Swyer Syndrome : A Case Report with Literature Review

Authors

  • R Patnayak Department of PathologySri Venkateswar Institute Of Medical Sciences TirupatiAndhra Pradesh-517507
  • V Suresh Department of Endocrinology Sri Venkateswar Institute Of Medical Sciences Tirupati Andhra Pradesh-517507
  • A Jena Department of Surgical Oncology Sri Venkateswar Institute Of Medical Sciences Tirupati Andhra Pradesh-517507
  • B Vijayalakshmi Department of Radiology Sri Venkateswar Institute Of Medical Sciences Tirupati Andhra Pradesh-517507
  • AP Reddy Department of Radiology Sri Venkateswar Institute Of Medical Sciences Tirupati Andhra Pradesh-517507
  • M Rukumangadha Department of Pathology Sri Venkateswar Institute Of Medical Sciences Tirupati Andhra Pradesh-517507
  • A Sachan Department of Endocrinology Sri Venkateswar Institute Of Medical Sciences Tirupati Andhra Pradesh-517507

DOI:

https://doi.org/10.31729/jnma.72

Abstract

Gonadoblastomas are known to develop in dysgenetic gonads, especially so, if Y chromosome material is present. A 20-years- old girl who noticed breast development since the age of 12 years presented with primary amenorhoea, distension of lower abdomen and intermittent pain for two months. She had breakthrough bleeding with six months of estrogen replacement. Tanner breast stage was fi ve and pubic hair stage was also fi ve. Examination revealed a mass in the lower abdomen extending into hypogastrium, umbilical and lumbar regions. Her gonadotropin levels were grossly elevated. Karyotyping showed 46XY. CT scan of abdomen showed a 17X11 cm mass in the pelvis without visible gonads. Surgical excision of the mass along with bilateral salpingophorectomy was performed. Histopathology revealed the mass to be a dysgerminoma, while the right gonad lodged gonadoblastoma. She was diagnosed as a rare case of Swyer syndrome.

Keywords: Dysgerminoma, gonadoblastoma, Swyer syndrome, XY gonadal dysgenesis.

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Published

2012-06-30

How to Cite

Patnayak, R., Suresh, V., Jena, A., Vijayalakshmi, B., Reddy, A., Rukumangadha, M., & Sachan, A. (2012). Swyer Syndrome : A Case Report with Literature Review. Journal of Nepal Medical Association, 52(186). https://doi.org/10.31729/jnma.72

Issue

Vol. 52 No. 186 (2012)

Section

Case Reports

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