Swyer Syndrome: A Case of Dysgerminoma Solely within the Fallopian Tube.
J Pediatr Adolesc Gynecol
; 34(6): 869-871, 2021 Dec.
Article
in En
| MEDLINE
| ID: mdl-33989803
ABSTRACT
BACKGROUND:
46XY pure gonadal dysgenesis (Swyer syndrome) is a rare disorder of sexual development. Patients have a 46XY karyotype, though phenotypically they appear female with normal external genitalia and vagina. Although patients exhibit normal Müllerian structures (uterus, fallopian tubes, and vagina), they possess a pair of bilateral undifferentiated gonad streaks. Delayed puberty and primary amenorrhea are the common presentations. There is an increased risk of developing tumors in the gonads and therefore a bilateral gonadectomy is recommended. CASE A 16-year-old girl who presented with primary amenorrhea was diagnosed with Swyer syndrome. She underwent prophylactic bilateral gonadectomy and salpingectomies. She was discovered to have no gonadal malignancy, conversely dysgerminoma solely within the fallopian tube. SUMMARY ANDCONCLUSION:
Both bilateral salpingectomies and bilateral gonadectomies should be recommended as the operation of choice in patients with Swyer Syndrome.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Ovarian Neoplasms
/
Gonadoblastoma
/
Dysgerminoma
/
Gonadal Dysgenesis, 46,XY
Type of study:
Diagnostic_studies
Limits:
Adolescent
/
Female
/
Humans
Language:
En
Journal:
J Pediatr Adolesc Gynecol
Journal subject:
GINECOLOGIA
/
PEDIATRIA
Year:
2021
Document type:
Article