Intestinal-type Adenocarcinoma Research Articles

Meningeal Metastasis from Naso-Ethmoidal Malignancies: Pathogenesis, Risk Factors, and Prognostic Impact

Introduction: Meningeal metastasis (MM) from naso-ethmoidal malignancies (NEMs) is rare, its metastatic route is still debated, and its prognostic impact remains unclear. Our aim is to analyze a retrospective series of NEMs with non-contiguous MM to study the possible route of spread and the prognostic value of MM. Materials and methods: The institutional database of SNC treated at the University of Brescia between 1995 and 2021 was reviewed. Clinical–pathological data were collected, and survivals were estimated with Kaplan–Meier. Univariate and multivariate logistic regression analysis were run to identify predictors of MM. Results: Among 296 patients, 17 experienced non-contiguous MM, all located along the dura. Intestinal-type adenocarcinoma (10/17) and olfactory neuroblastoma (3/17) were the most frequent histologies. At univariate analysis, brain edema (p < 0.0001), resection (p = 0.026) or invasion (p = 0.006) of brain parenchyma, and local (p = 0.0004) and nodal (p = 0.021) recurrence were predictors of MM. At multivariate analysis, local recurrence was confirmed as an independent factor (odds ratio: 11.88, p = 0.0005). Dural surgical resection was not a risk factor. The five-year disease-specific survival was longer in patients with exclusive MM compared with patients with distant metastasis at other sites (64.3% vs. 30.1% p = 0.046). Conclusions: Dural venous shunt is the most likely pathway of spread of MM. Local recurrence is the only independent risk factor. Exclusive MM has a better prognosis than extrameningeal metastasis.

Intestinal type adenocarcinoma in salivary glands: A case series

Intestinal type adenocarcinoma in salivary glands: A case series

Bilateral ovarian mature cystic teratoma with unilateral malignant transformation to adenocarcinoma: A case report.

Mature cystic teratoma (MCT) is the most common germ cell tumor of the ovary, comprising 20% of all ovarian neoplasms. Malignant transformation (MT) is an uncommon complication and occurs in approximately 1-3% of all MCTs. The most common histological type of MT is squamous cell carcinoma. Other rare transformations include - carcinoid tumors, melanoma, adenocarcinoma, and sarcoma. We present one such rare case of bilateral MCT with unilateral MT into mucinous adenocarcinoma of intestinal type.

Primary Vulvar and Vaginal Adenocarcinomas of Intestinal Type Are Closer To Colorectal Adenocarcinomas Than To Carcinomas of Müllerian Origin

Primary vulvar and vaginal adenocarcinomas of intestinal type (VVAIts) are very rare tumors, displaying morphologic and immunohistochemical overlap with colorectal adenocarcinomas. However, their immunoprofile and genomics are poorly studied, and their origin is still debated. Here, we studied a series of 8 VVAIts (4 vulvar and 4 vaginal) using a large panel of immunohistochemistry and DNA and RNA sequencing with clustering analyses. All tumors shared a similar morphology with colorectal adenocarcinomas and diffuse CK20 and CDX2 expression. SATB2 diffuse positivity was observed in 62.5% of tumors and CK7 in 82.5%, whereas PAX8, SOX17, p16, and estrogen and progesterone receptors were always negative. A p53 mutated-type expression was observed in 75% of tumors. All tumors were mismatch repair proficient. Neither human papillomavirus DNA nor pathogenic transcript fusions were detected. The most frequent molecular alterations were TP53 and KRAS variants in 71.4% and 42.9%, respectively. The transcriptomic analysis highlighted a robust VVAIts cluster distinct from endocervical, ovarian, lung, thyroid, salivary glands, breast, and renal carcinomas but failed to differentiate vulvar from vaginal intestinal-type tumors. On 2 different clustering analyses, VVAIts clustered altogether, very close to colorectal adenocarcinomas. Compared with endocervical adenocarcinomas of intestinal type, VVAIts had a significantly lower expression of SOX17 and epithelial-mesenchymal transition genes and a higher mitogen-activated protein kinase pathway gene expression. These results suggest that Müllerian structures leading to cervical adenocarcinomas may undergo intestinal-type transdifferentiation via an epithelial-mesenchymal transition phenomenon. Conversely, mitogen-activated protein kinase pathway activation in VVAIts, which plays a major role in colorectal adenocarcinomas, may indicate a close relationship in the carcinogenesis of these tumors. Our results indicate that adenocarcinomas of intestinal type, in the distal vagina or vestibular vulva, might be a unique and single entity, probably originating from cloacogenic embryonic remnants and/or ectopic colorectal mucosae inclusions. An open question would be to explore the efficacy of systemic drugs prescribed in colorectal cancers, in VVAIts.

Incidence of Gastric Adenocarcinoma in Those With Gastric Atrophy: A Systematic Review.

Gastric atrophy (GA), or atrophic gastritis, is a pre-neoplastic lesion of gastric cancer (GC). It is part of the Correa cascade, which culminates in intestinal-type gastric adenocarcinoma. The cascade posits that intestinal-type gastric adenocarcinoma develops along a defined pathway of pre-neoplastic stages. The cascade begins with chronic gastritis, most commonly caused by Helicobacter pylori (H. pylori) infection, and proceeds through GA, gastric intestinal metaplasia (GIM), both complete and incomplete, dysplasia, both low and high-grade, and culminating in intestinal-type gastric adenocarcinoma. Attempts in Europe have been made to identify patients at risk of developing GC and target them with surveillance oesophagogastroduodenoscopy (OGD). However, there remains uncertainty about GA's risk of developing into GC. This poses issues in terms of guiding the need for and determining intervals for surveillance OGDs, which are a costly form of surveillance. As such, we attempted to gather all available studies assessing the risk of GC developing from GA, which is the first step in the Correa cascade.This study was a comprehensive systematic review of published papers, reported per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. This systematic review, which included a substantial 25,455 patients across 18 studies, found that the relative risk (RR) of GC in those with GA, using standardised incidence ratios as a measure of RR, was 15.1, with a 95% confidence interval ranging from 13.5 to 16.9. We conclude thatGA does increase the risk of developing GC, and this risk may be higher than previously appreciated. Further large-scale studies are needed in Western cohorts of patients to precisely define this risk and guide the need for surveillance programs. These future studies must be standardised to account for H. pylori status, the topographical distribution of the GA, and the methods for assessing the degree of GA.

KLHL21 suppresses gastric tumourigenesis via maintaining STAT3 signalling equilibrium in stomach homoeostasis

ObjectivePrecancerous metaplasia transition to dysplasia poses a risk for subsequent intestinal-type gastric adenocarcinoma. However, the molecular basis underlying the transformation from metaplastic to cancerous cells remains poorly understood.DesignAn integrated analysis...

Sinonasal intestinal-type adenocarcinoma in northern Portugal: a woodworker's occupational hazard.

Sinonasal carcinomas (SNC) constitute about 3% of all head and neck carcinomas, squamous cell carcinoma being the most common histologic subtype. Some geographical patterns in incidence are observed, as occupational exposure to wood dust is associated with intestinal-type adenocarcinoma (ITAC). This study aims to describe the prevalence and characteristics of sinonasal ITAC cases in a region of northern Portugal with a robust wood industry. A retrospective study of all sinonasal malignancies diagnosed over 5 years, in a tertiary hospital, primarily focused on ITAC subtypes. Data on sociodemographic and clinical variables were collected through electronic medical records and telephonic assessments. Of all 33 cases, 85% were ITAC. ITAC predominantly affected males (96%) with a mean age of 66.2 years. Most patients diagnosed with sinonasal ITAC had occupational wood dust exposure (96%), particularly to hardwoods. Initial symptoms included unilateral nasal obstruction (75%) and epistaxis (71%). Despite a median time of 3 months from symptoms to consultation, 50% presented at an advanced stage, with a 29% mortality rate. Sinonasal ITAC is an occupational hazard of woodworkers and represents the vast majority of SNC in the region. This study advocates for targeted community interventions, emphasizing occupational safety measures and healthcare awareness to reduce morbimortality associated with this occupational cancer.

Histopathological Study of Whipple’s Pancreaticoduodenectomy Specimens at Tertiary Care Center

Introduction Whipple’s pancreaticoduodenectomy has become a widely used resection procedure for pancreatic head, periampullary, ampullary, and biliary tumors. This study was conducted to analyze the histopathological features of these tumors in pancreaticoduodenectomy specimen. MethodsA descriptive study was carried out in the Pathology Department of Kathmandu Medical College Teaching Hospital. Pancreaticoduodenectomy specimens received were assessed for tumor location, histopathologic categorization, grading, lymphovascular and perineural invasion, margin status, and lymph node status. AJCC TNM staging was done. ResultsOut of 41 Whipple's pancreaticoduodenectomy cases, 35 (85.4%) were malignant and 6 (14.6%) were benign. The patients' ages ranged from 30 to 84 years, with a mean age of 58.3 years and a male to female ratio of 1.2:1. Adenocarcinoma was the most common malignant lesion (73.2%; n=30) frequently found in the intra-ampullary and periampullary regions (31.4%; n=11). The intestinal type of adenocarcinoma was the most common (42.8%; n=15), followed by the pancreaticobiliary type (34.3%; n=12). Most tumors were moderately differentiated (74.3%; n=26). Perineural invasion was frequently seen in intra-ampullary and periampullary carcinoma (81.8%; n=9). Lymphovascular invasion was frequently seen in intra-ampullary and periampullary carcinoma (81.8%; n=9). Most tumors were in the T3 stage (42.9%; n=15), and overall nodal metastasis (N1 and N2) was seen in 57.1% (n=20). ConclusionAdenocarcinoma was the commonest diagsosis in pancreaticoduodenectomy specimens of the intra-ampullary and periampullary region. Malignant lesions were more frequent than benign lesion in our study.

Clinical Pathological and Immunohistochemical Correlations in Gastric Cancer.

Due to its high aggressiveness and polyclonal tumor state, stomach cancer is considered a severe health problem. In this study, we analyzed Her2 and Ki67 in correlation with patient data for the possibility of prognostic factors. The study included 48 cases of gastric tumors that had been surgically treated in a period of five years. The percentage was statistically significant for intestinal-type adenocarcinomas located in the medio-gastric region (p = 0.05); in the diffuse subtype, there were no Her2 positive samples, and in the mixed subtype only one out of three samples was Her2 positive. Our results confirm the existing data, and we can conclude that this link can be considered a prognostic factor in the progression and treatment effectiveness.

Prognostic value analysis and survival model construction of different treatment methods for advanced intestinal type gastric adenocarcinoma

Intestinal-type gastric adenocarcinoma, representing 95% of gastric malignancies, originates from the malignant transformation of gastric gland cells. Despite its prevalence, existing methods for prognosis evaluation of this cancer subtype are inadequate. This study aims to enhance patient-specific prognosis evaluation by analyzing the clinicopathological characteristics and prognostic risk factors of intestinal-type gastric adenocarcinoma patients using data from the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute (NCI). We extracted clinical data for patients diagnosed with intestinal-type gastric adenocarcinoma between 2010 and 2015 from the SEER database, selecting 257 cases based on predefined inclusion and exclusion criteria. Independent risk factors for overall survival (OS) and cancer-specific survival (CSS) were identified using a Cox regression model. A nomogram model for predicting OS or CSS was developed from the Cox risk regression analysis and validated through the consistency index (C-index), ROC curve, and calibration curve. Age, primary tumor resection, chemotherapy, lymph node metastasis, and tumor size were identified as independent prognostic factors for OS and CSS (P<0.05). The nomogram model, constructed from these indicators, demonstrated superior predictive consistency for OS and CSS compared to the AJCC-TNM staging system. ROC curve analysis confirmed the model's higher accuracy, and calibration curve analysis indicated good agreement between the nomogram's predictions and actual observed outcomes. The nomogram model derived from SEER database analyses accurately predicts OS and CSS for patients with intestinal-type gastric adenocarcinoma. This model promises to facilitate more tailored treatments in clinical practice.

Gender Differences in Sinonasal Cancer Incidence: Data from the Italian Registry.

Although rare, sinonasal cancers (SNCs) have a high occupational attributable fraction. We applied gender-based approaches to descriptive analyses, incidence, and patterns of exposures using the Italian National Sinonasal Cancer Registry (ReNaTuNS: Registro Nazionale Tumori Naso-Sinusali). The study included 2851 SNC patients. SNC was diagnosed more often in men (73%) than in women (27%). The most frequent morphology in men was intestinal-type adenocarcinoma (33%), whereas in women, it was squamous cell carcinoma (49%). Nasal cavities were predominant in both genders (50%), ethmoidal sinus in men (24%), and maxillary in women (24%). Incidence rates were 0.76 (per 100,000 person-years) in men and 0.24 in women and increased by age, more evidently in men, peaking over 75 years in both. Occupational exposures to wood and leather dusts were the most frequent (41% for men, 33% for women). Few exposures were extra-occupational or domestic. Unlikely exposure was relevant in women (57%). The surveillance of SNC cases through a registry that allows for the identification of and compensation for this occupational disease is important in Italy, where numerous workers are exposed to carcinogens for SNC, without even being aware. Considering the rarity of the disease, particularly among women, the ReNaTuNS can provide a method to analyze gender differences.

Possible Role of Cytomegalovirus in Gastric Cancer Development and Recurrent Macrolide-Resistant Campylobacter jejuni Infection in Common Variable Immunodeficiency: A Case Report and Literature Discussion.

Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency in adults. It comprises a group of syndromes whose etiology involves genetic, epigenetic, microbiota, and environmental factors. We present the case of a 46-year-old Caucasian male patient with CVID and an immune dysregulation phenotype. The particular elements of the case consisted of an atypical clinical course, which undoubtedly demonstrates the great variability of clinical manifestations that these types of patients can suffer from, including bacterial and viral infections, autoimmune phenomena, and neoplasia. Notably, the patient suffered from recurrent gastrointestinal infection with macrolide-resistant Campylobacter jejuni and gastroduodenal disease and viraemia by cytomegalovirus (CMV). In addition, CMV was postulated as the main pro-oncogenic factor contributing to the development of early-onset intestinal-type gastric adenocarcinoma, for which the patient underwent gastrectomy. The patient's evolution was difficult, but finally, as a result of the multidisciplinary approach, clinical stabilization and improvement in his quality of life were achieved. Based on our brief literature review, this is the first reported case of this clinical complexity. Our experience could help with the management of future patients with CVID and may also update current epidemiological data on CVID.

Tumor Budding, p53, and DNA Mismatch Repair Markers in Sinonasal Intestinal-Type Adenocarcinoma: A Retrospective Study Supports the Adverse Prognostic Impact of Tumor Budding.

Sinonasal intestinal-type adenocarcinoma (ITAC) is a very rare, closely occupational-related tumor with strong histological similarities to colorectal cancer (CRC). In the latter, tumor budding (TB) is widely recognized as a negative prognostic parameter. The aim of this study was to evaluate the prognostic role of TB in ITAC and to correlate it with other established or emerging biomarkers of the disease, such as p53 and deficient DNA mismatch repair (MMR) system status/microsatellite instability (MSI). We retrospectively analyzed 32 consecutive specimens of patients with ITAC diagnosis treated in two institutions in Northern Italy. We reviewed surgical specimens for TB evaluation (low-intermediate/high); p53 expression and MMR proteins were evaluated via immunohistochemistry. Results were retrospectively stratified using clinical data and patients' outcomes. According to bud counts, patients were stratified into two groups: intermediate/high budding (>4 TB) and low budding (≤4 TB). Patients with high TB (>4) have an increased risk of recurrence and death compared to those with low TB, with a median survival of 13 and 54 months, respectively. On multivariate analysis, considering TB, therapy, and stage as covariates, TB emerged as an independent prognostic factor net of the stage of disease or type of therapy received. No impact of p53 status as a biomarker of prognosis was observed and no alterations regarding MMR proteins were identified. The results of the present work provide further significant evidence on the prognostic role of TB in ITAC and underline the need for larger multicenter studies to implement the use of TB in clinical practice.

Association of E-Cadherin Expression with Histological Type and Grade of Gastric Adenocarcinoma

Objectives: To determine the immune expression of E-Cadherin in gastric adenocarcinomas and its association with tumor histological type and grade to evaluate its prognostic significance. Methodology: This descriptive cross-sectional study was conducted at the Pakistan Institute of Medical Sciences Islamabad, from December 2017 to January 2021. Eighty-one patients from both genders, aged 21 to 83 years, undergoing gastric biopsies and Gastrectomy specimens to diagnose gastric adenocarcinoma were included in our study. Immunohistochemical processing was performed and the results were interpreted by the consultant pathologist for the expression of E-cadherin on gastric adenocarcinoma. The data were analyzed with SPSS version 17. Results: There was a significant statistical association between loss of E-Cadherin with histological type of adenocarcinoma (p = 0.000). In total 81 cases, there were 53 (65%) cases of intestinal type adenocarcinoma and 28 (35%) cases of diffuse type adenocarcinoma. Of the 28 cases with diffuse type gastric Adenocarcinoma, 10 cases showed complete loss of E-Cadherin. This complete loss of Cadherin was also seen in four out of 53 cases of intestinal adenocarcinoma, all of which were poorly differentiated intestinal type of adenocarcinomas. Total cases of well differentiated type of adenocarcinoma were nine; all of these showed high expression of E-Cadherin. Conclusion: There is a strong association of E-Cadherin expression with the histological type and grade of gastric adenocarcinoma according to Lauren’s classification. As the grade of the adenocarcinoma gets higher there is more loss of E-Cadherin expression which proves its prognostic significance. It was also established that gastric adenocarcinoma is a disease of elderly with male predominance.

Genome-wide somatic mutation analysis of sinonasal adenocarcinoma with and without wood dust exposure

BackgroundSinonasal adenocarcinoma is a rare cancer, encompassing two different entities, the intestinal-type sinonasal adenocarcinoma (ITAC) and the non-intestinal-type sinonasal adenocarcinoma (non-ITAC). Occurrence of ITAC is strongly associated with exposure to hardwood dusts. In countries with predominant exposure to softwood dust the occurrence of sinonasal adenocarcinomas is lower and the relative amount of non-ITACs to ITACs is higher. The molecular mechanisms behind the tumorigenic effects of wood dust remain largely unknown.MethodsWe carried out whole-genome sequencing of formalin-fixed paraffin-embedded (FFPE) samples of sinonasal adenocarcinomas from ten wood dust-exposed and six non-exposed individuals, with partial tobacco exposure data. Sequences were analyzed for the presence of mutational signatures matching COSMIC database signatures. Driver mutations and CN variant regions were characterized.ResultsMutation burden was higher in samples of wood dust-exposed patients (p = 0.016). Reactive oxygen species (ROS) damage-related mutational signatures were almost exclusively identified in ITAC subtype samples (p = 0.00055). Tobacco smoke mutational signatures were observed in samples of patients with tobacco exposure or missing information, but not in samples from non-exposed patients. A tetraploidy copy number (CN) signature was enriched in ITAC subtype (p = 0.042). CN variation included recurrent gains in COSMIC Cancer Gene Census genes TERT, SDHA, RAC1, ETV1, PCM1, and MYC. Pathogenic variants were observed most frequently in TP53, NF1, CHD2, BRAF, APC, and LRP1B. Driver mutations and copy number gains did not segregate by subtype.ConclusionsOur analysis identified distinct mutational characteristics in ITAC and non-ITAC. Mutational signature analysis may eventually become useful for documentation of occupation-related cancer, while the exact mechanisms behind wood dust-driven carcinogenesis remain elusive. The presence of homologous recombination deficiency signatures implies a novel opportunity for treatment, but further studies are needed.

Retrospective analysis of the incidence of appendiceal neoplasm and malignancy in patients treated for suspected acute appendicitis

BackgroundNonoperative management of uncomplicated appendicitis is currently being promoted as treatment option, albeit 0.7–2.5% of appendectomies performed due to suspected acute appendicitis show histologically malignant findings. The purpose of this study was to investigate the incidence of neoplasm and malignancy of the appendix in patients presenting with suspected acute appendicitis in real world setting.MethodsThis is a retrospective single-centre investigation of 457 patients undergoing appendectomy between the years 2017–2020. The patients’ demographics, symptoms and diagnosis, intraoperative findings, and histopathological results were analysed.ResultsIn 3.7% (n = 17) histological analysis revealed neoplasms or malignancies. Median age was 48 years (20–90 years), without sex predominance. Leukocytes (11.3 ± 3.7 G/l) and C-reactive protein (54.2 ± 69.0 mg/l) were elevated. Histological analysis revealed low-grade mucinous appendiceal neoplasia (n = 3), sessile serrated adenoma of the appendix (n = 3), neuroendocrine tumours (n = 7), appendiceal adenocarcinoma of intestinal type (n = 3), and goblet cell carcinoma (n = 1). Additional treatment varied between no treatment or follow-up due to early tumour stage (n = 4), follow-up care (n = 3), additional surgical treatment (n = 8), or best supportive care (n = 2).ConclusionsPreoperative diagnosis of appendiceal tumours is difficult. Nonoperative management of patients with acute, uncomplicated appendicitis potentially prevents the correct diagnosis of malignant appendiceal pathologies. Therefore, close follow-up or surgical removal of the appendix is mandatory.

Genomic and Pathologic Profiling of Very Well-Differentiated Gastric Adenocarcinoma of Intestinal Type: A Study With Emphasis on Diffuse-Type Transformation.

Very well-differentiated adenocarcinoma of intestinal type is a distinct subtype of gastric cancer characterized by anastomosing glands with a hand-in-hand pattern and low-grade cytologic atypia resembling intestinal metaplasia. This is a slow-growing neoplasm with an indolent clinical course; however, a subset demonstrates transformation into adenocarcinoma with higher-grade histology, typically diffuse-type carcinoma, and behaves aggressively. This study aimed to better characterize the genomic and pathologic features, with a focus on factors associated with diffuse-type transformation. A total of 58 cases with (n=31) and without (n=27) diffuse-type transformation were analyzed for molecular and pathologic features. First, comprehensive deep DNA sequencing was conducted in 18 cases (discovery cohort), followed by a digital droplet polymerase chain reaction of hot spot RHOA mutations in 40 cases (validation cohort). In total, RHOA mutations were the most common alteration (34%), followed by loss of ARID1A (12%), p53 alterations (10%), and CLDN18 :: ARHGAP26/6 fusions (3.4%). FGFR2 amplification was identified in an advanced case with a p53 alteration. Altered p53 expression was recognized only in higher-grade components and was significantly associated with advanced disease ( P =0.0015) and diffuse-type transformation ( P =0.026). A mixed mucin phenotype was also strongly correlated with advanced disease ( P <0.001) and diffuse-type transformation ( P <0.001). Decreased E-cadherin expression was frequently observed (74%) in poorly cohesive components. This study demonstrated that a subset of RHOA -mutant diffuse-type gastric cancers develops through the transformation of very well-differentiated adenocarcinoma of intestinal type. Our observations suggest a mixed mucin phenotype as a risk factor and alterations in p53 and E-cadherin as drivers of diffuse-type transformation.

Assessment of targeted therapy opportunities in sinonasal cancers using patient-derived functional tumor models

Patient-derived ex vivo tumor models representing four distinct sinonasal cancer subtypes: sinonasal intestinal-type adenocarcinoma, sinonasal neuroendocrine carcinoma, sinonasal undifferentiated carcinoma and SMARCB1 deficient sinonasal carcinoma were included in the analyses. Results of functional drug screens of 160 anti-cancer therapies were integrated with gene panel sequencing and histological analyses of the tumor tissues and the ex vivo cell cultures to establish associations between drug sensitivity and molecular characteristics including driver mutations. The different sinonasal cancer subtypes display considerable differential drug sensitivity. Underlying the drug sensitivity profiles, each subtype was associated with unique molecular features. The therapeutic vulnerabilities correlating with specific genomic background were extended and validated with in silico analyses of cancer cell lines representing different human cancers and with reported case studies of sinonasal cancers treated with targeted therapies. The results demonstrate the importance of understanding the differential biology and the molecular features associated with the different subtypes of sinonasal cancers. Patient-derived ex vivo tumor models can be a powerful tool for investigating these rare cancers and prioritizing targeted therapeutic strategies for future clinical development and personalized medicine.

Twelve years after: The french national network on rare head and neck tumours (REFCOR)

Rare cancers constitute less than 10% of head and neck cancers and lack sufficient evidence for standardized care. The French Rare Head and Neck Cancer Expert Network (REFCOR) as established a national database to collect data on these rare cancers. This study aims to describe patient and tumour characteristics in this database. Prospective data collection was conducted across multiple centers. Survival analyses were performed using Kaplan Meier method and Log Rank test. Odds ratios were used for comparing proportions. A total of 7208 patients were included over a period of 10years. The most frequent histologies were: Not Otherwise Specified (NOS) adenocarcinoma 13%, adenoid cystic carcinoma 12%, squamous cell carcinoma of rare locations 10%, mucoepidermoid carcinoma 9%, intestinal-type adenocarcinoma (8%). Tumours were located in sinonasal area (38%); salivary glands (32%); oral cavity / oropharynx / nasopharynx (16%); larynx / hypopharynx (3%); ears (1%); others (3%). Tumours were predominantly classified as T4 (23%), N0 (54%), and M0 (62%). Primary treatment approach involved tumour resection (78%) and / or radiotherapy (63%). Patients with salivary gland cancers exhibited better 5-year overall survival (OS) rates (p<0.05), and lower recurrence rates compared to patients with sinonasal, laryngeal/ hypopharyngeal cancers. No significant differences were observed in the other comparisons. Acinar cell carcinoma demonstrated the best OS while mucous melanoma had the poorest prognosis. Melanoma, carcinoma NOS, and sinonasal undifferenciated carcinoma still have poor prognoses. Efforts are being made, including training and guidelines, to expand network coverage (REFCOR, EURACAN), improve data collection and contribute to personalized therapies.

Sinonasal Adenocarcinoma: Clinicopathological Characterization and Prognostic Factors.

Sinonasal (SN) malignancies are rare. Within SN adenocarcinomas, the most frequent are intestinal-type adenocarcinomas (ITACs). ITAC has been associated with wood and leather dust occupational exposure and TP53 mutations. Not much information is available regarding its characterization and treatment. The aim of this study is to characterize the clinicopathologic and prognostic factors of patients with sinonasal adenocarcinomas (SNACs) treated in our tertiary-level hospital. A retrospective, consecutive study including SNAC patients diagnosed between 2004-2023 was conducted. Clinicopathological data was collected, and p53 status was assessed in the tumor specimens. The association between p53 status and clinicopathological variables, as well as their impact on survival, was evaluated. In total, 35 were included, most of them having ITAC (91.4%) with papillary subtype (37.5%); the majority were subjected to occupational risk exposure (82.9%). Overexpression of p53 was identified in 48.6% of the tumors. Papillary and colonic subtypes were associated with higher median progression-free survival (mPFS) than mucinous and solid subtypes (mPFS 37 months, 95% CI, 20.0-54.0, vs. 9 months, 95% CI, 7.15-10.85, p=0.01); the former was also associated with higher median overall survival (mOS) (mOS 64 months, 95% CI, 37.18-90.81 vs. 14 months, 95% CI, 0-41.58, p=0.02). Histologic grade 1-2 and macroscopic complete resection were associated with higher PFS (PFS of five months of 90.9% vs. 33.3%, p=0.01; mPFS of 37 months, 95% CI, 4.93-69.07 vs. 10 months, 95% CI, 6.43-13.57, p=0.04, respectively). Disease recurrence with distant metastases was associated with lower OS (11 months, 95% CI, 6.1-15.9 vs. 53 months, 95% CI, 22.70-83.30, p=0.04). This study reinforces the importance of protective occupational measures. Future studies will be important to validate the best treatment strategy in the advanced stages of this disease and also to identify new prognostic and/or therapeutic target biomarkers in SNAC.