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      Adenoid Cystic Carcinoma of the Bartholin's Gland: A Diagnostic Dilemma

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          Abstract

          Adenoid cystic carcinomas of the Bartholin's gland are extremely rare and are often misdiagnosed. There are currently no definite treatment guidelines. This article describes the case of a 33-year-old female who was managed at our centre for adenoid cystic carcinoma of the Bartholin's gland. She presented with a prolonged history of a vulvar lesion which was eventually diagnosed as adenoid cystic carcinoma of the Bartholin's gland. She was subsequently treated with wide local excision of the primary and inguinal lymph node dissection followed by adjuvant radiotherapy and chemotherapy. She had gross perineural invasion on MRI imaging. The present case highlights the diagnostic dilemma in this extremely rare cancer and the literature further explores the natural history and treatment options.

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          Most cited references33

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          Adenoid cystic carcinoma of the head and neck: Incidence and survival trends based on 1973-2007 Surveillance, Epidemiology, and End Results data.

          Adenoid cystic carcinoma (ACC) of the head and neck (ACCHN) is a rare tumor of minor salivary, parotid, and submandibular glands. The biologic behavior of the disease is poorly understood, and nonsurgical treatment strategies have yet to be standardized. The long-term prognosis continues to be guarded, with an estimated 10-year survival of <60%. Population-based studies examining ACC are scarce. The authors aimed to analyze incidence rates and survival outcomes for patients diagnosed with ACCHN using national population-based data. Data were obtained from the US National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program. Newly diagnosed ACCHN cases reported to SEER from 1973 through 2007 were categorized according to their sex, race, age, year of diagnosis, marital status, treatment interventions, primary tumor site, and disease stage. Incidence of ACCHN and postdiagnosis survival were examined over time and compared across different demographic and disease-related categories. The authors identified 3026 patients with ACCHN. The mean age at diagnosis among those cases was 57.4 years (range, 11-99 years). Analyses of incidence data demonstrated a decline in ACCHN rates between 1973 and 2007, noted across all sexes and races with no detectable inflexion points. The overall 5-year, 10-year, and 15-year survival outcomes for ACCHN patients were 90.3%, 79.9%, and 69.2%, respectively. Females, patients with localized disease, and younger patients were found to have significantly better survival across all time periods (all comparison-specific log-rank P values <0.001). Multivariate analyses revealed better prognosis among women compared with men (hazard ratio [HR], 0.73; 95% confidence interval [CI], 0.65-0.82), among married compared with unmarried individuals (HR, 0.81; 95% CI, 0.71-0.91), with certain sites of origin and stage of disease (HR, 2.788; 95% CI, 2.36-3.29), and in those who had surgery of the primary tumor site (HR, 0.45; 95% CI, 0.37-0.54). The overall incidence of ACC is declining. The noted differences in survival based on sex, marital status, site of origin, and treatment intervention require further investigation. Copyright © 2012 American Cancer Society.
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            Adenoid cystic carcinoma of breast: Recent advances.

            Adenoid cystic carcinoma (ACC) of the breast is a rare special subtype of breast cancer characterized by the presence of a dual cell population of luminal and basaloid cells arranged in specific growth patterns. Most breast cancers with triple-negative, basal-like breast features (i.e., tumors that are devoid of estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 expression, and express basal cell markers) are generally high-grade tumors with an aggressive clinical course. Conversely, while ACCs also display a triple-negative, basal-like phenotype, they are usually low-grade and exhibit an indolent clinical behavior. Many discoveries regarding the molecular and genetic features of the ACC, including a specific chromosomal translocation t(6;9) that results in a MYB-NFIB fusion gene, have been made in recent years. This comprehensive review provides our experience with the ACC of the breast, as well as an overview of clinical, histopathological, and molecular genetic features.
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              Factors influencing survival rate in adenoid cystic carcinoma of the salivary glands.

              Ninety-one cases of adenoid cystic carcinoma (ACC) of the salivary glands with more than ten years' follow up were studied to investigate factors influencing the survival rate of patients, which vary according to site, histological type, clinical stage and nature of therapy. The data were statistically analysed for survival curves. Log rank tests were employed to assess the statistical significance of various groups. As a result, it may be concluded that tumour site, clinical stage and histological type are the important factors influencing the prognosis. ACC of the palate and parotid, early clinical stage, glandular/tubular histological type, and tumour without nerve involvement had the best prognosis. ACC in the submandibular gland, maxillary antrum and tongue, advanced clinical stage (stage III and IV), solid histological type, and tumour with nerve involvement had a poor prognosis.
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                Author and article information

                Contributors
                Journal
                Case Rep Obstet Gynecol
                Case Rep Obstet Gynecol
                CRIOG
                Case Reports in Obstetrics and Gynecology
                Hindawi
                2090-6684
                2090-6692
                2019
                18 August 2019
                : 2019
                : 1784949
                Affiliations
                1Faculty of Health and Medicine, University of Newcastle, Newcastle, New South Wales, Australia
                2Central Coast Cancer Centre, Gosford District Hospital, Gosford, New South Wales, Australia
                3John Hunter Hospital, Newcastle, New South Wales, Australia
                Author notes

                Academic Editor: John P. Geisler

                Author information
                https://orcid.org/0000-0002-7319-2352
                https://orcid.org/0000-0001-8798-1638
                https://orcid.org/0000-0002-0345-3924
                Article
                10.1155/2019/1784949
                6720055
                05dbaff3-7415-4f0c-ab09-58520d50b1b6
                Copyright © 2019 Charmaine C. W. Lo et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 31 March 2019
                : 22 July 2019
                Categories
                Case Report

                Obstetrics & Gynecology
                Obstetrics & Gynecology

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