Incidental gonadoblastoma in swyer syndrome: a case report with brief review of literature

Authors

  • Gayatri Suresh Department of Obstetrics and Gynecology, AIIMS, New Delhi, India
  • Rinchen Zangmo Department of Obstetrics and Gynecology, AIIMS, New Delhi, India
  • Kallol Kumar Roy Department of Obstetrics and Gynecology, AIIMS, New Delhi, India
  • Rakhi Rai Department of Obstetrics and Gynecology, AIIMS, New Delhi, India
  • Deepali Garg Department of Obstetrics and Gynecology, AIIMS, New Delhi, India
  • Sandeep Mathur Department of Pathology, AIIMS, New Delhi, India
  • Jayati . Department of Pathology, AIIMS, New Delhi, India

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20211552

Keywords:

Gonadoblastoma, Gonadal dysgenesis, Swyer syndrome

Abstract

Swyer syndrome is a disorder of sexual differentiation with an incidence of 1 in 80,000 population. Dysgenetic gonads have a propensity for malignant transformation particularly in the presence of Y chromosome and hence need prophylactic removal. We report a case of an adolescent girl who presented with primary amenorrhea who was identified as a case of 46 XY dysgenesis after karyotype studies. Extirpation of gonads were done laparoscopically and on histopathological assessment gonadoblastoma was detected. This case report aims to reiterate the importance of gonadectomy in patients with swyer syndrome as tumors could arise even in the absence of frank adnexal masses.

Author Biography

Gayatri Suresh, Department of Obstetrics and Gynecology, AIIMS, New Delhi, India

Assistant Professor 

Department of Obstetrics and Gynaecology

All India Institute of Medical Sciences New Delhi

References

Hanlon AJ, Kimble RM. Incidental gonadal tumors at the time of gonadectomy in women with Swyer syndrome: a case series. J Pediatr Adolesc Gynecol. 2015;28(2):27-9.

Kapur, Anupam, Agarwal, Raju, Magdu Hrishikesh Madhusudan D. Swyers Syndrome: primary amenorrohea with gonadal dysgenesis a rare cause. Internat J Biomed and Adv Res. 2014;5:388.

Malhotra N, Dadhwal V, Sharma KA, Gupta D, Agarwal S, Deka D. The laparoscopic management of Swyer syndrome: Case series. J Turk Ger Gynecol Assoc. 2015;16(4):252-6.

Gupta A, Bajaj R, Jindal UN. A Rare Case of Swyer Syndrome in Two Sisters with Successful Pregnancy Outcome in Both. J Hum Reprod Sci. 2019;12(3):267-9.

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Published

2021-04-23

Issue

Section

Case Reports