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PURPOSE: Sinonasal mucosal melanoma (SNMM) is a rare malignancy, characterised by high (local) recurrence rates and poor survival. Comprehensive understanding of tumour etiology is currently lacking, which complicates adequate tumour treatment. Besides examining trends in incidence, this study aims to assess the association between clinical characteristics, treatment practices and patient outcomes, with the objective of establishing a baseline from which SNMM management can be enhanced. METHODS: All newly diagnosed SNMM cases in The Netherlands between 2001 and 2021 were included using data from The Netherlands Cancer Registry (NCR). RESULTS: A total of 320 patients were included. The annual incidence rate for the overall population was stable over the inclusion period with an annual percentage change (APC) of only - 0.01%. The 5-year overall survival (OS) and relative survival (RS) were 24.5 and 32.4%, respectively. Relative survival did not increase over time. The addition of adjuvant radiotherapy to surgery was not associated with a higher OS and RS compared to surgery alone. CONCLUSION: Sinonasal mucosal melanoma is a rare disease with stable incidence rates in the Netherlands between 2001 and 2021. There has been no improvement in survival over the course of the inclusion period. The study reaffirms that adjuvant radiotherapy does not seem to improve patient outcomes. Given the generally poor outcomes for SNMM patients, novel therapeutic options ought to be considered in order to improve care.
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Melanoma , Mucosa Nasal , Neoplasias dos Seios Paranasais , Sistema de Registros , Humanos , Países Baixos/epidemiologia , Melanoma/epidemiologia , Melanoma/terapia , Masculino , Feminino , Neoplasias dos Seios Paranasais/epidemiologia , Neoplasias dos Seios Paranasais/terapia , Pessoa de Meia-Idade , Idoso , Incidência , Mucosa Nasal/patologia , Adulto , Idoso de 80 Anos ou mais , Taxa de Sobrevida , Adulto Jovem , AdolescenteRESUMO
PURPOSE: Early-stage glottic cancer can be treated with radiotherapy only. Modern radiotherapy solutions allow for individualized dose distributions, hypofractionation and sparing of organs at risk. The target volume used to be the entire voice box. This series describe the oncological outcome and toxicity of individualized vocal cord-only hypofractionated radiotherapy for early stage (cT1a-T2 N0). METHODS: Retrospective cohort study with patients treated in a single center between 2014 and 2020. RESULTS: A total of 93 patients were included. Local control rate was 100% for cT1a, 97% for cT1b and 77% for cT2. Risk factor for local recurrence was smoking during radiotherapy. Laryngectomy-free survival was 90% at 5 years. Grade III or higher late toxicity was 3.7%. CONCLUSION: Vocal cord-only hypofractionated radiotherapy appears to be oncologically safe in early-stage glottic cancer. Modern, image-guided radiotherapy led to comparable results as historical series with very limited late toxicity.
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Neoplasias de Cabeça e Pescoço , Neoplasias Laríngeas , Humanos , Prega Vocal/patologia , Carcinoma de Células Escamosas de Cabeça e Pescoço/patologia , Neoplasias Laríngeas/cirurgia , Estudos Retrospectivos , Glote/cirurgia , Neoplasias de Cabeça e Pescoço/patologia , Resultado do Tratamento , Recidiva Local de Neoplasia/patologia , Estadiamento de NeoplasiasRESUMO
PURPOSE: Pharyngocutaneous fistula (PCF) formation and swallowing difficulties are common and troublesome complications following total laryngectomy (TL). Prior (chemo)radiotherapy ((C)RT) is thought to be a risk factor for these complications, but there is conflicting evidence as to whether the time interval between (C)RT and TL is important. The impact of time interval on these complications and also its impact on overall survival are investigated. METHODS: This is a retrospective case note review of all patients undergoing TL at the University Medical Center, Utrecht, The Netherlands over the 10-year period from January 2008 to December 2017. The cohort was split into those who underwent TL within a year of finishing (C)RT and those longer than 1 year. RESULTS: One hundred and twenty-six patients (108 males, 18 females), with a mean age of 66 underwent total laryngectomy after prior (C)RT in the study period. Overall 5-year survival was 35% with a median follow-up of 30 months. Fifty-four patients underwent laryngectomy within a year of their (C)RT versus 72 patients who had a time interval of more than one year. No differences in PCF rate, risk of dilatation or overall survival could be found between the two groups. CONCLUSIONS: In this modern cohort, time interval between (C)RT and surgery did not impact PCF rate, risk of dilatation or overall survival.
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Fístula Cutânea , Neoplasias Laríngeas , Doenças Faríngeas , Quimiorradioterapia/efeitos adversos , Feminino , Humanos , Neoplasias Laríngeas/cirurgia , Laringectomia , Masculino , Países Baixos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Terapia de SalvaçãoRESUMO
BACKGROUND: Transoral robotic surgery (TORS) is increasingly used in head and neck surgery and in carcinoma of unknown primary (CUP) origin specifically. Due to the rising incidence of human papillomavirus (HPV)-related oropharyngeal squamous cell carcinoma (OPSCC), there is a rationale for finding ways to de-escalate treatment strategies. This review aims to test the hypothesis that TORS is a meaningful adjunct in the diagnostic (and therapeutic) pathway in CUP in head and neck. METHODS: A structured search of the literature was performed with the search terms 'TORS' and 'Carcinoma of Unknown Primary'. RESULTS: Two hundred and seventy four cases of CUP in which TORS was used were identified for further analysis. Workup for CUP was comparable in all series with regard to physical examination, fine and/or gross needle examination of cervical nodes, fibre optic endoscopy, imaging and robot assisted mucosectomy of the base of tongue (BOT). Identification rate of the primary tumour was 72% on average (range 17%- 90%), and 55%- 96% were HPV positive. Clear margins were achieved in 60% (range 0%-85%) of resected occult tumours. Complication rate of TORS BOT mucosectomy was low with mainly grade I-III sequelae according to Clavien-Dindo. CONCLUSIONS: Transoral robotic surgery seems to be a useful and safe adjunct in the diagnostic and therapeutic pathway in case of CUP in an era of increasing incidence of HPV-positive OPSCC.
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Neoplasias de Cabeça e Pescoço/cirurgia , Mucosa Bucal/cirurgia , Cirurgia Endoscópica por Orifício Natural/métodos , Neoplasias Primárias Desconhecidas/cirurgia , Procedimentos Cirúrgicos Robóticos/métodos , Língua/cirurgia , Neoplasias de Cabeça e Pescoço/secundário , Humanos , Boca , Neoplasias Primárias Desconhecidas/diagnóstico , Língua/patologiaRESUMO
A newly proposed classification by the European Laryngological Society (ELS) of glottic lesions by narrow-band imaging (NBI) divides their vascular patterns into longitudinal and perpendicular ones. The latter are further subdivided into the wide and narrow patterns. The longitudinal, wide, and narrow patterns are characteristic of benign disease, papilloma, and malignancy, respectively. The aim of the study was to investigate the diagnostic effectiveness of the classification. Forty patients with glottic lesions underwent microlaryngoscopy. The vascular patterns of all vocal cords were defined with NBI. The affected vocal cords were histologically analysed and comprised the arm (A). Unaffected vocal cords were not histologically analysed but followed-up and comprised the arm (B) and were regarded as true negatives if no suspicious changes appeared during the follow-up. The vocal cords from the arm A were categorised into the benign and malignant group according to the histologic result. The ratio of vascular patterns was determined and the groups were statistically compared using the Chi-square test and Fisher's exact test. Perpendicular changes were observed in 36.6% (9/26) of benign diseases and in 100% (23/23) of cancer conditions (p < 0.001). Wide perpendicular changes appeared only in papillomas (6/6) while narrow ones mostly in malignancies (23/26) and also in benign conditions (3/26) (p < 0.001). The sensitivity, specificity, positive and negative predictive values, and accuracy were 100, 95, 88, 100 and 96%, respectively. The new ELS classification can be used effectively and safely to differentiate malignant from benign disease.
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Doenças da Laringe/classificação , Laringoscopia , Imagem de Banda Estreita , Prega Vocal/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Europa (Continente) , Feminino , Seguimentos , Humanos , Doenças da Laringe/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Sensibilidade e Especificidade , Sociedades Médicas , Prega Vocal/patologia , Adulto JovemRESUMO
BACKGROUND: Sinonasal squamous cell carcinoma is known for its rarity and poor prognosis. This study aims to investigate the trends in the incidence of sinonasal squamous cell carcinoma in the Netherlands and to analyze patient characteristics and treatment practices in order to assess their relation with patient outcomes. METHODS: In this prospectively designed population-based cohort study, all patients diagnosed with sinonasal squamous cell carcinoma between 2008 and 2021 in the Netherlands were included. Patients with a squamous cell carcinoma of the nasal vestibule were excluded, leading to a definitive study population of 568 cases. RESULTS: The annual incidence rate for the overall population displayed a slight upward trend over the inclusion period with an annual percentage change of 1.6%. The 5-year overall, disease-free, and relative survival were 43.0%, 52.0%, and 47.2%, respectively. Relative survival decreased marginally over the course of the inclusion period. CONCLUSION: Sinonasal squamous cell carcinoma is rare with approximately stable incidence rates. There is a limited, non-statistically significant, decrease in survival between 2008 and 2021. Therefore, there is a clear need for innovative treatment approaches to improve outcomes for patients with sinonasal squamous cell carcinoma.
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The FGFR3::TACC3 fusion has been reported in subsets of diverse cancers including urothelial and squamous cell carcinomas (SCC). However, the morphology of FGFR3::TACC3-positive head and neck carcinomas has not been well studied and it is unclear if this fusion represents a random event, or if it might characterize a morphologically distinct tumor type. We describe nine FGFR3::TACC3 fusion-positive head and neck carcinomas affecting six males and three females aged 38 to 89 years (median, 59). The tumors originated in the sinonasal tract (n = 4), parotid gland (n = 2), and one case each in the oropharynx, submandibular gland, and larynx. At last follow-up (9-21 months; median, 11), four patients developed local recurrence and/or distant metastases, two died of disease at 11 and 12 months, one died of other cause, one was alive with disease, and two were disease-free. Three of six tumors harbored high risk oncogenic HPV infection (HPV33, HPV18, one unspecified). Histologically, three tumors revealed non-keratinizing transitional cell-like or non-descript morphology with variable mixed inflammatory infiltrate reminiscent of mucoepidermoid or DEK::AFF2 carcinoma (all were HPV-negative), and three were HPV-associated (all sinonasal) with multiphenotypic (1) and non-intestinal adenocarcinoma (2) pattern, respectively. One salivary gland tumor showed poorly cohesive large epithelioid cells with prominent background inflammation and expressed AR and GATA3, in line with a possible salivary duct carcinoma variant. Two tumors were conventional SCC. Targeted RNA sequencing revealed an in-frame FGFR3::TACC3 fusion in all cases. This series highlights heterogeneity of head and neck carcinomas harboring FGFR3::TACC3 fusions, which segregates into three categories: (1) unclassified HPV-negative category, morphologically distinct from SCC and other entities; (2) heterogeneous group of HPV-associated carcinomas; and (3) conventional SCC. A driver role of the FGFR3::TACC3 fusion in the first category (as a potential distinct entity) remains to be further studied. In the light of available FGFR-targeting therapies, delineation of these tumors and enhanced recognition is recommended.
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Background and purpose: Brachytherapy is treatment of choice for early stage nasal vestibule cancer. Over the years improvements were achieved by means of image guided target definition, interstitial implant techniques and also individual mold techniques. The aim of this study was to improve the technique of the implant so that the need for interstitial catheters can be limited by making use of patient individualized 3D-printed applicators. Materials and Methods: In 19 patients 3D-printed applicators were used to deliver pulse dose rate (PDR) brachytherapy. All patients underwent computed tomography (CT) and magnetic resonance imaging (MRI). A pre-plan with tumor delineation and manually optimized catheter positions to achieve tumor coverage was made. Based on the pre-plan a 3D-printed applicator was manufactured. Dose was evaluated by several indices: Conformity Index, Healthy Tissues Conformity Index, Dose Homogeneity Index, Dose non-uniformity ratio, Conformal index and high dose (HD) index. Results: A high target coverage was achieved, with a median V100%CTV of 99.1 % (range, 81.8-100 %) and median CI of 0.99 (range, 0.82-1.00), as well as a median V0.7GyGTV of 100 % (range, 93.0-100 %). The median HD was 0.39 (range, 0.20-0.83). Interstitial catheters were needed in 12 patients. None of the patients developed grade ≥ II toxicity within the median follow up of 18 months. Conclusions: This study shows that using 3D-printed applicators limits the need for interstitial catheters and also limits the high doses in normal tissue.
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With the wide use of RNA sequencing technologies, the family of FET::CREB fusion mesenchymal neoplasms has expanded rapidly to include potentially aggressive neoplasms, not fitting any well established WHO entity. Recently, a group of intra-abdominal FET(EWSR1/FUS)::CREB(CREM/ATF1) fused unclassified neoplasms has been reported followed by recent recognition of an analogous extra-abdominal category of unclassified neoplasms carrying EWSR1::ATF1 fusions. We describe 9 additional tumors (5 extra-abdominal and 4 abdominal) carrying an EWSR1::CREM (n = 8) and FUS::CREM (n = 1) fusion. Patients were 7 females and 2 males aged 10 to 75 years (median, 34). Extra-abdominal tumors originated in the head and neck (2 sinonasal, 1 orbital) and soft tissues (1 gluteal, 1 inguinal). Abdominal tumors involved stomach (2), mesentery (1), and kidney (1). Tumor size ranged from 3.5 to 11 cm (median, 6). Treatment was radical surgery with (5) or without (2) neo/adjuvant radio/chemotherapy. Extended follow-up of 5 patients (21-52 months; median, 24) showed an aggressive course in two (40%); one died of disseminated metastases 52 months after several intensified chemotherapy regimens, and one was alive with progressive abdominal disease at 21 months. The immunophenotype of the two subcohorts was significantly overlapping with variable expression of EMA (7 of 8), keratin AE1/AE3 (5 of 9), CD99 (4 of 7), MUC4 (2 of 8), ALK (3 of 8), synaptophysin (3 of 9), chromogranin (1 of 8), CD34 (3 of 6), CD30 (1 of 6), PAX8 (1 of 7), and inhibin (1 of 7), but no reactivity with desmin (0 of 8), S100 (0 of 8), and SOX10 (0 of 8). This series further solidifies the notion that FET::CREB fusions are not limited to the triad of angiomatoid fibrous histiocytoma, clear cell sarcoma, and malignant gastrointestinal neuroectodermal tumor, but characterize an emerging family of potentially aggressive neoplasms occurring at both intra- and extra-abdominal sites. These tumors underscore the promiscuity of the FET::CREB fusions and highlight the pivotal role of phenotype-oriented classification of these neoplasms that share the same genotype, still featuring significant biological and behavioral distinctness.
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BACKGROUND: Squamous cell carcinoma of the nasal vestibule (SCCNV) is a rare disease, distinctly different in presentation, treatment, and outcome from squamous cell carcinoma (SCC) of the nasal cavity and paranasal sinuses. However, these are often not analyzed separately. METHODS: The Netherlands Cancer Registry (NCR) and pathology reports from the Dutch Nationwide Pathology Databank (PALGA) were used to identify all newly diagnosed SCCNV cases in the Netherlands between 2008 and 2021. RESULTS: A total of 763 patients were included. The yearly incidence rate displayed a significant downward trend with an annual percentage change (APC) of -3.9%. The 5-year overall survival (OS) and disease-free survival were 69.0% and 77.2%, respectively. The 5-year relative survival was 77.9% and improved slightly over the inclusion period. OS for patients who were staged cT3 appeared to be worse than those staged cT4a, calling the applicability of the TNM-classification into question. CONCLUSION: SCC of the nasal vestibule is rare, with declining incidence rates. Introducing a specific topography code for SCCNV is recommended to enhance registration accuracy. The TNM classification seems poorly applicable to SCCNV, suggesting the need to explore alternative staging methods.
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Carcinoma de Células Escamosas , Cavidade Nasal , Neoplasias Nasais , Sistema de Registros , Humanos , Países Baixos/epidemiologia , Masculino , Feminino , Neoplasias Nasais/patologia , Neoplasias Nasais/epidemiologia , Neoplasias Nasais/mortalidade , Neoplasias Nasais/terapia , Idoso , Pessoa de Meia-Idade , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/epidemiologia , Carcinoma de Células Escamosas/mortalidade , Cavidade Nasal/patologia , Incidência , Adulto , Idoso de 80 Anos ou mais , Estadiamento de Neoplasias , Intervalo Livre de Doença , Taxa de SobrevidaRESUMO
INTRODUCTION: There is a lack of comprehensive and uniform data on head and neck paragangliomas (HNPGLs), and research is challenging due to its rarity and the involvement of multiple medical specialties. To improve current research data collection, we initiated the Head and Neck Paraganglioma Registry (HNPGL Registry). The aim of the HNPGL Registry is to a) collect extensive data on all HNPGL patients through a predefined protocol, b) give insight in the long term outcomes using patient reported outcome measures (PROMs), c) create uniformity in the diagnostic and clinical management of these conditions, and thereby d) help provide content for future (randomized) research. METHODS AND ANALYSIS: The HNPGL Registry is designed as a prospective longitudinal observational registry for data collection on HNPGL patients and carriers of (likely) pathogenic variants causative of HNPGLs. All patients, regardless of the received treatment modality, can be included in the registry after informed consent is obtained. All relevant data regarding the initial presentation, diagnostics, treatment, and follow-up will be collected prospectively in an electronic case report form. In addition a survey containing the EuroQol 5D-5L (EQ-5D-5L), European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30), Modified Fatigue Impact Scale (MFIS), Short QUestionnaire to Assess Health-enhancing physical activity (SQUASH), Cancer Worry Scale (CWS) and Hospital Anxiety and Depression Scale (HADS) will be sent periodically. The registry protocol was approved by the Medical Ethical Review Board of the University Medical Center Utrecht. CONCLUSION: The HNPGL Registry data will be used to further establish the optimal management for HNPGL patients and lay the foundation for guideline recommendations and the outline of future research.
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Neoplasias de Cabeça e Pescoço , Paraganglioma , Qualidade de Vida , Sistema de Registros , Humanos , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/terapia , Paraganglioma/diagnóstico , Paraganglioma/terapia , Estudos Prospectivos , Coleta de Dados , Medidas de Resultados Relatados pelo Paciente , Masculino , Feminino , Inquéritos e QuestionáriosRESUMO
The Dutch guideline for patients suspected of head and neck paragangliomas (HNPGLs) recommends magnetic resonance imaging (MRI) and/or computed tomography (CT) of the head and neck area. Additionally, it suggests considering additional nuclear imaging. The aim of this study was to evaluate the outcomes of [68Ga]Ga-DOTATOC PET/CT compared to MRI in patients with suspected HNPGLs and carriers of genetic variations. METHODS: In this single-center pilot study, retrospective data were obtained from consecutive patients between 2016 and 2023. Both MRI and [68Ga]Ga-DOTATOC PET/CT were performed within 12 months. The primary outcome was the location of HNPGLs. RESULTS: A total of 25 consecutive patients were included, and 7 patients (28.0%, p = 0.5) showed differences between the imaging modalities, of whom 5 patients had unexpected localizations with additional uptake by somatostatin receptors (SSTR) on the [68Ga]Ga-DOTATOC PET/CT. CONCLUSIONS: The authors recommend performing baseline imaging with [68Ga]Ga-DOTATOC PET/CT (if available) in variant carriers and using MRI/CT for follow-up according to the regional protocol, thereby shifting the gold standard for baseline imaging from MRI/CT to [68Ga]Ga-DOTATOC PET/CT.
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Broncopatias/induzido quimicamente , Broncoscopia , Aspiração Respiratória/patologia , Mucosa Respiratória/patologia , Nitrato de Prata/efeitos adversos , Idoso de 80 Anos ou mais , Broncopatias/etiologia , Broncopatias/patologia , Tecido de Granulação , Humanos , Laringectomia , Laringe Artificial , Masculino , Aspiração Respiratória/complicaçõesRESUMO
BACKGROUND: Sinonasal mucosal melanoma (MM) is a rare, aggressive melanoma subtype. Complete surgical excision, with or without adjuvant radiotherapy, remains the cornerstone of treatment and yields adequate locoregional control. Metastatic MM is managed similarly to metastatic cutaneous melanoma but with poorer survival. PReferentially expressed Antigen in MElanoma (PRAME) has been identified as a potential diagnostic marker and therapeutic target in the treatment of cutaneous melanoma. METHODS: Retrospective analysis of the clinical characteristics and immunohistochemical features of all sinonasal MM patients referred to the department of Head and Neck Surgical Oncology, UMC Utrecht Cancer Center, between 2011 and 2021 was performed. Single nucleotide polymorphism (SNP) array and next-generation sequencing (NGS) were performed in selected cases. RESULTS: A total of 26 patients with an MM were included. The median follow-up duration was 15 months. At the end of follow-up, 13 patients had died due to progression of their disease, and one patient died of intercurrent disease. PRAME immunohistochemistry was performed in 23 out of 26 cases, all displaying PRAME expression. In two cases PRAME expression was present both within the melanoma cells and in melanocytes in adjacent mucosa. SNP array showed ≥ 5 copy number variants (CNV) in all tested cases, with a median of 29.5 CNVs (IQR 23.25-40). The three most common mutations identified by NGS were NRAS (7 cases) and NF1 (2 cases). CONCLUSION: We show that expression of PRAME is common in sinonasal MM, making PRAME a useful ancillary diagnostic tool and a potential therapeutic target in sinonasal MM. The demonstrated occurrence of extensive presence of PRAME-positive melanocytes in the surrounding mucosa of sinonasal MM might explain the multifocal nature of melanoma in the (sinonasal) mucosa, and would be an extra argument for a PRAME targeting treatment in preventing local disease recurrence.
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Melanoma , Neoplasias dos Seios Paranasais , Neoplasias Cutâneas , Humanos , Melanoma/genética , Melanoma/patologia , Neoplasias Cutâneas/patologia , Estudos Retrospectivos , Neoplasias dos Seios Paranasais/patologia , Antígenos de Neoplasias/metabolismo , Melanoma Maligno CutâneoRESUMO
PURPOSE: Cancer of the nasal vestibule is a rare type of malignancy constituting less than one percent of all head and neck cancers. These tumors are typically diagnosed at an early stage. Both surgery and radiotherapy provide excellent oncological results, but esthetic results are better after radiotherapy. The aim of this study was to evaluate the long-term oncological follow-up after brachytherapy for early stage squamous cell carcinoma of the nasal vestibule. METHODS AND MATERIALS: Retrospective analysis of patients with carcinoma of the nasal vestibule who were treated with primary brachytherapy in the Utrecht University Medical Center. RESULTS: In this single center experience over a 17-year period 68 patients with early stage squamous cell carcinoma of the nasal vestibule were treated with brachytherapy. Two patients had lymph node metastases at first clinical presentation. Median follow-up duration was 46.5 months. Five-year locoregional recurrence-free survival, disease-specific survival, and overall survival were 91.1%, 96.1%, and 66.2%, respectively. All recurrences occurred within the first 3 years of follow-up. CONCLUSIONS: Brachytherapy offers excellent oncological outcomes and is a safe and effective treatment for early stage carcinoma of the nasal vestibule. Recurrences typically occur within 3 years after treatment.
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Braquiterapia , Carcinoma de Células Escamosas , Neoplasias Nasais , Humanos , Seguimentos , Braquiterapia/métodos , Estudos Retrospectivos , Recidiva Local de Neoplasia/radioterapia , Recidiva Local de Neoplasia/etiologia , Carcinoma de Células Escamosas/patologia , Dosagem RadioterapêuticaRESUMO
Background: Sinonasal teratocarcinosarcoma is a rare, aggressive malignancy located almost exclusively in the nasal cavity, paranasal sinuses, or anterior skull base. Histopathological diagnosis can be challenging due to the heterogeneous composition. Methods: Retrospective analysis of 3 patients with sinonasal teratocarcinosarcoma diagnosed and treated at the University Medical Center Utrecht was conducted. Results: Patients presented with nasal obstruction, epistaxis, headaches, or behavioral changes. All three patients had locally advanced disease, and one had lymph node metastases. Two patients underwent surgery followed by radiotherapy, and one underwent neoadjuvant chemotherapy followed by surgery. The follow-up duration ranged from 3 to 32 months. All three patients died due to progression of their disease. Conclusion: Sinonasal teratocarcinosarcoma is characterized by rapid, aggressive local expansion. The prognosis is poor due to a high risk of metastases and locally recurrent disease. Multimodality treatment consisting of surgery, followed by (chemo)-radiotherapy, is essential for optimizing outcomes. Neoadjuvant therapy offers a promising treatment option.
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Squamous cell carcinoma of the nasal vestibule is reported to account for less than one percent of all head and neck malignancies. It lacks a designated WHO ICD-O topography code, and multiple systems are available for the staging of this disease, which results in unwanted variability and the subsequent poor reliability of data. The aim of this study was to evaluate the currently available staging systems for cancer of the nasal vestibule, including the recently introduced classification by Bussu et al., which built on Wang's original concept but with clearer anatomical cutoffs. Different staging systems for cancer of the nasal vestibule (UICC nasal cavity, UICC skin cancer of the head and neck, Wang and Bussu et al.) were evaluated via a retrospective analysis of 148 patients. The staging system, per Bussu et al., had the most balanced allocation of patients among the stages. When using the Wang classification as a reference, stage migration occurred less frequently with the Bussu classification. The widespread adoption of a single staging system, as well as the introduction of a designated topography code for cancer of the nasal vestibule, could lead to more uniformity in data reporting and improve an understanding of the incidence and disease outcome. The newly proposed carcinoma of the nasal vestibule classification by Bussu et al. has the potential to improve the staging and allocation among stages. Further analysis of survival data is needed to assess which classification system is best suited for nasal vestibule carcinoma.
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Squamous cell carcinoma of the nasal vestibule is considered a rare malignancy that differs from other sinonasal malignancies in many respects. Four staging systems currently exist for this disease, the most recent addition being the "Rome" classification. This study assesses the use of this new classification and its prognostic value regarding various outcome measures. A retrospective multicenter cohort study of patients with a primary squamous cell carcinoma of the nasal vestibule who were treated in three tertiary head and neck oncology referral centers was conducted. A total of 149 patients were included. The median follow-up duration was 27 months. Five-year locoregional control (LRC), disease-specific survival (DSS), and overall survival (OS) were 81.6%, 90.1, and 62.5% respectively. A statistically significant association was observed between the Rome classification and all survival outcomes in both univariable and multivariable analyses. Moreover, it appeared to perform better than the Union for International Cancer Control TNM classification for tumors of the nasal cavity and paranasal sinuses. The new Rome classification can be used effectively and is associated with LRC, DSS, and OS. However, it requires further validation in a larger (prospective) study population.
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OBJECTIVES: Surgical removal of squamous cell carcinoma of the tongue (SCCT) with tumour-free margin status (≥5 mm) is essential for loco-regional control. Inadequate margins (<5 mm) often indicate adjuvant treatment, which results in increased morbidity. Ultrasound (US)-guided SCCT resection may be a useful technique to achieve more adequate resection margins compared to conventional surgery. This study evaluates the application and accuracy of this technique. METHODS: Forty patients with SCCT were included in a consecutive US cohort. During surgery, the surgeon aimed for a 10-mm echographic resection margin, while the tumour border and resection plane were captured in one image. Ex-vivo US measurements of the resection specimen determined whether there was a need for an immediate re-resection. The margin status and the administration of adjuvant treatment were compared those of with a consecutive cohort of 96 tongue cancer patients who had undergone conventional surgery. A receiver operating characteristic analysis was done to assess the optimal margin of ex-vivo US measurements to detect histopathologically inadequate margins. RESULTS: In the US cohort, the frequency of free margin status was higher than in the conventional cohort (55% vs. 16%, p < 0.001), and the frequency of positive margins status (<1 mm) was lower (5% vs. 15%, respectively, p < 0.001). Adjuvant radiotherapy was halved (10% vs. 21%), and the need for re-resection was comparable (10% vs. 9%). A cut-off value of 8 mm for ex-vivo measurements prevented histopathologically inadequate margins in 76%. CONCLUSION: US-guided SCCT resections improve margin status and reduce the frequency of adjuvant radiotherapy.