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Background: Germline CDKN2A mutations are a well-known cause of familial atypical multiple mole melanoma (OMIM #155601) and melanoma-pancreatic cancer syndrome (OMIM #606719). Increased risk of head and neck squamous cell carcinoma (HNSCC), particularly oral squamous cell carcinoma (OSCC) in those with germline CDKN2A mutations, has been described. However, screening for HNSCC is not a routine practice in patients with CDKN2A germline mutations and these mutations are not a conventional test for HNSCC patients without obvious risk factors., Case Presentation: We describe a female with no smoking history who developed oral squamous cell carcinoma at age 39 and had a complex clinical course of recurrent multifocal squamous cell carcinoma (SCC) and carcinoma in situ of the oral cavity and oropharynx. Detailed family history demonstrated that her mother was diagnosed with OSCC and melanoma in her 40 s, and her maternal grandfather was diagnosed with metastatic melanoma in his 40 s. Genetic testing of the patient and her mother revealed CDKN2A c.301G>T mutation. She was referred to genetic counseling as well as to dermatology, gastroenterology, and neurology for cancer surveillance. She was treated with resections and has no evidence of disease 3 years after diagnosis., Conclusions: We report a family with a CDKN2A c.301 G>T mutation who also have significant history of OSCC, adding to the growing body of literature suggesting increased risk of HNSCC, particularly OSCC, in CDKN2A germline mutation carriers. It is important to consider CDKN2A mutation testing in familial HNSCC and young patients without obvious risk factors. Moreover, surveillance for HNSCC should be routine practice in those with a CDKN2A germline mutation., (© 2022. The Author(s).)

Background: Oral cavity carcinomas individually are the fifth-leading cause of overall cancer mortality in the Northern Mariana Islands, which is likely a representative statistic for many other betel-nut-endemic Pacific islands. Factors associated with survival have been minimally evaluated in this region. The purpose of this study is to further characterize oral cavity carcinoma outcomes and associated prognostic factors in the United States commonwealth of the Northern Mariana Islands (CNMI)., Methods: A single-institution retrospective review was undertaken for 81 patients diagnosed with head and neck cancers at the CNMI's only regional hospital complex from 2005 to 2019. A subset of patients diagnosed with oral cavity carcinoma was further evaluated for survival outcomes. Cox proportional hazard regressions were performed to evaluate for variables associated with survival., Results: A majority of patients had cancer of the oral cavity (64/81, 79%). Fifty-five of these patients had sufficient data for review. The average age at the time of diagnosis was 48 and over half were diagnosed with stage IV disease (29/55, 53%). Five-year overall survival (OS) was 49.5% (95% CI, 33.3-63.7%). Factors associated with worse OS were lymph node metastases at presentation ( P  = .031), higher overall stage (III or IV vs I or II, P  = .016), and higher T-stage (III or IV vs I or II, P  = .027). Those who used betel nut were diagnosed at a significantly younger age than those who did not (47.2 vs 55.4, P  = .001)., Conclusions: The head and neck cancer burden in the CNMI is dominated by betel nut related oral cavity disease that is characterized by delayed presentations in younger patients and decreased OS. Future studies are indicated to improve health literacy as well as to investigate the potential for screening programs.

Areca nut use is a cause of higher rates of oral cavity cancer in the Commonwealth of the Northern Mariana Islands (CNMI). Little is known about patient insights into the risks of areca nut use worldwide. The purpose of this study is to evaluate perceptions of areca nut use and oral cancer among chewers in the CNMI. This is a survey study undertaken at the CNMI's only regional health center-300 adult participants completed a 21-question survey that assessed demographics, chewing behaviors, perceptions of areca nut use and oral cancer, and the willingness to participate in cessation and screening programs. Data was analyzed using chi-squared tests, at a significance value of P < .05. The participant average age was 38, and 41% were male. Almost all (92%) knew that chewing areca nut causes oral cancer, but only 13% correctly identified the actual areca nut as a carcinogen. About half (59%) believed that oral cancer could be treated. Most people (74%) were willing to participate in screening programs for oral cancer. Those who chewed areca nut daily were more likely to be interested in medicated replacement products relative to those who chewed less frequently ( P = .048). In conclusion, there are drastic misperceptions about areca nut and oral cancer in the CNMI. Efforts should be made towards promoting awareness of the carcinogenicity of the actual areca nut, and the treatability of oral cancer. Mandated educational warnings should be required with areca nut sales. Further research evaluating substitution methods and screening programs is indicated., (©Copyright 2020 by University Health Partners of Hawai‘i (UHP Hawai‘i).)

Purpose: Betel nut consumption contributes to higher rates of oral cavity cancer throughout Micronesia. The purpose of this study is to review local surveys and cancer data to further characterize these issues in the Northern Mariana Islands (CNMI)., Methods: Two commonwealth-wide health inquiries were reviewed: The Non-Communicable Diseases Survey (NCDS), 2016 and The Youth Risk Behavior Survey (YRBS), 2013. Data pertaining to betel nut, tobacco and alcohol use was extracted. Relevant cancer data from the Commonwealth Healthcare Corporation (CHC) of Saipan and the Surveillance, Epidemiology, and End Results (SEER) databases was assessed., Results: Betel nut chewing was reported by 43% of Asian Pacific Islander (API) adults, with 88% adding tobacco to the chew. Adults aged 20-30 had significantly higher rates of chewing relative to older groups (p < .0001). Tobacco smoking and alcohol use were reported by 25% and 23% of adults, respectively. Betel nut chewing was reported by 33% of high school students. From 2007 to 2016, oral cavity cancers contributed to 9% of all cancer diagnoses and 13% of cancer-related mortalities. SEER data supported oral cavity cancer diagnoses at younger ages in APIs., Conclusion: These results demonstrate concerning trends regarding alcohol, tobacco and betel nut use in the CNMI. Betel nut use is prevalent among APIs of nearly all ages, with the majority adding tobacco to their chew. The available data suggests a drastic oral cavity cancer burden in the CNMI. Efforts should be made to evaluate for effective means of primary and secondary prevention in API regions., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective The National Cancer Center Network recommends starting radiation therapy within 6 weeks after surgery for oral cavity squamous cell carcinoma (OCSCC), but there is limited evidence of the importance of the total time from surgery to completion of radiation therapy (package time). We set out to determine if there was an association between package time and survival in OCSCC and to evaluate the impact of treatment location on outcomes. Study Design Retrospective cohort study. Setting Tertiary academic medical center. Subjects and Methods We reviewed the records of patients with OCSCC who completed postoperative radiation therapy at an academic medical center from 2008 to 2016. The primary endpoints were overall survival and recurrence-free survival. Statistical analysis included χ 2 tests and Cox proportional hazards regressions. Results We identified 132 patients with an average package time of 12.6 weeks. On multivariate analysis, package time >11 weeks was independently associated with decreased overall survival (hazard ratio, 6.68; 95% CI, 1.42-31.44) and recurrence-free survival (hazard ratio, 2.94; 95% CI, 1.20-7.18). Patients who received radiation therapy at outside facilities were more likely to have treatment delays (90.2% vs 62.9%, P = .001). Conclusions Prolonged package times are associated with decreased overall and recurrence-free survival among patients with OCSCC. Patients who received radiation therapy at outside facilities are more likely to have prolonged package times.