Journal of Human Reproductive Science
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CASE REPORT Table of Contents   
Year : 2019  |  Volume : 12  |  Issue : 4  |  Page : 345-347
Swyer syndrome with heterotopic adrenal cortical tissue in streak gonads: A rare case and review of literature

1 Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Correspondence Address:
Dr. Manu Goyal
AIIMS Residential Complex, AIIMS Campus, Basni Industrial Area, Jodhpur - 342 005, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jhrs.JHRS_44_19

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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.

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