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      Call for Papers: Beyond Biology: The Crucial Role of Sex and Gender in Oncology

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      About Oncology Research and Treatment: 2.4 Impact Factor I 3.3 CiteScore I 0.495 Scimago Journal & Country Rank (SJR)

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      Adenoid Cystic Carcinoma of Bartholin’s Gland: What Is the Best Approach?

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          Abstract

          Background and summary: Among all vulvar cancers, primary adenoid cystic carcinoma (ACC) of Bartholin’s gland is a very rare tumor characterized by a slow growth, a high local aggressiveness, and a remarkable recurrence rate. Due to its rarity, treatment remains a challenge for oncologists and gynecological surgeons. Key message: The present paper reports clinical, radiological, and histological features of ACC of Bartholin’s gland and reviews the literature data on the treatment options with a particular focus on the potential role of particle radiation therapy.

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          Most cited references73

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          Whole exome sequencing of adenoid cystic carcinoma.

          Adenoid cystic carcinoma (ACC) is a rare malignancy that can occur in multiple organ sites and is primarily found in the salivary gland. While the identification of recurrent fusions of the MYB-NFIB genes have begun to shed light on the molecular underpinnings, little else is known about the molecular genetics of this frequently fatal cancer. We have undertaken exome sequencing in a series of 24 ACC to further delineate the genetics of the disease. We identified multiple mutated genes that, combined, implicate chromatin deregulation in half of cases. Further, mutations were identified in known cancer genes, including PIK3CA, ATM, CDKN2A, SF3B1, SUFU, TSC1, and CYLD. Mutations in NOTCH1/2 were identified in 3 cases, and we identify the negative NOTCH signaling regulator, SPEN, as a new cancer gene in ACC with mutations in 5 cases. Finally, the identification of 3 likely activating mutations in the tyrosine kinase receptor FGFR2, analogous to those reported in ovarian and endometrial carcinoma, point to potential therapeutic avenues for a subset of cases.
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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 the head and neck: predictors of morbidity and mortality.

              To review 160 patients treated at a single institution for adenoid cystic carcinoma during the 20 years between 1977 and 1996, applying a consistent treatment of surgery and postoperative radiation therapy to 140 patients. To analyze factors governing treatment failure, treatment-related morbidity, and mortality. Retrospective review. Tertiary referral center. Seventy-seven males and 83 females aged 13 to 89 years (average age, 49.5 years). Combined treatment yielded an 85% locoregional freedom from relapse and disease-specific survival at 5, 10, and 15 years was 89%, 67.4%, and 39.6%, respectively. Thirty-five patients (21.9%) had distant metastases as the only site of failure. Patients treated for paranasal sinus tumor experienced the most treatment-related morbidity vs other sites. Perineural invasion of major nerves, positive margins at surgery, and solid histological features were associated with increased treatment failures. Four or more symptoms present at diagnosis, positive lymph nodes, solid histology, and perineural invasion of major nerves were associated with increased mortality from disease.
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                Author and article information

                Journal
                OCL
                Oncology
                10.1159/issn.0030-2414
                Oncology
                S. Karger AG
                0030-2414
                1423-0232
                2020
                August 2020
                14 May 2020
                : 98
                : 8
                : 513-519
                Affiliations
                [_a] aNational Center of Oncological Hadrontherapy (Fondazione CNAO), Pavia, Italy
                [_b] bDepartment of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy
                [_c] cDepartment of Translational Medicine, Division of Radiation Oncology, University of Pisa, Pisa, Italy
                [_d] dDepartment of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Pavia, Italy
                Author notes
                *Dr. Amelia Barcellini, Fondazione CNAO, Strada Campeggi 53, IT–27100 Pavia (Italy), amelia.barcellini@cnao.it
                Author information
                https://orcid.org/0000-0002-1595-104X
                Article
                506485 Oncology 2020;98:513–519
                10.1159/000506485
                32408297
                b4790b15-bb9c-4d3c-b265-cbe352558d1c
                © 2020 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 30 January 2020
                : 13 February 2020
                Page count
                Pages: 7
                Categories
                Review

                Oncology & Radiotherapy,Pathology,Surgery,Obstetrics & Gynecology,Pharmacology & Pharmaceutical medicine,Hematology
                Hadrontherapy,Vulvar cancer,Chemoresistance,Adenoid cystic carcinoma,Rare gynecological tumors,Radioresistance

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