Thyroid cancer epidemiology in Ireland from 1994 to 2019 - Rising diagnoses without mortality benefit.

BACKGROUND: The epidemiology and management of thyroid cancer has changed radically in the recent past, with rising international incidence of early-stage papillary thyroid cancer (PTC) in particular. In this paper, we review the epidemiology of thyroid cancer in Ireland. METHODS: A retrospective cohort study of National Cancer Registry of Ireland data, 1994-2019. RESULTS: Records from 4158 patients were analysed. 73 % (n = 3040) were female. The average age was 50.4 years. Patient sex did not change over time (p = 0.662), while age decreased significantly (p < 0.0001). The most common diagnoses were PTC (n = 2,905, 70 %) and follicular thyroid carcinoma (n = 549, 13 %). Incidence rose over threefold (1.8-6.2 cases/100000 person-years). The incidence of T1 PTC rose over twelvefold (0.169-2.1 cases/100000 person-years), while the incidence of stage III and IV disease did not change significantly. Five-year disease-specific survival (DSS) was 85 % and varied significantly by diagnosis - 97 % for PTC versus 5 % for anaplastic thyroid carcinoma. Survival did not change significantly over time. Male sex was a risk factor for more advanced disease (p < 0.0001) but did not independently predict overall survival except in PTC (HR 1.6, p = 0.03). The use of radioactive iodine declined markedly from 49 % to 12.5 %. RAI improved DSS for PTC patients aged over 55 years (p = 0.02) without a notable effect on survival for those under 55 years (p = 0.99). CONCLUSION: The epidemiology and management of thyroid cancer in Ireland has changed dramatically in a manner reflective of international trends.

Epidemiology and management of oral cavity squamous cell carcinoma in Ireland.

INTRODUCTION: The epidemiology and management of oral cavity cancer have changed considerably in recent decades. This study examines epidemiological and management trends in oral cavity squamous cell carcinoma (OCSCC). METHODS: A retrospective cohort study of data from the National Cancer Registry of Ireland between 1994 and 2014. RESULTS: A total of 2725 patients were identified. The most common subsites were the tongue (34 %, n = 1025), lip (19 %, n = 575), floor of mouth (FOM) (18 %, n = 550), and retromolar trigone (RMT) (6 %, n = 189). The incidence of OCSCC remained largely unchanged (3.14 cases/100000/year) during the study period. 5-year disease-specific survival (DSS) was 58.6 % overall, varying between subsites (lip 85 %, RMT 62.9 %, tongue 54.7 %, and FOM 47.3 %). DSS improved over the study period (p = 0.03), in particular for tongue primaries (p = 0.007). Primary surgery significantly improved DSS versus radiotherapy (HR 0.28, p < 0.0001). Survival of T4 disease managed surgically was superior to that of T1 disease managed with radiotherapy. In node positive patients, chemotherapy improved overall survival (HR 0.8 p = 0.038) but not DSS (HR 0.87 p = 0.215). CONCLUSION: Primary surgery remains the standard of care in the management of OCSCC. Prognosis has improved in line with an increase in the use of primary surgery in the same time frame, though the incidence remains unchanged.

Survival in an era of organ preservation: an update on laryngeal cancer in Ireland.

BACKGROUND: Laryngeal cancer epidemiology has changed in recent years, with falling incidence observed internationally. Organ preservation therapies have revolutionised management, though some patients may be unsuitable and survival was noted to fall in the 2000s. This study examines trends in laryngeal cancer in Ireland. METHODS: A retrospective cohort study of National Cancer Registry of Ireland data from 1994 to 2014. RESULTS: From a cohort of 2651, glottic disease was most common (62%, n = 1646). Incidence rose to 3.43 cases/100,000/year for 2010-2014. 5-year disease-specific survival (DSS) was 60.6% and did not change significantly over time. Overall survival (OS) for T3 disease managed with primary radiotherapy was similar to primary surgery (HR 0.98, p = 0.9). DSS for T3 disease improved with primary radiotherapy (HR 0.72, p = 0.045). CONCLUSION: Incidence of laryngeal cancer in Ireland rose despite international trends, while survival changed little. Radiotherapy improves DSS for T3 disease but does not improve OS, possibly secondary to poor organ function post-radiotherapy.

Non-conventional laryngeal malignancies: a multicentre review of management and outcomes.

PURPOSE: Non-conventional laryngeal malignancies (NSCC) often have limited published data to guide management despite individual histopathological subtypes often exhibiting heterogeneous behaviour, characteristics, and treatment responses compared to laryngeal squamous cell carcinoma (SCC). This study aimed to compare oncological outcomes with SCC, specifically disease-free survival (DFS), disease-specific survival (DSS) and overall survival (OS). Secondary objectives were to compare treatment differences and perform a state of the art review. METHODS: This was a multicentre retrospective cohort study at four tertiary head and neck centres. Survival outcomes between NSCC and SCC patients were analysed with Kaplan-Meier curves and compared by log rank testing. Univariate Cox regression analysis was performed to predict survival by histopathological subgroup, T-stage, N-stage and M-stage. RESULTS: There were no significant differences in 3-year DFS (p = 0.499), DSS (p = 0.329), OS (p = 0.360) or Kaplan Meier survival curves (DSS/OS) between SCC and overall NSCC groups. However, univariate Cox regression analysis identified "rare" histopathologies (mostly small cell carcinoma) to be predictive of less favourable OS (p = 0.035) but this result was not observed for other NSCC histopathological subgroups. N-stage (p = 0.027) and M-stage (p = 0.048) also predicted OS for NSCC malignancies. Significant differences in treatment modalities were identified with treatment of NSCC typically involving surgical resection and SCC often managed non-surgically (e.g., primary radiotherapy). CONCLUSIONS: Although overall NSCC is managed differently compared to SCC, there do not appear to be differences in survival outcomes between these groups. N-stage and M-stage appear to be more predictive of OS than histopathology than many NSCC subtypes.

High frequency and mortality of head and neck malignancy in organ transplant recipients in Ireland - A national cohort study.

BACKGROUND: Solid organ transplant recipients are recognized to carry a high burden of malignancy and frequently this cancer develops in the head and neck region. Furthermore, cancer of the head and neck post-transplant carries a significantly increased mortality. In this study, we aim to conduct a national retrospective cohort study to investigate the impact of head and neck cancer in terms of frequency and mortality in a large group of solid organ transplant recipients over a 20 year time span and compare the mortality in transplant patients to non-transplant patients with head and neck cancer. METHODS: Patients in the Republic of Ireland who underwent solid organ transplantation between 1994 and 2014 who developed post-transplant head and neck malignancy were identified from the records of two prospective, national databases (National Cancer Registry of Ireland (NCRI) and The Irish Transplant Cancer Group database) working in conjunction with each other. Incidence of head and neck malignancy post-transplant was compared with the general population by means of standardised incidence ratios (SIR). Cumulative incidence of all cause and cancer related mortality from head and neck keratinocytic was undertaken by a competing risks analysis. RESULTS: A total of 3346 solid organ transplant recipients were identified, 2382 (71.2 %) kidney, 562 (16.8 %) liver, 214 (6.4 %) cardiac and 188 (5.6 %) lung. During the period of follow up of 428 patients developed head and neck cancer, representing (12.8 %) of the population. 97 % of these patients developed keratinocytic cancers, specifically, of head and neck. The frequency of post-transplant head and neck cancer was related to the duration of immunosuppression with 14 % of patients developing cancer at 10 years and 20 % having developed at least one cancer by 15 years. 12 (3 %) patients developed non-cutaneous head and neck malignancy. 10 (0.3 %) patients died due to head and neck keratinocytic malignancy post-transplant. Competing risk analysis demonstrated that organ transplantation conferred a strong independent effect of death, compared to non-transplant patients with head and neck keratinocytes. This applied specifically for kidney (HR 4.4, 95 % CI 2.5-7.8) and heart transplants (HR 6.5, 95 % CI 2.1-19.9), and overall, across the four transplant categories (P < 0.001). The SIR of developing keratinocyte cancer varied based on primary tumor site, gender, and type of transplant organ. CONCLUSION: Transplant patients demonstrate a particularly high rate of head and neck keratinocyte cancer with a very high rate of associated mortality. Physicians should be cognizant of the increased rate of malignancy in this population and monitor for red flag signs/symptoms.

Age Alone Is Not a Barrier to Concurrent Chemoradiotherapy for Advanced Head and Neck Cancer.

BACKGROUND: Head and Neck Cancer (HNC) is associated with significant morbidity and mortality, especially when high stage disease is present. There exists a range of options for the management of locoregionally advanced HNC, though doubt remains as to the optimal strategy in the elderly population. AIMS: To evaluate the benefits imparted by concurrent chemoradiotherapy (CCRT) to the elderly population of HNC patients in Ireland. METHODS: A retrospective cohort study was conducted using 20 years of cancer registry data provided by the National Cancer Registry of Ireland. Cox multivariate regression analysis was applied to test for the benefits of CCRT in HNC. RESULTS: Survival analysis showed an overall benefit to the use of CCRT in patients with advanced disease over 70 years, particularly when used for hypopharyngeal, oropharyngeal, and laryngeal malignancy. There was a benefit to cancer-specific but not all-cause mortality in those over 75 years, and no benefit was observed to the addition of chemotherapy in those over 80 years; only 8 patients over 80 received CCRT. There was no statistically significant difference in the benefits derived by those over 70 years relative to those under 70 years. CONCLUSION: CCRT confers significant survival benefits to appropriately selected elderly HNC patients and should therefore not be withheld solely on the basis of age.

Surgical aspects and controversies in the management of medullary thyroid cancer.

Medullary thyroid cancer (MTC) accounts for only 4% of thyroid carcinomas but 15% of thyroid cancer deaths. MTC is a tumour of the calcitonin secreting parafollicular C cells in the thyroid which can occur sporadically or be hereditary in multiple endocrine neoplasias type 2 syndromes due to germline RET mutations. Sporadic forms of MTC can also be caused by mutations in the RET protooncogene. MTC commonly presents in a late stage, with 70% of patients presenting with local nodal metastasis. Currently, the only curative treatment for MTC is surgical removal. The aim of this paper is to describe the current guidelines and progressions of the surgical management of MTC and to highlight up-and-coming chemotherapies. A database literature review was completed utilizing PubMed to cumulate the extant literature, screening for most recent guidelines and publications regarding the management of MTC. Current guidelines were described by the American Thyroid Association in 2015. Controversial publications continue to present supporting evidence for varying degrees of thyroidectomy and neck dissections. Recently, researchers have been exploring non-surgical options including external beam radiotherapy and multikinase inhibitors such as vandetanib and cabozantinib for the treatment of MTC. Surgical management of MTC remains controversial and varies significantly dependent on the extent of disease. Chemotherapeutic options have undetermined effects on survival to date.

The changing face of Irish head and neck cancer epidemiology: 20 years of data.

BACKGROUND: Head and neck cancer (HNC) is associated with significant morbidity and mortality, especially when high stage disease is present. The epidemiology and prognosis of HNC has changed considerably over the last 20 years. AIMS: This study aimed to examine the epidemiological trends in HNC patients over a prolonged period in Ireland. METHODS: We conducted a retrospective cohort study using 20 years of cancer registry data provided by the National Cancer Registry of Ireland. Baseline characteristics and survival statistics were thereby generated. RESULTS: 10,148 patients were identified. There is a growing population of young (< 50 years) and very old (> 85 years) HNC patients; 48.15% of the population was elderly (> 65 years). Oral cavity (29.8%) and laryngeal cancer (28.1%) remain the most prevalent subsites, though oral cavity cancer prevalence declined from 35.9% in 1994 to 27.5% in 2014. Oropharyngeal cancer prevalence increased from 13.6 to 22.2% over the same period. Overall 5-year survival has improved significantly to 56.8% in 2010 but there remains a disparity between the elderly and adult cohorts (42.0% vs 60.7%). 5-year survival for hypopharyngeal and oropharyngeal cancers has improved from 11.8% and 33.3% to 22.2% and 44.8%, respectively, while laryngeal and oral cavity cancer survival remains approximately stable at 58.7% and 61.5%, respectively. CONCLUSION: HNC survival in Ireland has improved in line with increasing recognition of the value of multidisciplinary assessment, subspecialisation in cancer care, and targeted therapies based on tumour subsites. Survival in the elderly cohort remains poor despite increasing recognition of the challenges such cases pose.

Metastatic and locally aggressive BCC: Current treatment options.

The treatment of locally advanced and metastatic BCC presents a significant clinical challenge. Treatment options have evolved recently to include the use of hedgehog inhibitors Vismodigib and Sonidigib and immunotherapy with Cemiplimab.

Assessing the quality of life of head and neck healthcare workers during the COVID-19 pandemic-A self-reported global cross-sectional questionnaire study by the International Federation of Head and Neck Oncologic Societies.

BACKGROUND: Considering the pandemic's mode of transmission, the impact on quality of life (QOL) is likely to be exaggerated among healthcare workers (HCWs) who treat head and neck diseases (hHCWs). METHODS: A cross-sectional self-reported QOL assessment was undertaken between July and September 2020 using the World Health Organization Quality of Life instrument sent out to hHCWs. Factors that predicted a poorer QOL were identified using regression models and mediation analysis. RESULTS: Responses from 979 individuals across 53 countries were analyzed with 62.4% participation from low- and middle-income countries. The physical domain had the highest mean scores of 15 ± 2.51, while the environmental domain was the lowest (14.17 ± 2.42). Participants from low- and middle-income countries had a significantly worse physical (p < 0.001) and environmental (p < 0.001) domains, while a low coronavirus disease 2019-related mortality significantly impacted the environmental domain (p-0.034). CONCLUSION: QOL-related issues among hHCWs are a vexing problem and need intervention at an individual and systems level in all parts of the world.

The Royal College of surgeons multidisciplinary guidelines on elective tracheostomy insertion in COVID-19 ventilated patients.

BACKGROUND: The current COVID-19 pandemic has placed enormous strain on healthcare systems worldwide. Understanding of COVID-19 is rapidly evolving. Pneumonia associated with COVID-19 may lead to respiratory failure requiring mechanical ventilation. The rise in patients requiring mechanical ventilation may lead to an increase in tracheostomies being performed in patients with COVID-19. Performing tracheostomy in patients with active SARS-CoV-2 infection poses a number of challenges. METHODS: These guidelines were written following multidisciplinary agreement between Otolaryngology, Head and Neck Surgery, Respiratory Medicine and the Department of Anaesthetics and Critical Care Medicine in the Royal College of Surgeons in Ireland. A literature review was performed and a guideline for elective tracheostomy insertion in patients with COVID-19 proposed. CONCLUSION: The decision to perform tracheostomy in patients with COVID-19 should be undertaken by senior members of the multidisciplinary team. Steps should be taken to minimise risks to healthcare workers.

Management of Temporal Bone Malignancy in Ireland.

Objectives Lateral temporal bone malignancy remains a challenging rare disease. We report 17 years of multidisciplinary care of these tumors with univariate and multivariate analyses of key prognostic indicators for consideration in contemporary oncological management. Design This is a retrospective cohort study. Setting This is set at a tertiary referral center. Participants All patients presenting with histopathologically newly diagnosed cases of temporal bone malignancy between 2000 and 2017 were included. Main Outcome Measures The main outcome measures are disease-specific and recurrence-free survival rates. Results In this study, 48 cases of temporal bone malignancy were diagnosed. Median age at diagnosis was 69 years (range: 5-88). Fourteen patients were female. Squamous cell carcinoma was the predominant malignancy in 34 cases (71%). Surgical treatment was undertaken in 37 patients. Mean length of follow-up was 32 months (range: 0.7-117). Overall 5-year disease-specific survival was 52.4%, while overall 5-year recurrence-free survival was 53.5%. On univariate analysis, significantly worse survival was seen in females ( p = 0.008), those with distant metastatic disease ( p = 0.041), and in middle ear involvement ( p = 0.012) with no difference for involvement of the external auditory canal ( p = 0.98) or mastoid ( p = 0.78). Only middle ear involvement remained significant on multivariate analysis. Conclusion A wide variety of malignant pathology may present in the temporal bone. Recurrence-free survival is equivalent to international data; however, this figure is low. This emphasizes the need to treat these tumors appropriately with radical resection, where possible, at first presentation. Therefore, multidisciplinary surgical input is recommended. Middle ear involvement was a negative prognosticator for disease-specific and recurrence-free survivals.

Post-transplant malignancy in solid organ transplant recipients in Ireland, The Irish Transplant Cancer Group.

OBJECTIVE: Solid organ transplant recipients are at increased risk of cancer compared to the general population. To date, this risk in Ireland has not been investigated. We conducted a national registry study of cancer incidence following solid organ transplantation. METHODS: National centers for solid organ transplantation supplied their respective registry databases to cross-reference with episodes of malignancy from the National Cancer Registry Ireland (NCRI) between 1994 and 2014. Standardized incidence of cancer post-transplant was compared to the general population by means of standardized incidence ratios (SIRs), and between solid organ transplant types by incidence rate ratios. RESULTS: A total of 3346 solid organ transplant recipients were included in this study. Kidney transplant recipients constituted the majority of participants (71.2%), followed by liver (16.8%), heart (6.4%), and lung (5.6%) transplants. The most common cancers within the composite of all transplant recipients included the following (SIR [95% CI]): squamous and basal cell carcinoma (20.05 [17.97, 22.31] and 7.16 [6.43, 7.96], respectively), non-Hodgkin lymphoma (6.23 [4.26, 8.59]), and renal cell carcinoma (3.36 [1.96, 5.38]). CONCLUSIONS: This study reports the incidence of cancer following solid organ transplantation in Ireland. These results have significant national policy implications for surveillance, and early diagnosis in this patient group.

Variation in Nonmelanoma Skin Cancer Incidence by Treatment Modality Among Patients Receiving Multiple Kidney Transplants.

IMPORTANCE: Existing data suggest that nonmelanoma skin cancer (NMSC) is more common in renal transplant recipients than in maintenance dialysis patients. However, whether the risk of NMSC varies as the treatment modality for end-stage kidney disease (ESKD) changes between dialysis and transplantation is not well described. OBJECTIVE: To determine whether the incidence of NMSC is attenuated during periods of graft loss with a return to dialysis in those who receive multiple kidney transplants. DESIGN, SETTING, AND PARTICIPANTS: Retrospective analysis of data from recipients of kidney transplants from the Irish National Kidney Transplant Service database, linked with the Irish Cancer Registry, from 1994 to 2014. All analysis took place between January 10, 2018 and March 31, 2018. Standardized incidence ratios (SIRs) were calculated for NMSC incidence in comparison with the general population using Irish census data as the denominator. Incidence of NMSC was calculated with modality of treatment for ESKD varying over time; incidence rates and rate ratios associated with dialysis intervals were calculated using Poisson regression; and disease was defined according to International Statistical Classification of Diseases and Related Health Problems, Tenth Revision codes for cancer diagnosis. EXPOSURES: Kidney transplantation. MAIN OUTCOMES AND MEASURES: Incidence rates per 1000 patient-years and incident rate ratios of NMSC after kidney transplant. RESULTS: Data from the records of 3821 deceased or living donor kidney transplant recipients were assessed; 2399 (62.8%) male and 1422 (37.2%) female recipients; mean (SD) age at time of first data recorded, 41.9 (16.0) years. A total of 3433 recipients were included who had a functioning transplant on January 1, 1994, or received a transplant after that date up to December 31, 2014: 3215 received 1 transplant, 522 a second kidney transplant, and 84 had 3 or more kidney transplants. Periods of treatment with a functioning transplant were associated with a higher incidence of NMSC diagnosis than periods of graft failure: adjusted incidence rate ratio (aIRR), 2.19 (95% CI, 1.56-3.07), P < .001. The aIRRs of NMSC fell from 41.7 (95% CI, 39.38-44.15) per 1000 patient-years in the first transplant to 19.29 (95% CI, 13.41-27.76) in the dialysis period following the first allograft failure. Incidence similarly rose and fell following each subsequent consecutive transplant. CONCLUSIONS AND RELEVANCE: In recipients of multiple kidney transplants, while the incidence of NMSC fell during periods defined by transplant failure, there was residual elevated risk. While ascertainment bias may have contributed to the observed trends, the stagnant incidence of invasive cancer overall highlights the need for continued cancer surveillance during graft failure.

Changing Trends in the Management of Esthesioneuroblastoma: Irish and International Perspectives.

Objectives Evaluation of the changing trends in esthesioneuroblastoma in an Irish context and review of management options nationally to clarify the best current therapeutic approach by comparing with international research on this uncommon malignancy. Design Retrospective review. Setting Tertiary referral center. Participants All patients presenting with esthesioneuroblastoma in Beaumont hospital or on the National Cancer Registry of Ireland between 1994 and 2013. Main Outcome Measures Recurrence-free and overall survival. Results During the study period, 32 cases of esthesioneuroblastoma were diagnosed (0.4 per million per year). Average age at diagnosis was 57 years; however, two cases were under 20. The majority (62.5%) were male. Patients predominantly presented with epistaxis or nasal congestion (73%), while two cases were identified incidentally on radiological investigations. Twenty-seven cases underwent primary surgical management (two post neo-adjuvant treatment) with seventeen requiring bifrontal craniotomy. Twenty-four of these received postoperative radiation therapy. Overall, 5-year survival was 65%. Kadish A/B patients exhibited 100% 5-year disease-specific survival versus 54% in Kadish C/D ( p = 0.011). Hyams grade I/II patients exhibited 75% 5-year disease-specific survival versus 63% in Hyams grade III/IV ( p = 0.005). Patients treated endoscopically exhibited 100% 5-year disease-specific survival versus 51% in those treated via an open approach ( p = 0.102). Conclusions Many controversies exist in the diagnosis and management of this condition. Despite this, results from Irish data are mostly concordant with the international literature. The rising incidence of this disease may represent improved pathological recognition. An increasing number of esthesioneuroblastoma cases are being successfully treated via endoscopic surgery.

Acute airway compromise due to parathyroid tumour apoplexy: an exceptionally rare and potentially life-threatening presentation.

BACKGROUND: Spontaneous haemorrhage into a parathyroid adenoma is a rare and potentially life-threatening presentation. CASE PRESENTATION: We report the case of a 45 year old female recently diagnosed with primary hyperparathyroidism who presented with chest discomfort and acute airway compromise due to spontaneous extracapsular haemorrhage into a parathyroid adenoma. Computed tomography (CT) imaging showed a hypopharyngeal haematoma extending 10 cm into the superior mediastinum. Surgical decompression of the cyst followed by enbloc resection of the parathyroid tumour was performed after elective intubation. Calcium and parathyroid hormone (PTH) levels had fallen prior to surgery and remain normal post-operatively. CONCLUSION: Spontaneous parathyroid haemorrhage should be considered in any patient with unexplained spontaneous cervical haemorrhage, particularly if there is a history of hyperparathyroidism. Initial evaluation of such patients should include serum calcium and PTH as well as imaging.