuello siguen diagnosticándose en estadios avanzados. No se ha encontrado relación entre el diagnóstico en estadios avanzados y la demora en el diagnóstico. Aun así, es necesario adoptar medidas para disminuir estas excesivas demoras. (AU)

Introduction and objectives: Tumour stage is an important prognostic factor in head and neck tumours. Many tumours are diagnosed in advanced stages despite almost universal healthcare and their being symptomatic. This paper seeks to determine the diagnostic delay in head and neck tumours in our health department, to analyse factors associated with delay and if it is associated with diagnosis in advanced stages. Methods: Retrospective study of 137 patients with head and neck cancer diagnosed from 2016-2018. Patient delay, delay in primary health care, delay in secondary health care, diagnostic delay and possible associated factors (smoking, location, stage, …) were evaluated. Results: Many patients (44.5%) were diagnosed in advanced stages. The median patient delay was 30 days. The median referral to otorhinolaryngology was 3.5 days. If the referral was made by another specialist (p=.008), the patients were under previous treatment (P=.000) and the tumours were in initial stages (P=.038) this delay was greater. The median from the first visit to otorhinolaryngology was 15 days, higher in regular referrals (43%) (P=.000). The median diagnostic delay was 12 days, higher in surgical biopsies (P=.000). The median professional delay was 58.5 days and total delay was 118.5 days. Conclusions: Many head and neck tumours are diagnosed in advanced stages. A relationship was not found between diagnosis in advanced stages and diagnostic delay. However, steps must be taken to reduce these excessive delays. (AU)

Management and work-up procedures of patients with head and neck malignancies treated by radiation.

Radiotherapy alone or in association with systemic treatment plays a major role in the treatment of head and neck tumours, either as a primary treatment or as a postoperative modality. The management of these tumours is multidisciplinary, requiring particular care at every treatment step. We present the update of the recommendations of the French Society of Radiation Oncology on the radiotherapy of head and neck tumours from the imaging work-up needed for optimal selection of treatment volume, to optimization of the dose distribution and delivery.

Kimura Disease: A Rare Cause of Head and Neck Tumour in Children- A Case Report.

BACKGROUND: Kimura disease is a rare inflammatory condition classically manifested as painless subcutaneous nodules in the head and neck region and associated with regional cervical lymphadenopathy and salivary gland involvement. OBJECTIVE: The purpose of this report is to illustrate the diagnostic difficulty due to its rarity and non-specific clinical presentation. CASE PRESENTATION: We present a case of Kimura disease with bilateral eyelid swelling, parotid involvement, and cervical lymphadenopathy in a young boy. Computed Tomography (CT) showed ill- defined enhancing masses in both upper eyelids with lacrimal gland infiltration, multiple nodular lesions in both parotid glands, and bilateral enlargement of cervical nodes. After several multidisciplinary discussions and trials of medications, Kimura's disease was finally confirmed by histology examination. CONCLUSION: In conclusion, a rare disease such as Kimura must be considered earlier if the patient is not responding to the treatment.

Heterogeneity of Cancer Stem Cells in Tumorigenesis, Metastasis, and Resistance to Antineoplastic Treatment of Head and Neck Tumours.

The discovery of a small subset of cancer cells with self-renewal properties that can give rise to phenotypically diverse tumour populations has shifted our understanding of cancer biology. Targeting cancer stem cells (CSCs) is becoming a promising therapeutic strategy in various malignancies, including head and neck squamous cell carcinoma (HNSCC). Diverse sub-populations of head and neck cancer stem cells (HNCSCs) have been identified previously using CSC specific markers, the most common being CD44, Aldehyde Dehydrogenase 1 (ALDH1), and CD133, or by side population assays. Interestingly, distinct HNCSC subsets play different roles in the generation and progression of tumours. This article aims to review the evidence for a role of specific CSCs in HNSCC tumorigenesis, invasion, and metastasis, together with resistance to treatment.

Novel modified patient immobilisation device with an integrated coil support system for MR-guided online adaptive radiotherapy in the management of brain and head-and-neck tumours.

Magnetic resonance imaging (MR)-guided online adaptive radiotherapy is a promising technique in the field of radiation oncology providing excellent visualisation of soft-tissues, and allowing for online plan adaptation and tumour tracking. In order to facilitate the accurate dose delivery to the target volume while sparing healthy surrounding normal tissue in the brain or head-and-neck (H&N) region, precise patient immobilisation with good image quality is pertinent. Herein, we present a customised thermoplastic mask holder with an integrated anterior MR receiver coil support system for MR-guided online adaptive radiotherapy in the brain and head-and-neck region. The approved medical product was developed by Innovative Technologie Voelp (IT-V), Innsbruck, Austria. MR image uniformity measurements demonstrated improved image uniformity at the expense of decreased signal-to-noise ratio due to a more defined and larger distance between the anterior receiver coil and the phantom or patient. This integrated coil support system represents a practical solution facilitating stable and reproducible anterior coil placement while maintaining the thermoplastic mask holder functionality, a widely established immobilisation technique.

Factors associated with diagnosis delay in head and neck cancer. / Factores asociados a la demora diagnóstica en tumores de cabeza y cuello.

INTRODUCTION AND OBJECTIVES: Tumour stage is an important prognostic factor in head and neck tumours. Many tumours are diagnosed in advanced stages despite almost universal healthcare and their being symptomatic. This paper seeks to determine the diagnostic delay in head and neck tumours in our health department, to analyse factors associated with delay and if it is associated with diagnosis in advanced stages. METHODS: Retrospective study of 137 patients with head and neck cancer diagnosed from 2016-2018. Patient delay, delay in primary health care, delay in secondary health care, diagnostic delay and possible associated factors (smoking, location, stage, …) were evaluated. RESULTS: Many patients (44.5%) were diagnosed in advanced stages. The median patient delay was 30 days. The median referral to otorhinolaryngology was 3.5 days. If the referral was made by another specialist (p=.008), the patients were under previous treatment (P=.000) and the tumours were in initial stages (P=.038) this delay was greater. The median from the first visit to otorhinolaryngology was 15 days, higher in regular referrals (43%) (P=.000). The median diagnostic delay was 12 days, higher in surgical biopsies (P=.000). The median professional delay was 58.5 days and total delay was 118.5 days. CONCLUSIONS: Many head and neck tumours are diagnosed in advanced stages. A relationship was not found between diagnosis in advanced stages and diagnostic delay. However, steps must be taken to reduce these excessive delays.

Difficult airways: a 3D printing study with virtual fibreoptic endoscopy.

Head and neck cancer patients present unique airway challenges, and oropharyngeal, laryngeal, and hypopharyngeal tumours considerably distort and narrow the anatomy of the airway. We describe the use of 3D augmented reality software combined with 3D printed models to assess the anatomy of difficult airways and to assist in the formulation of the most optimal airway management strategy in such patients. The reported patients had computed tomograms (CT) of the neck prior to their anaesthetic and surgical management. DICOM files of the respective scans were imported to 3D rendering software (OsiriX, Pixmeo). We constructed volume rendered models for initial assessment of the airway then generated serial surface rendered models to create a virtual endoscopic path of the airway to simulate the fibreoptic approach. To further facilitate the study of difficult airways we have subsequently printed 3D models of those that were most difficult using rapid prototyping. Head and neck tumours significantly distort the airway. Thorough study of the relevant anatomy prior to airway management for operating reasons enhances communication between the surgeon and anaesthetist, and aids selection of the most appropriate intubation approach. In conclusion, this paper highlights a useful and novel pre-assessment strategy that allows a virtual, visual, 3-dimensional assessment of the airway anatomy combined with 3D modelling and 3D printing. This enables the airway specialist, anaesthetist, and head and neck surgeon to anticipate any critical steps and adjust the plan accordingly.

A Comparison of 18F-FDG-PET/MRI and 18F-FDG-PET/CT in the Cancer Staging of Locoregional Lymph Nodes.

AIM: The aim of the study was to evaluate the yields of 2-deoxy-2-[18F]-fluoro-D-glucose positron-emission tomography/magnetic resonance imaging (18F-FDG-PET/MRI) and 18F-FDG-PET/computed tomography (CT) for the detection of metastatic involvement of locoregional neck nodes in patients with head and neck malignancy. PATIENTS AND METHODS: A total of 90 patients (66 men and 24 women) met the inclusion criteria. Preoperative staging was performed: 53 examinations using PET/CT and 37 using PET/MRI. RESULTS: For PET/MRI, the study demonstrated a sensitivity of 89%, specificity of 64%, positive predictive value of 85%, and negative predictive value of 70%; PET/CT had a sensitivity of 95%, specificity 47%, positive predictive value of 82%, and negative predictive value of 78%. CONCLUSION: Both methods have a high yield in N-clinical staging with statistically insignificant differences. We assume PET/MRI to be the first-choice method in organ-targeted examinations, for pediatric patients and repeat examinations. In cases of MRI contraindication, PET/CT can be used with no impact on the quality of care.

Transmandibular Approach in Head and Neck Oncological Surgery.

Objective: To evaluate the advantages and disadvantages of the transmandibular approach to the posterior area of the maxilla, oropharyngeal region and the hypopharynx in head and neck surgery. Methods: A series of 42 patients who underwent a lip-split mandibulotomy procedure to access malignant tumours affecting deep areas of the head and neck region between 2008 and 2018 in the Department of Oral and Maxillofacial Surgery at the Ramón y Cajal University Hospital (Madrid, Spain) were retrospectively reviewed. The diagnosis and operations data of the patients were collected and analysed. Results: Using the transmandibular approach, 42 patients were operated on to access malignant tumours located in the oropharynx (n = 23, 54.76%) including the posterior third of the tongue, tonsil and soft palate, retromolar trygone (n = 9, 21.43%), floor of the mouth (n = 3, 7.14%), skull base (n = 2, 4.76%), superior maxilla (n = 3, 7.14%) and deep lobe of the parotid gland (n = 2, 4.76%). Primary reconstruction was carried out in all cases. The most used flap reconstruction method was the forearm fasciocutaneous flap in 48.71% of cases, followed by the anterolateral thigh flap in 20.51% of cases. The remaining cases were treated with other methods. The most frequent complication was surgical wound infection. Conclusion: The transmandibular approach is a good alternative to provide access for the removal of complex tumours affecting the oropharyngeal region. This approach facilitates direct visualisation of the lesion and bleeding control, allowing tumour resection with wide margins and making primary reconstruction easier. Although further progress in the transoral robotic approach could be a good option in selected cases, given the current state of knowledge, the transmandibular approach is a good option to access tumours affecting deep areas of the oral cavity and oropharynx.

Desmoplastic ameloblastoma: a systematic review of the cases reported in the literature.

The aim of this study was to integrate the available data published on desmoplastic ameloblastoma (DA) into a comprehensive analysis of its clinical/radiographic features. As it is not clear whether the hybrid ameloblastoma has a unique biological behaviour, the clinical/radiographic features of conventional DA were compared with those of the hybrid variant. An electronic search was undertaken in May 2019. Eligibility criteria included publications with sufficient clinical/radiographic/histological information to confirm the diagnosis. A total of 128 publications reporting 285 DAs were included (246 central non-hybrid, 33 central hybrid, 6 peripheral). There was a statistically significant difference between non-hybrid and hybrid cases concerning lesion location, radiographic limits, and size. In contrast to non-hybrid tumours, which showed a similar distribution in the mandible and maxilla, hybrid tumours showed a high predominance of mandible location. Non-hybrid lesions more often showed ill-defined radiographic margins, and were smaller tumours than hybrid DAs. Enucleation with an additional therapy (either curettage or peripheral osteotomy) or resection led to a lower recurrence risk in comparison to curettage or enucleation without additional therapy. The location of the lesion and type of treatment are related to tumour recurrence. There are important differences in location and radiographic limits between hybrid and non-hybrid DAs, which support their classification as distinct lesions.

ΔNp63 promotes IGF1 signalling through IRS1 in squamous cell carcinoma.

Accumulating evidence has proved that deregulation of ΔNp63 expression plays an oncogenic role in head and neck squamous cell carcinomas (HNSCCs). Besides p63, the type 1-insulin-like growth factor (IGF) signalling pathway has been implicated in HNSCC development and progression. Most insulin/IGF1 signalling converges intracellularly onto the protein adaptor insulin receptor substrate-1 (IRS-1) that transmits signals from the receptor to downstream effectors, including the PI3K/AKT and the MAPK kinase pathways, which, ultimately, promote proliferation, invasion, and cell survival. Here we report that p63 directly controls IRS1 transcription and cellular abundance and fosters the PI3K/AKT and MAPK downstream signalling pathways. Inactivation of ΔNp63 expression indeed reduces tumour cell responsiveness to IGF1 stimulation, and inhibits the growth potential of HNSCC cells. In addition, a positive correlation was observed between p63 and IRS1 expression in human HNSCC tissue arrays and in publicly available gene expression data. Our findings indicate that aberrant expression of ΔNp63 in HNSSC may act as an oncogenic stimulus by altering the IGF signalling pathway.

Oral Cancer.

The management of oral cancer is a multidisciplinary endeavour, as each patient presents the treating clinicians with a unique set of challenges the management of which impacts on both survival and quality of life. This article focuses on the management of oral cancer. We highlight the epidemiology and risk factors for oral cancer in Australia, the various clinical presentations that occur and the staging of oral cancer. In the vast majority of cases surgery remains the mainstay of treatment. Radiation and medical oncology is usually used in an adjuvant context. Dental professionals play a critical role in many stages of management from the initial detection, to optimising pre treatment dental health and managing the short and long term sequelae of treatment. Monitouring for recurrence and the development of second primary tumours is a key role.

Cystic lesions of the parotid gland: radiologic-pathologic correlation according to the latest World Health Organization 2017 Classification of Head and Neck Tumours.

The latest World Health Organization 2017 Classification of Head and Neck Tumours includes a new chapter on tumors and tumor-like lesions of the neck and lymph nodes. Tumor-like lesions include a variety of cystic lesions of the parotid gland. Cystic lesions of the parotid gland can be divided into three groups: non-neoplastic cysts, benign tumors with macrocystic change, and malignant tumors with macrocystic change. It is important to distinguish these lesions from one another because treatment and patient management differ among the three groups. The purpose of this review is to describe the magnetic resonance imaging and scintigraphy findings and the histopathologic characteristics of each parotid gland lesion based on the latest World Health Organization 2017 Classification of Head and Neck Tumours and to summarize the key points of differential diagnosis for cystic lesions of the parotid gland.

Sclerosing odontogenic carcinoma: current diagnostic and management considerations concerning a most unusual neoplasm.

Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaws that has been listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumours (2017). The aim of this study was to analyse and interpret the existing literature on SOC in the context of a clinical case treated in the authors' department. A systematic search of the PubMed database was performed in accordance with the PRISMA guidelines, yielding nine cases of SOC reported so far. In summary, characteristic clinical and radiological features of SOC include asymptomatic swelling, location predominantly in the mandible, tumour primarily lytic in appearance, presence of cortical bone destruction, and lack of metastatic spread. Due to the rarity of the disease, close collaboration between oral/maxillofacial surgeons and pathologists is crucial to avoid misdiagnosis. With complete excision, no recurrence of SOC should be expected.

Analysis of the integration of human papillomaviruses in head and neck tumours in relation to patients' prognosis.

Integration, which leads to the disruption of the circular HPV genome, is considered as a critical, albeit not obligatory, step in carcinogenic progression. Although cervical carcinomas with extrachromosomal HPV plasmid genomes have been described, the virus is integrated in 70% of HPV16-positive cervical tumours. Limited information is available about HPV integration in head and neck tumours (HNC). In this study, we have characterised the physical status of HPV in a set of tonsillar tumour samples using different methods--the mapping of E2 integration breakpoint at the mRNA level, the 3' RACE based Amplification of Papillomavirus Oncogene Transcripts (APOT) assay and Southern blot. Furthermore, the impact of HPV integration on patients' prognosis has been evaluated in a larger set of 186 patients with head and neck cancer. Based on the analysis of E2 mRNA, HPV was integrated in the host genome in 43% of the HPV-positive samples. Extrachromosomal or mixed form was present in 57%. In fresh frozen samples, the APOT and E2 mapping results were in agreement. The results were confirmed using Southern blotting. Furthermore, the type and exact site of integration were determined. The survival analysis of 186 patients revealed HPV positivity, tumour size and lymph node positivity as factors that influence disease specific survival. However, no statistically significant difference was found in disease specific survival between patients with HPV-positive integrated vs. extrachromosomal/mixed forms of the virus.