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Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review

BACKGROUND: Gonadoblastoma (GB) is a rare mixed germ cell-sex cord-stromal tumour, first described in humans, commonly found in dysgenetic gonads of intersex patients that have a Y chromosome. However, this entity in not recognized in the WHO classification of tumours of genital system of domestic a...

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Autores principales: Flores, Ana R., Lobo, João, Nunes, Francisco, Rêma, Alexandra, Lopes, Paula, Carvalho, Luís, Bartosch, Carla, Amorim, Irina, Gärtner, Fátima
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6757443/
https://www.ncbi.nlm.nih.gov/pubmed/31547830
http://dx.doi.org/10.1186/s13048-019-0561-x
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author Flores, Ana R.
Lobo, João
Nunes, Francisco
Rêma, Alexandra
Lopes, Paula
Carvalho, Luís
Bartosch, Carla
Amorim, Irina
Gärtner, Fátima
author_facet Flores, Ana R.
Lobo, João
Nunes, Francisco
Rêma, Alexandra
Lopes, Paula
Carvalho, Luís
Bartosch, Carla
Amorim, Irina
Gärtner, Fátima
author_sort Flores, Ana R.
collection PubMed
description BACKGROUND: Gonadoblastoma (GB) is a rare mixed germ cell-sex cord-stromal tumour, first described in humans, commonly found in dysgenetic gonads of intersex patients that have a Y chromosome. However, this entity in not recognized in the WHO classification of tumours of genital system of domestic animals. Herein, we describe a case of ovarian gonadoblastoma with proliferation of dysgerminoma and sex cord-stromal tumour components, in a phenotypically and cytogenetically normal bitch. CASE PRESENTATION: A 17-year-old cross-breed bitch had a firm, grey-white multinodular mass in the left ovary. The tumour was submitted to histopathological examination and Y chromosome detected through karyotype analysis and PCR studies. Microscopically, the ovary was almost replaced by an irregular neoplasm composed of three distinct, intermixed elements: dysgerminoma, mixed germ cell-sex cord-stromal tumour resembling human GB and a proliferative sex cord-stromal tumour component. The germ cells of gonadoblastoma and dysgerminoma components were immunoreactive for c-KIT. Sex cord-stromal cells of gonadoblastoma were immunoreactive for α-inhibin. The sex cord-stromal tumour was immunoreactive for AE1/AE3, occasionally for α-inhibin and negative for epithelial membrane antigen (EMA). The karyotype was 78, XX and PCR analysis confirmed the absence of the Y chromosome. CONCLUSION: Based on these findings, a diagnosis of gonadoblastoma with proliferation of dysgerminoma and sex cord-stromal tumour was made. This is the first case of ovarian gonadoblastoma in a female dog. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s13048-019-0561-x) contains supplementary material, which is available to authorized users.
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spelling pubmed-67574432019-09-30 Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review Flores, Ana R. Lobo, João Nunes, Francisco Rêma, Alexandra Lopes, Paula Carvalho, Luís Bartosch, Carla Amorim, Irina Gärtner, Fátima J Ovarian Res Case Report BACKGROUND: Gonadoblastoma (GB) is a rare mixed germ cell-sex cord-stromal tumour, first described in humans, commonly found in dysgenetic gonads of intersex patients that have a Y chromosome. However, this entity in not recognized in the WHO classification of tumours of genital system of domestic animals. Herein, we describe a case of ovarian gonadoblastoma with proliferation of dysgerminoma and sex cord-stromal tumour components, in a phenotypically and cytogenetically normal bitch. CASE PRESENTATION: A 17-year-old cross-breed bitch had a firm, grey-white multinodular mass in the left ovary. The tumour was submitted to histopathological examination and Y chromosome detected through karyotype analysis and PCR studies. Microscopically, the ovary was almost replaced by an irregular neoplasm composed of three distinct, intermixed elements: dysgerminoma, mixed germ cell-sex cord-stromal tumour resembling human GB and a proliferative sex cord-stromal tumour component. The germ cells of gonadoblastoma and dysgerminoma components were immunoreactive for c-KIT. Sex cord-stromal cells of gonadoblastoma were immunoreactive for α-inhibin. The sex cord-stromal tumour was immunoreactive for AE1/AE3, occasionally for α-inhibin and negative for epithelial membrane antigen (EMA). The karyotype was 78, XX and PCR analysis confirmed the absence of the Y chromosome. CONCLUSION: Based on these findings, a diagnosis of gonadoblastoma with proliferation of dysgerminoma and sex cord-stromal tumour was made. This is the first case of ovarian gonadoblastoma in a female dog. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s13048-019-0561-x) contains supplementary material, which is available to authorized users. BioMed Central 2019-09-23 /pmc/articles/PMC6757443/ /pubmed/31547830 http://dx.doi.org/10.1186/s13048-019-0561-x Text en © The Author(s). 2019 Open AccessThis 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. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Flores, Ana R.
Lobo, João
Nunes, Francisco
Rêma, Alexandra
Lopes, Paula
Carvalho, Luís
Bartosch, Carla
Amorim, Irina
Gärtner, Fátima
Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
title Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
title_full Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
title_fullStr Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
title_full_unstemmed Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
title_short Canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
title_sort canine ovarian gonadoblastoma with dysgerminoma overgrowth: a case study and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6757443/
https://www.ncbi.nlm.nih.gov/pubmed/31547830
http://dx.doi.org/10.1186/s13048-019-0561-x
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