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      Mesonephric adenocarcinoma of the vagina : Diagnosis and multimodal treatment of a rare tumor and analysis of worldwide experience Translated title: Das mesonephrische Adenokarzinom der Vagina

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          Abstract

          Background

          Mesonephric adenocarcinoma of the vagina is an extremely rare tumor of the female genital tract, with only a few cases reported so far worldwide. Consequently, there is no established standard treatment and limited knowledge about the prognosis and biologic behavior of vaginal mesonephric adenocarcinoma.

          Methods

          This report documents a new case of vaginal mesonephric adenocarcinoma diagnosed in a 54-year-old woman, and analyzes this in the context of all previously published cases.

          Results

          MRI demonstrated that the 2.5 × 1.8 cm tumor of the vaginal wall was invading urethra and bladder. Following surgical excision, histologic analysis determined mesonephric adenocarcinoma of the vagina, stage pT2 R1. In order to avoid the mutilating extended surgery which would be required to reach R0 and considerable impairment of quality of life, adjuvant radiochemotherapy was administered with external radiation and brachytherapy, including 5 cycles of cisplatin (40 mg/m²) for radiosensitization. After 4 years of continuous oncologic follow-up, the patient is alive and clinically free of disease.

          Conclusion

          In this case it was shown that adjuvant radiochemotherapy with radiation and brachytherapy was effective to manage the surgical R1 situation and maintain the patient’s life quality. More published cases reports are needed to gradually substantiate optimal treatment strategies.

          Zusammenfassung

          Hintergrund

          Das mesonephrische Adenokarzinom der Vagina ist ein äußerst seltener Tumor des weiblichen Genitaltrakts. In der internationalen Literatur finden sich nur wenig gut dokumentierte Fälle. Das biologische Verhaltensmuster dieses Tumors und dessen Prognose sind weitgehend unbekannt.

          Methoden

          In diesem Bericht wird ein neuer Fall einer 54-jährigen Frau mit einem mesonephrischen Adenokarzinom der Vagina vorgestellt und unter Berücksichtigung aller bisher publizierten Fälle analysiert.

          Ergebnisse

          Bei dem 2,5 × 1,8 cm großen Tumor der Vaginalwand zeigte sich in der Magnetresonanztomographie eine Infiltration der Urethra und Harnblase. Es wurde eine Exzision des Tumors vorgenommen. Histologisch ergab sich ein mesonephrisches Adenokarzinom der Vagina im Stadium pT2 R1. Eine R0-Resektion wäre nur durch ein aggressives chirurgisches Vorgehen möglich gewesen. Um diese massive Einschränkung der Lebensqualität zu vermeiden, wurde eine adjuvante Radiochemotherapie mit externer Bestrahlung und Brachytherapie mit 5 Zyklen Cisplatin (40 mg/m²) zur Strahlensensibilisierung durchgeführt. Die Patientin ist im 4‑Jahres-Follow-up beschwerdefrei und ohne Rezidivgeschehen.

          Schlussfolgerung

          In diesem Fall ist die adjuvante Radiochemotherapie mit Brachytherapie die bestmögliche Strategie, um auf die chirurgische R1-Situation einzugehen und dabei die Lebensqualität der Patientin zu erhalten. Weitere Fallberichte sind nötigt, um optimale Behandlungsstrategien zu etablieren.

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          Most cited references 15

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          Adenocarcinoma of the vagina. Association of maternal stilbestrol therapy with tumor appearance in young women.

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            Mesonephric adenocarcinomas of the uterine cervix and corpus: HPV-negative neoplasms that are commonly PAX8, CA125, and HMGA2 positive and that may be immunoreactive with TTF1 and hepatocyte nuclear factor 1-β.

            Mesonephric adenocarcinomas are rare neoplasms that most commonly arise in the uterine cervix and exceptionally rarely in the uterine corpus. Although the morphologic features of these neoplasms are well described, there has been relatively limited investigation of the immunoprofile. We report a series of 8 mesonephric adenocarcinomas arising in the uterine cervix (7 cases) and corpus (1 case) and undertake a comprehensive immunohistochemical analysis. This includes markers that have not been investigated previously in mesonephric adenocarcinomas but that are commonly used in gynecologic pathology and may be undertaken when other, mainly Mullerian, adenocarcinomas are considered in the differential diagnosis. Linear array human papillomavirus (HPV) genotyping was also performed. Our results broadly confirm the immunohistochemical profile demonstrated in previous studies with the majority of mesonephric adenocarcinomas staining positively with CD10 (6 of 8), epithelial membrane antigen (8 of 8), vimentin (8 of 8), and calretinin (7 of 8). Estrogen receptor was positive in 2, carcinoembryonic antigen in 3, and inhibin in 4 cases. p16 was positive in 5 cases (1 diffuse and strong), despite all being HPV negative (in 1 case, there was insufficient DNA for HPV analysis). Novel findings in our study were the demonstration of nuclear positivity with PAX8 and HMGA2 in 7 cases, CA125 immunoreactivity in all 8 cases, and TTF1 and hepatocyte nuclear factor 1-β staining in 3 cases. As PAX8, CA125, HMGA2, and hepatocyte nuclear factor 1-β are commonly positive in a variety of Mullerian adenocarcinomas arising in the female genital tract, this may result in diagnostic confusion. All cases were WT1 negative.
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              Regulation of Wolffian duct development.

              Wolffian ducts (WDs) are the embryonic structures that form the male internal genitalia. These ducts develop in both the male and female embryo. However, in the female they subsequently regress, whereas in the male they are stabilised by testosterone. The WDs then develop into separate but contiguous organs, the epididymis, vas deferens and seminal vesicles. Recently, considerable progress has been made in identifying genes that are involved in these different stages of development which is described in this review. In addition, WD development in (atypical forms of) cystic fibrosis and intersex disorders, such as the complete androgen insensitivity syndrome, 17beta-hydroxysteroid dehydrogenase deficiency and LH-receptor defects, is discussed. The apparent increase in male reproductive tract disorders is briefly discussed from the perspective of the potential endocrine-disrupting effects of the numerous chemicals in the environment to which the developing male foetus can be exposed.
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                Author and article information

                Contributors
                +43-57255-55802 , i.haberlehner@salk.at
                Journal
                Strahlenther Onkol
                Strahlenther Onkol
                Strahlentherapie Und Onkologie
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                0179-7158
                1439-099X
                27 June 2016
                27 June 2016
                2016
                : 192
                : 9
                : 668-671
                Affiliations
                [1 ]Department of Obstetrics and Gynecology (OB/GYN), Paracelsus Medical University (PMU), Müllner Hauptstr. 48, 5020 Salzburg, Austria
                [2 ]Department of Radiology and Radiation Oncology, Paracelsus Medical University (PMU), Müllner Hauptstrasse 48, 5020 Salzburg, Austria
                1004
                10.1007/s00066-016-1004-x
                4996863
                27349710
                © The Author(s) 2016

                Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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                © Springer-Verlag Berlin Heidelberg 2016

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