Dysgerminoma and ovarian gonadoblastoma in Swyer syndrome

XY karyotype and poor secondary sexual character and normal external genitalia. She suffered of abdominal pain. In palpation of the ab-domen, an irregular mobile mass was detected in left lower quadrant. The ultrasound revealed uterine size approximate dimensions 3×2 cm (infantile) and a 19 cm pelvic mass heterogeneous and multi-loculated in left side of the pelvic cavity with possible origin of the left ovary. In addition, in right pelvic fossa, a mass about 6 cm was detected. CT-Scan showed a pelvic mass with overall dimensions of 10 cm with vicinity to the left iliac vessels, modest amounts of ascities along with evidence of peritoneal dis-semination (seeding). In laparotomy we observed massive ascities and a 20 cm solid mass in left ovary and a small mass in right ovary and involvement para aortic lymph node. Pathological report indicated as stage III of dysgerminoma in left ovary and gonadoblastom in right ovary. Conclusion: This case is presented because it could have excellent prognosis if not missed opportunities of early recognizing and furthermore adequate treatment with gonadectomy. Abstract

[1]  R. Kimble,et al.  Incidental gonadal tumors at the time of gonadectomy in women with Swyer syndrome: a case series. , 2015, Journal of pediatric and adolescent gynecology.

[2]  Yongmei Li,et al.  46,XY female sex reversal syndrome with bilateral gonadoblastoma and dysgerminoma , 2014, Experimental and therapeutic medicine.

[3]  M. De Santis,et al.  Successful twin pregnancy in a 46,XY pure gonadal dysgenesis , 2013, Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology.

[4]  N. Zech,et al.  Unexpected labor and successful twin birth to a pure gonadal dysgenetic woman , 2013, Archives of Gynecology and Obstetrics.

[5]  L. Looijenga,et al.  Delayed Recognition of Disorders of Sex Development (DSD): A Missed Opportunity for Early Diagnosis of Malignant Germ Cell Tumors , 2012, International journal of endocrinology.

[6]  Z. Maleki,et al.  Coexistence of gonadoblastoma and dysgerminoma in a dysgenetic gonad on touch preparation: A case report , 2011, Diagnostic cytopathology.

[7]  S. Kane,et al.  Bilateral gonadoblastomas with unilateral dysgerminoma in a case of 46 XY pure gonadal dysgenesis (Swyer syndrome). , 2010, Indian journal of pathology & microbiology.

[8]  Yun-Fai Chris Lau,et al.  Gonadoblastoma locus and the TSPY gene on the human Y chromosome. , 2009, Birth defects research. Part C, Embryo today : reviews.

[9]  L. Michala,et al.  Swyer syndrome: presentation and outcomes , 2008, BJOG : an international journal of obstetrics and gynaecology.

[10]  R. Scully Gonadoblastoma. A gonadal tumor related to the dysgerminoma (Seminoma) and capable of sex‐hormone production , 1953, Cancer.

[11]  Xi-shi Liu,et al.  Swyer syndrome, 46,XY gonadal dysgenesis, a sex reversal disorder with dysgerminoma: a case report and literature review. , 2011, Clinical and experimental obstetrics & gynecology.