Ocular surface squamous neoplasia in New Zealand: a ten-year review of incidence in the Waikato region.

BACKGROUND: Ocular surface squamous neoplasia (OSSN) is a relatively rare disease with a low mortality and highly variable incidence. Despite a high incidence of OSSN in the Southern hemisphere, there is limited epidemiological data for New Zealand. The current study aims to assess the incidence, demographics, and histological grade of OSSN in the Waikato region of New Zealand, home to ~10% of the population of New Zealand. METHODS: Non-interventional retrospective cohort study. All conjunctival biopsy histology reports from 2010 to 2019 in the Waikato region of New Zealand were analysed. Age, sex, and ethnicity were analysed and the incidence of OSSN was calculated. Main outcome measures included histological grade, rate of recurrence, and incidence of OSSN. RESULTS: A total of 386 patients underwent conjunctival biopsy with histology during the study period. Eighty-three lesions (22%) involving 80 patients (21%) were reported positive for OSSN. Patients with OSSN had a mean age of 68.9 years (SD = 13.2), were predominantly male (76%), and of New Zealand-European ethnicity (53%). Conjunctival intraepithelial neoplasia-1 (30%) was the most frequent diagnosis. Three patients (4%) had recurrent lesions requiring repeat biopsy. The peak annual OSSN incidence rate was 3.81/100,000 population in 2019. The overall ten-year incidence was 2.13/100,000 population/year. CONCLUSION: This is the largest study to investigate OSSN incidence in New Zealand. The incidence rate of OSSN is one of the highest rates reported in the literature.

Incidence of NUT carcinoma in Western Australia from 1989 to 2014: a review of pediatric and adolescent cases from Perth Children's Hospital.

BACKGROUND: NUT carcinoma (NC), previously known as NUT midline carcinoma, is a rare and very aggressive cancer that occurs in both children and adults. NC is largely chemoresistant, with an overall survival of less than 7 months. Because the carcinoma is not restricted to a particular organ, diagnosis is often a challenge. In the absence of a clearly determined incidence for NC, we sought to study the diagnosis of patients in a well-defined population. METHODS: We systematically reviewed records of all patients that presented to the Oncology Department of the Princess Margaret Hospital for Children from 1989 to 2014. This institution in the geographically isolated state of Western Australia has a catchment population of around 2 million. We then identified all high grade undifferentiated sarcomas or carcinomas in the 0-16 year age group. RESULTS: Over 26 years, we found 14 patients of 16 years or younger with undifferentiated malignant tumors. Of these, five tumors were positive by immunohistochemistry for the NUT/NUTM1 (Nuclear Protein in Testis) protein and/or the translocation t(15;19). Three patients presented with thoracic tumors, one with a para-spinal tumor, and one had an upper airway nasopharyngeal carcinoma. In all five cases, there was an initial response to therapy and then progression. This 26-year survey was conducted in a geographically isolated state with a well-defined population, and we determined an estimated incidence of NC of around 0.41 per million child years (0-16 yrs. of age) at risk. From three patients it was feasible to derive cell lines for further genetic analyses and drug screening. CONCLUSIONS: For the first time, the incidence of NC could be determined in a well-defined geographic area. The calculated rate of NC incidence is consistent with a history of under-recognition for this malignancy. These findings indicate that improved diagnostic detection of NC would enable better management and counselling of patients. Our findings emphasize the heterogeneity of NC, and they highlight the need to develop personalised therapy options, and to consider a diagnosis of NC in undifferentiated malignant tumors.

Prevalence and predictors of precancerous cervical lesions among HIV-positive women in Jos, north-central Nigeria.

OBJECTIVE: To determine the prevalence and predictors of precancerous cervical lesions among HIV-positive women in Jos, Nigeria. METHODS: A cross-sectional study was conducted from October 2017 to January 2018 among 326 HIV-positive women. Cervical smears were collected for examination at the AIDS Preventive Initiative of Nigeria clinics of Jos University Teaching Hospital (JUTH) and Bingham University Teaching Hospital (BhUTH), Jos, Nigeria. Demographic characteristics of participants were documented using a structured questionnaire. Data were entered and analyzed using SPSS version 21. RESULTS: Of the 326 participants, precancerous cervical lesions were present in 40 (12.2%) women: 4 (1.2%) had atypical squamous cells of undetermined significance, 19 (5.8%) had low-grade squamous intraepithelial lesions, 1 (0.3%) had atypical squamous cells cannot exclude high-grade squamous intraepithelial lesions, 13 (4.0%) had high-grade squamous intraepithelial lesions, and 3 (0.9%) had high-grade squamous intraepithelial lesions, suspected for invasion. The multivariate logistics regression model showed that parity (odds ratio 3.4, 95% confidence interval 1.3-9.5, P=0.043) was a significant predictor of precancerous cervical lesions. CONCLUSION: The prevalence of precancerous cervical lesions among HIV-infected women is relatively low compared to earlier reported prevalence in an HIV population in Jos. Increasing parity was a significant predictor.

Welding and the risk of head and neck cancer: the ICARE study.

OBJECTIVE: To investigate the association between occupational exposure to welding and the risk of head and neck cancer in a large French population-based case-control study, the Investigation of occupational and environmental CAuses of REspiratory cancers study. METHODS: Analyses were restricted to men (2703 controls and 1588 cases of squamous-cell carcinoma of the oral cavity, oropharynx, hypopharynx and larynx). Welding activity and potential confounders were assessed by detailed questionnaires. ORs and CIs (95% CI) were estimated by unconditional logistic regression, adjusted for age, area of residence, tobacco smoking, alcohol consumption and occupational exposure to asbestos. RESULTS: Welding was associated with an increased risk of head and neck cancer overall (OR=1.31, 95% CI 1.03 to 1.67). The association was strongest for laryngeal cancer (OR=1.66, 95% CI 1.15 to 2.38) and the risk increased with the cumulative duration (p-trend <0.01) and the weighted duration (p-trend <0.01) of welding. A cumulative duration and a weighted duration of welding of more than 10 years were also associated with a significantly increased risk of oral cancer (OR=1.82, 95% CI 1.09 to 3.04; OR=2.10, 95% CI 0.99 to 4.45, respectively). A long duration of arc welding was associated with laryngeal cancer, whereas a long duration of spot welding was associated with oral cancer. Welding was not associated with the risk of oropharyngeal and hypopharyngeal cancer. CONCLUSION: Our findings suggest that welding and several welding-related tasks increase the risk of laryngeal cancer and to a lesser extent oral cancer.

A Meta-Analysis on Human Papillomavirus Type Distribution among Women with Cervical Neoplasia in the WHO Eastern Mediterranean Region.

INTRODUCTION: To date, the human papillomavirus (HPV) vaccine has not been integrated into the national vaccination program of most countries of the WHO Eastern Mediterranean Region (EMRO), except for the United Arab Emirates and Libya. The knowledge of HPV genotype distribution in cervical neoplasia is valuable to predict the impact of current HPV vaccines on cancer prevention and can help the health policymakers to select the most appropriate vaccine types in their countries. METHODS: Hence, this meta-analysis recapitulates all available data on HPV prevalence and genotypes in women with atypical squamous cells of undetermined significance (ASCUS), cervical intraepithelial neoplasia (CIN) I-III or low- and high-grade squamous intraepithelial lesions (LSIL and HSIL, respectively), and invasive cervical cancer (ICC) in EMRO countries. RESULTS: The meta-analysis included 5,990 cases of cervical precancer and cancer. The overall HPV prevalence was 85.4, 71.3, 59.2, and 34.8% in women with ICC, CIN II-III or HSIL, CIN I or LSIL, and ASCUS, respectively. HPV 16 was the most common genotype followed by HPV 18, representing 58 and 16.5% in ICC cases, respectively. CONCLUSION: This meta-analysis showed that the introduction of current HPV vaccines into national vaccination programs and the establishment of comprehensive screening programs in EMRO countries is beneficial by preventing 74.5% of cervical neoplasia.

The relation between systemic inflammation and incident cancer in patients with stable cardiovascular disease: a cohort study.

AIMS: Low-grade inflammation, measured by elevated plasma concentrations of high-sensitive C-reactive protein (CRP), is a risk factor for cardiovascular disease (CVD). There is evidence that low-grade inflammation is also related to a higher risk of cancer. The present prospective cohort study evaluates the relation between low-grade systemic inflammation and risk of cancer in patients with stable CVD. METHODS AND RESULTS: In total, 7178 patients with stable CVD and plasma CRP levels ≤10 mg/L were included. Data were linked to the Dutch national cancer registry. Cox regression models were fitted to study the relation between CRP and incident CVD and cancer. After a median follow-up time of 8.3 years (interquartile range 4.6-12.3) 1072 incident cancer diagnoses were observed. C-reactive protein concentration was related to total cancer [hazard ratio (HR) 1.35; 95% confidence interval (CI) 1.10-1.65] comparing last quintile to first quintile of CRP. Especially lung cancer, independent of histopathological subtype, was related to CRP (HR 3.39; 95% CI 2.02-5.69 comparing last to first quintile of CRP). Incidence of epithelial neoplasms and especially squamous cell neoplasms were related to CRP concentration, irrespective of anatomical location. Sensitivity analyses after excluding patients with a cancer diagnosis within 1, 2, and 5 years of follow-up showed similar results. No effect modification was observed by smoking status or time since smoking cessation (P-values for interaction > 0.05). CONCLUSION: Chronic systemic low-grade inflammation, measured by CRP levels ≤10 mg/L, is a risk factor for incident cancer, markedly lung cancer, in patients with stable CVD. The relation between inflammation and incident cancer is seen in former and current smokers and is uncertain in never smokers.

Validation of a Clinical Cancer Register at the Head and Neck Oncology Center in Örebro.

BACKGROUND: This was a validation study of a regional register of oral cancer in Örebro, Sweden. The purpose was to assess the rate of errors in baseline, and treatment, and the completeness and accuracy of data on recurrences. MATERIALS AND METHODS: A total of 653 cases with squamous cell cancer in the oral cavity were identified from the register. A randomized sample of 73 (11%) was selected, and a set of relevant data was compared to medical records. RESULTS: Data on patient and tumour characteristics showed high accuracy, with 98% correct data and more than 99% of treatment data were correct. Follow-up data had a higher rate of errors, with 23% of recurrences not recorded, 13.6% misclassified, and 9.1% of cases showing errors in timing of the recurrence. CONCLUSION: data concerning patients, tumour status, and treatment in the Regional Head and Neck Register in Örebro are highly accurate. However, the follow-up data contain a higher rate of errors, that must be taken into consideration when evaluating outcome after treatment.

Plasma selenoprotein P concentration and lung cancer risk: results from a case-control study nested within the Shanghai Men's Health Study.

Selenoprotein P (SELENOP) is a major selenoenzyme in plasma and linked to antioxidant properties and possibly to lung cancer; however, supporting evidence is limited. We investigated the association between pre-diagnostic plasma SELENOP concentration and lung cancer risk in a case-control study of 403 cases and 403 individually matched controls nested within the Shanghai Men's Health Study. SELENOP concentration in pre-diagnostic plasma samples was measured by a sandwich enzyme-linked immunosorbent assay. Cases were diagnosed with lung cancer between 2003 and 2010. Multivariate conditional logistic regression was used to estimate odds ratios (OR) and the corresponding 95% confidence intervals (CI) for studying the association between plasma SELENOP concentration and lung cancer risk. Cases had slightly lower plasma SELENOP concentration than controls (4.3 ± 1.2 versus 4.4 ± 1.1 mg/l, P difference = 0.09). However, the multivariate analysis showed no association between plasma SELENOP concentration and lung cancer risk among all participants (OR = 1.08, 95% CI = 0.54-2.14 for quartile 4 versus quartile 1), or by smoking status or tumor aggressiveness. In contrast, although the number of cases was limited, plasma SELENOP concentration was positively associated with lung adenocarcinoma risk (OR = 5.38, 95% CI = 1.89-15.35 for tertile 3 versus tertile 1), but not with squamous cell lung carcinoma (OR = 1.69, 95% CI = 0.43-6.70). Our study of adult men living in selenium non-deficient areas in China provides little support for the inverse association between pre-diagnostic plasma SELENOP concentration and lung cancer risk. Our finding of a positive association with risk of lung adenocarcinoma needs to be interpreted with caution.

[Cervical cancer has increased in Sweden in women who had a normal cell sample]. / Livmoderhalscancer ökar hos kvinnor med normalt cellprov - Skillnader mellan laboratorier tyder på att ökningen orsakas av faktorer som kan åtgärdas.

Cervical cancer has increased in Sweden in recent years. The increase is 17% in 2014-15 compared to the reference period 2002-13. The increase is largest for adenocarcinoma (+ 31%) and shows remarkable differences between counties, from continued incidence decreases to increases of >80%. The increase is seen in most ages that are offered screening, but is confined to early stage cancers and there is no increase in mortality. Population test coverage of screening has increased since 2002. The Swedish National Cervical Screening Registry has analysed the increase in relation to screening history. The most significant increase (+ 30%) is seen in women who had a normal cervical smear (P < 0.0001) in the preceding screening interval. The cancer risk for women who previously had a high grade abnormality has also increased (P = 0.0009). Data from several laboratories still show very low cancer risk following normal cytology, indicating that the increase is related to factors that can be addressed. All data on re-review of samples taken before cancer and high grade intraepithelial neoplasia will be requested and nationally analyzed to further elucidate the exact cause.

Decision tree analysis to stratify risk of de novo non-melanoma skin cancer following liver transplantation.

PURPOSE: Non-melanoma skin cancer (NMSC) is the most common de novo malignancy in liver transplant (LT) recipients; it behaves more aggressively and it increases mortality. We used decision tree analysis to develop a tool to stratify and quantify risk of NMSC in LT recipients. METHODS: We performed Cox regression analysis to identify which predictive variables to enter into the decision tree analysis. Data were from the Organ Procurement Transplant Network (OPTN) STAR files of September 2016 (n = 102984). RESULTS: NMSC developed in 4556 of the 105984 recipients, a mean of 5.6 years after transplant. The 5/10/20-year rates of NMSC were 2.9/6.3/13.5%, respectively. Cox regression identified male gender, Caucasian race, age, body mass index (BMI) at LT, and sirolimus use as key predictive or protective factors for NMSC. These factors were entered into a decision tree analysis. The final tree stratified non-Caucasians as low risk (0.8%), and Caucasian males > 47 years, BMI < 40 who did not receive sirolimus, as high risk (7.3% cumulative incidence of NMSC). The predictions in the derivation set were almost identical to those in the validation set (r2 = 0.971, p < 0.0001). Cumulative incidence of NMSC in low, moderate and high risk groups at 5/10/20 year was 0.5/1.2/3.3, 2.1/4.8/11.7 and 5.6/11.6/23.1% (p < 0.0001). CONCLUSIONS: The decision tree model accurately stratifies the risk of developing NMSC in the long-term after LT.

Descriptive epidemiological study of vaginal cancer using data from the Osaka Japan population-based cancer registry: Long-term analysis from a clinical viewpoint.

Vaginal cancer is such a rare tumor that epidemiological and clinical information for it is based mainly on studies of small numbers of cases. The aim of the present study was to perform a descriptive epidemiological analysis of vaginal cancer using a significantly larger population-based dataset from the Japanese Osaka Cancer Registry.The age-standardized incidence of vaginal cancer per 1,000,000 persons, from 1976 to 2010, was calculated and examined for trends. Relative-survival analysis was applied to estimate a more up-to-date 10-year period calculation, using data from recently followed-up patients. The conditional 5-year survival of patients who survived for 0 to 5 years after diagnosis was calculated.A total of 481 cases of vaginal cancer were registered in Osaka during the 35-year period from 1976 to 2010. The age-adjusted incidence rate has significantly and consistently decreased over this time [annual percent change (APC) = -1.29, 95% confidence interval (95% CI): -0.3 ∼ -2.2]; however, due to significant population aging, the raw incidence of vaginal cancer appeared to have been increasing. The 10-year relative survival of patients with surgery-based treatments was comparable to that of radiation-based treatments, implying that surgery and radiotherapy provide similar therapeutic benefits (P = .98). The 10-year relative survival was not significantly different during the period of 1976 to 2000 compared with the period of 2001 to 2008, although there has been, in the latter period, a tendency for improvement of long-term survival, especially for survival longer than 5 years. The longer the time after diagnosis, the higher the conditional 5-year relative-survival at 0 to 4 years after diagnosis.The age-adjusted incidence of vaginal cancer has decreased since 1976. Regrettably, the 10-year survival rate did not similarly improve, and it remained stable during the period from 2001 to 2008, compared with the period from 1976 to 2000, indicating that significant work remains to be done to develop more effective vaginal cancer treatments.

Increased PTPRA expression leads to poor prognosis through c-Src activation and G1 phase progression in squamous cell lung cancer.

PTPRA is reported to be involved in cancer development and progression through activating the Src family kinase (SFK) signaling pathways, however, the roles of PTPRA in the squamous cell lung cancer (SCC) development are unclear. The purpose of this study was to clarify the clinical relevance and biological roles of PTPRA in SCC. We found that PTPRA was upregulated in squamous cell lung cancer compared to matched normal tissues at the mRNA (N=20, P=0.004) and protein expression levels (N=75, P<0.001). Notably, high mRNA level of PTPRA was significantly correlated with poorer prognosis in 675 SCC patients from the Kaplan-Meier plotter database. With 75 cases, we found that PTPRA protein expression was significantly correlated with tumor size (P=0.002), lymph node metastasis (P=0.008), depth of tumor invasion (P<0.001) and clinical stage (P<0.001). The Kaplan-Meier plot suggested that high expression of PTPRA had poorer overall survival in SCC patients (P=0.009). Multivariate Cox regression analysis suggested that PTPRA expression was an independent prognostic factor in SCC patients. In the cellular models, PTPRA promotes SCC cell proliferation through modulating Src activation as well as cell cycle progression. In conclusion, higher PTPRA level was associated with worse prognosis of SCC patients and PTPRA could promote the cell cycle progression through stimulating the c-Src signaling pathways.

New Developments and Challenges in Rare Genitourinary Tumors: Non-Urothelial Bladder Cancers and Squamous Cell Cancers of the Penis.

The diagnosis and treatment of rare genitourinary tumors is inherently challenging. The Rare Diseases Act of 2002 initially defined a rare disorder as one that affects fewer than 200,000 Americans. The lack of widely available clinical guidelines, limited research funding, and inaccessible clinical trials often lead to difficulty with treatment decisions to guide practitioners in rendering effective care for patients with rare genitourinary cancers. This article will discuss basic tenets of diagnosis and treatment as well as recent developments and clinical trials in rare non-urothelial bladder cancers and penile squamous cell cancers.

Biology and patterns of response to EGFR-inhibition in squamous cell cancers of the lung and head & neck.

The identification of common molecular aberrations that drive cancer progression has led to targeted therapies that improve treatment efficacy in many tumor types. Epidermal growth factor receptor (EGFR) inhibitors have been used to treat both lung and head and neck cancers with squamous cell histology. These tumors often show high EGFR expression and/or increased gene copy number, but low incidence of the activating kinase domain mutations common to adenocarcinomas of the lung. In this manuscript, we review clinical trial data on EGFR-inhibitors in the treatment of squamous cell carcinoma (SqCC) of the lung and head and neck (SCCHN), including both efficacy and biomarker analyses. Although some efficacy with use of EGFR inhibitors is observed, the level of benefit varies within and across tumor types, and the predictive capacity of high EGFR protein expression and/or gene amplification, if any, is limited. Due to the lack of candidate biomarkers that consistently predict response to EGFR-inhibitor therapy across treatment setting and class of agent in SqCC of the lung and SCCHN, we explore the biology, genomics and patterns of response to EGFR-inhibitors to inform identification of potential biomarkers, highlighting several key molecules that have shown promise in preclinical studies and clinical trials across multiple cancer sites.

Long Term Outcome of Routine Image-enhanced Endoscopy in Newly Diagnosed Head and Neck Cancer: a Prospective Study of 145 Patients.

Synchronous second primary tumors (SPTs), especially esophageal squamous cell neoplasia (ESCN), in patients with head and neck squamous cell carcinoma (HNSCC) are not uncommon. Image-enhanced endoscopy (IEE) screening may identify SPTs while there is no evidence to support its benefit. We prospectively recruited an adult cohort with newly-diagnosed HNSCC for IEE screening of upper gastrointestinal (UGI) tract neoplasia. 145 HNSCC patients were recruited. 22 (15.2%) patients had synchronous UGI tract neoplasia, including 20 ESCNs and 2 gastric adenocarcinoma. At a median follow-up of 2.72 (±1.73) years, the 3-year overall survival (OS) rate was 0.71. HNSCC patients with synchronous ESCN/UGI tract neoplasia had poorer prognosis than those without (multivariate analysis, hazard ratio [HR] 2.75/2.79, 95% confidence interval [CI] 1.11~6.82/1.15~6.80, p = 0.03/0.02). HNSCC patients with advanced (stage III&IV) ESCN had worst survivals (p < 0.001). Among those with synchronous ESCNs, hypopharyngeal cancers were associated with poorer prognosis when compared with oral cancers (HR 2.36, 95% CI 1.08~5.15, p = 0.03). IEE screening for UGI SPTs in HNSCC patients could be used for risk stratification and prognosis prediction. HNSCC patients with advanced ESCN had the worst prognosis. Further studies are needed to demonstrate the survival benefits from IEE screening.

Natural Antibodies to Human Papillomavirus 16 and Recurrence of Vulvar High-Grade Intraepithelial Neoplasia (VIN3).

OBJECTIVES: Approximately 30% of women treated for squamous high-grade intraepithelial neoplasia (VIN3), often associated with human papillomavirus (HPV), have recurrent disease. In this study, we assess predictors of recurrence that may provide targets for early prevention or treatment. MATERIALS AND METHODS: Women with VIN3 who participated in a previous population-based case-control study with blood and tumor samples completed a follow-up telephone interview an average of 5 years after initial diagnosis. The risk of recurrence was determined by proportional hazards modeling. RESULTS: Women with VIN3 in the follow-up study (n = 65) were similar to women with VIN3 in the parent study (n = 215) with regard to age at primary diagnosis, level of current cigarette smoking (>60%), and lifetime number of partners. We found that 22 (33.8%) of 65 participants had a vulvar recurrence and that 73.4% recurred within 3 years of treatment. Recurrences occurred more often among women with common warts in the decade before diagnosis (hazard ratio [HR] = 2.5, 95% CI = 1.1-5.8) and among those with a previous anogenital cancer (HR = 2.7, 95% CI = 1.2-6.3). Interestingly, recurrence was less frequent among women who mounted a natural antibody response to HPV16 (HR = 0.4, 95% CI = 0.2-0.9). CONCLUSIONS: These data provide strong preliminary evidence that VIN3 recurrence was less frequent among those with HPV16 antibodies. Vaccination with the currently licensed HPV vaccine as part of adjunctive therapy for VIN3 would increase antibody response and may decrease risk of recurrence. Randomized controlled trials are needed to determine whether HPV vaccination is effective against VIN3 recurrence.

Multidimensional analysis of the effect of occupational exposure to organic solvents on lung cancer risk: the ICARE study.

BACKGROUND: The association between lung cancer and occupational exposure to organic solvents is discussed. Since different solvents are often used simultaneously, it is difficult to assess the role of individual substances. OBJECTIVES: The present study is focused on an in-depth investigation of the potential association between lung cancer risk and occupational exposure to a large group of organic solvents, taking into account the well-known risk factors for lung cancer, tobacco smoking and occupational exposure to asbestos. METHODS: We analysed data from the Investigation of occupational and environmental causes of respiratory cancers (ICARE) study, a large French population-based case-control study, set up between 2001 and 2007. A total of 2276 male cases and 2780 male controls were interviewed, and long-life occupational history was collected. In order to overcome the analytical difficulties created by multiple correlated exposures, we carried out a novel type of analysis based on Bayesian profile regression. RESULTS: After analysis with conventional logistic regression methods, none of the 11 solvents examined were associated with lung cancer risk. Through a profile regression approach, we did not observe any significant association between solvent exposure and lung cancer. However, we identified clusters at high risk that are related to occupations known to be at risk of developing lung cancer, such as painters. CONCLUSIONS: Organic solvents do not appear to be substantial contributors to the occupational risk of lung cancer for the occupations known to be at risk.

Sphenoid sinus malignancies: a population-based comprehensive analysis.

BACKGROUND: Sinonasal malignancies are rare, representing less than 1% of all cancers, with the sphenoid sinus accounting for 1% to 2% of these cases. Sphenoid sinus malignancies exhibit very poor outcomes. There is a paucity of literature describing their histopathological features, incidence trends, treatment, and survival. We seek to elucidate these factors using a national population-based resource. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify malignant sphenoid sinus tumors. The results were analyzed for demographics, incidence, and clinicopathologic trends. Survival was calculated using Kaplan-Meier analysis. RESULTS: The search identified 472 cases. The mean and median age at diagnosis was 60.0 years. Males represented 54.9% of cases. By race/ethnicity, 82.4% were white and 8.5% were black. The four most common histopathologies were squamous cell neoplasms (29.4%), adenocarcinomas (14.4%), non-Hodgkin's mature B-cell lymphomas (13.1%), and unspecified epithelial neoplasms (11.0%). The overall incidence from 2000 to 2012 was 0.030 per 100,000. Kaplan-Meier analysis demonstrated an overall 5-year disease-specific survival (DSS) of 48.1%. Of the most common histopathological subtypes, 5-year DSS was best for mature B-cell NHL (64.0%) and worst for unspecified epithelial neoplasms (25.6%). CONCLUSION: Sphenoid sinus malignancies are rare, with high prevalence in white males. The most common histopathology is squamous cell neoplasms. They exhibit significant locoregional extension. Of the common sphenoid sinus malignant subtypes, 5-year DSS is best for mature B-cell NHL and worst for unspecified epithelial neoplasms.