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      High-Grade Transformation in Adenoid Cystic Carcinoma of the Bartholin Gland: Case Report Translated title: Transformação de alto grau em carcinoma adenoide cístico de glândula de Bartholin: relato de caso

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          Abstract

          Introduction  In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors.

          Case Report  A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V–Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy.

          Conclusion  As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.

          Resumo

          Introdução  Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço.

          Relato de caso  Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia.

          Conclusão  Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.

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          Most cited references16

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          Dedifferentiation of low-grade chondrosarcomas.

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            Adenoid cystic carcinoma with high-grade transformation: a report of 11 cases and a review of the literature.

            High-grade transformation of adenoid cystic carcinoma (ACC) (previously referred to as dedifferentiation) is a rare phenomenon that does not fit into the traditional ACC grading schemes. The importance and minimal criteria for distinction from solid (grade III) ACC are not well established. We report 11 new cases and review the literature to further define the profile of this tumor. The median age was 61 years (range: 32 to 72 y) with a male predominance (male to female ratio of 1.75:1). The most commonly involved sites were sinonasal (4/11) and submandibular (4/11). Lymph nodes were pathologically positive in 4/7 (57.1%) cases. Distant metastases to the lung (n=2) and soft tissue of the shoulder (n=1) were observed. Five of 9 patients (55.6%) died, all within 5 years with a median overall survival of 12 months. Histologically, ACC with high-grade transformation was distinguished from conventional ACC by nuclear enlargement and irregularity, higher mitotic counts, and the loss of the biphasic ductal-myoepithelial differentiation. Useful supportive criteria were prominent comedonecrosis and fibrocellular desmoplasia. The most common morphologies for the high-grade component were poorly differentiated cribriform adenocarcinoma and solid undifferentiated carcinoma. Micropapillary and squamoid patterns were occasionally present. Ki-67 and p53 labeling indices were elevated in the high-grade components, though c-kit and cyclin-D1 were not. ACC-high-grade transformation is a highly aggressive salivary gland tumor with a variety of histologic patterns. The high propensity for lymph node metastases suggests a role for neck dissection in patients with this rare tumor.
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              Prognosis and risk factors for early-stage adenoid cystic carcinoma of the major salivary glands.

              Adenoid cystic carcinoma (ACC) is characterized by slow growth, frequent local recurrences, and distant metastasis (DM). However, these findings frequently are reported in patients with advanced-stage tumors, but the outcomes of early-stage tumors are poorly defined. We sought to evaluate the risk factors for the development of DM in early-stage ACC.
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                Author and article information

                Journal
                Rev Bras Ginecol Obstet
                Rev Bras Ginecol Obstet
                10.1055/s-00030576
                RBGO Gynecology & Obstetrics
                Thieme Revinter Publicações Ltda. (Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil )
                0100-7203
                1806-9339
                21 December 2021
                December 2021
                1 December 2021
                : 43
                : 12
                : 980-984
                Affiliations
                [1 ]Gynecology Department, Universidade Estadual Paulista “Júlio de Mesquita Filho”, Botucatu, SP, Brazil
                [2 ]Gynecology Department, Univeridade Federal de Minas Gerais, Belo Horizonte, MG, Brazil
                [3 ]Pathology Department, Hospital MaterDei, Belo Horizonte, MG, Brazil
                [4 ]Gynecology Department, Hospital MaterDei, Belo Horizonte, MG, Brazil
                Author notes
                Address for correspondence Agnaldo Lopes da Silva Filho, PhD Avenida Professor Alfredo Balena 190, 30130-100, Belo Horizonte, MGBrazil agnaldo.ufmg@ 123456gmail.com
                Author information
                http://orcid.org/0000-0002-4562-3784
                http://orcid.org/0000-0002-5036-3702
                http://orcid.org/0000-0002-1891-1457
                http://orcid.org/0000-0002-2336-5999
                http://orcid.org/0000-0002-2903-7232
                http://orcid.org/0000-0002-8486-7861
                Article
                200269
                10.1055/s-0041-1736301
                10183931
                34933392
                b53e6c61-bb84-41c0-a457-550b6f8b1c19
                Federação Brasileira de Ginecologia e Obstetrícia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. ( https://creativecommons.org/licenses/by/4.0/ )

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

                History
                : 29 July 2020
                : 30 August 2021
                Categories
                Case Report

                adenoid cystic carcinoma,bartholin gland,gynecology,oncology,vulvar neoplasms,carcinoma adenoide cístico,glândula de bartholin,ginecologia,oncologia,neoplasias vulvares

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