Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature
Swyer syndrome is a 46 XY pure gonadal dysgenesis. Affected individuals are phenotypically female with external female genitalia, hypoplastic Mullerian structures, and the presence of streak gonads. Gonadectomy is indicated due to increased risk of development of malignancy in streak gonads. We here...
| Κύριοι συγγραφείς: | , , , , |
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| Μορφή: | Άρθρο |
| Γλώσσα: | English |
| Έκδοση: |
Wolters Kluwer Medknow Publications
2019-01-01
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| Σειρά: | Journal of Human Reproductive Sciences |
| Θέματα: | |
| Διαθέσιμο Online: | http://www.jhrsonline.org/article.asp?issn=0974-1208;year=2019;volume=12;issue=4;spage=345;epage=347;aulast=Goyal |
| _version_ | 1828445471579832320 |
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| author | Manu Goyal Shuchita Batra Pratibha Singh Shashank Shekhar Poonam Elhence |
| author_facet | Manu Goyal Shuchita Batra Pratibha Singh Shashank Shekhar Poonam Elhence |
| author_sort | Manu Goyal |
| collection | DOAJ |
| description | Swyer syndrome is a 46 XY pure gonadal dysgenesis. Affected individuals are phenotypically female with external female genitalia, hypoplastic Mullerian structures, and the presence of streak gonads. Gonadectomy is indicated due to increased risk of development of malignancy in streak gonads. We hereby report a case of 15-year-old patient with Swyer syndrome who underwent laparoscopic gonadectomy. Histopathological examination showed streak gonads with the presence of accessory adrenal cortical tissue. The patient was put on hormonal replacement therapy postsurgery. Heterotopic adrenal rest may be an incidental finding in normal ovary and testis. Its presence in streak gonads has never been reported, and we propose a theory of increased risk of gonadal and adrenal tumors, possibly because of mutations in disorders of sex differentiation. |
| first_indexed | 2024-12-10T21:58:35Z |
| format | Article |
| id | doaj.art-9b0b51b8fb044d579f368d175f2268c4 |
| institution | Directory Open Access Journal |
| issn | 0974-1208 1998-4766 |
| language | English |
| last_indexed | 2024-12-10T21:58:35Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Human Reproductive Sciences |
| spelling | doaj.art-9b0b51b8fb044d579f368d175f2268c42022-12-22T01:31:59ZengWolters Kluwer Medknow PublicationsJournal of Human Reproductive Sciences0974-12081998-47662019-01-0112434534710.4103/jhrs.JHRS_44_19Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literatureManu GoyalShuchita BatraPratibha SinghShashank ShekharPoonam ElhenceSwyer syndrome is a 46 XY pure gonadal dysgenesis. Affected individuals are phenotypically female with external female genitalia, hypoplastic Mullerian structures, and the presence of streak gonads. Gonadectomy is indicated due to increased risk of development of malignancy in streak gonads. We hereby report a case of 15-year-old patient with Swyer syndrome who underwent laparoscopic gonadectomy. Histopathological examination showed streak gonads with the presence of accessory adrenal cortical tissue. The patient was put on hormonal replacement therapy postsurgery. Heterotopic adrenal rest may be an incidental finding in normal ovary and testis. Its presence in streak gonads has never been reported, and we propose a theory of increased risk of gonadal and adrenal tumors, possibly because of mutations in disorders of sex differentiation.http://www.jhrsonline.org/article.asp?issn=0974-1208;year=2019;volume=12;issue=4;spage=345;epage=347;aulast=Goyaladrenal tissuestreak gonadsswyer syndrome |
| spellingShingle | Manu Goyal Shuchita Batra Pratibha Singh Shashank Shekhar Poonam Elhence Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature Journal of Human Reproductive Sciences adrenal tissue streak gonads swyer syndrome |
| title | Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature |
| title_full | Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature |
| title_fullStr | Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature |
| title_full_unstemmed | Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature |
| title_short | Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature |
| title_sort | swyer syndrome with heterotopic adrenal cortical tissue in streak gonads a rare case and review of literature |
| topic | adrenal tissue streak gonads swyer syndrome |
| url | http://www.jhrsonline.org/article.asp?issn=0974-1208;year=2019;volume=12;issue=4;spage=345;epage=347;aulast=Goyal |
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