46, XXovotesticular disorder of sex development with serous cystadenomas of the ovary

Fernandes Gwendolyn, Shital Munde, Pranav Pramod Patwardhan, Naik Leena


Ovotesticular disorder of sex development (DSD) is an extremely rare condition and is defined as presence of both ovarian and testicular tissue in the same individual, regardless of the karyotype and the status of the external or internal genitalia. This disorder was formerly called True hermaphroditism and 400-500 cases have been reported in literature till date. Various chromosomal abnormalities have been described but 46XX is the commonest. Both Mullerian and Wolffian duct derivatives are present in the same individual and the external as well as internal genitalia display a spectrum of phenotypes. The external genitalia are ambiguous and a uterus and a phallus areoften present. The gonads may be ovotestis or a combination of ovary on one side and testes or ovotestis on the other side. These gonads are prone to develop both benign and malignant neoplasms. Tumors like dysgerminoms, seminomas, gonadoblastoma, and mucinous cystadenomas have been described. Serous cystadenomas however, have not been described.

Full Text:



Hisamatsu E, Nakagawa Y, Sugita Y. Two cases of late-diagnosed ovotesticular disorder of sex development. APSP J Case Rep. 2013 Oct 22;4(3):40. eCollection 2013.

SwainS, Pradhan L, Satpathy R, Mahapatra P. A rare case report on ovotesticular disorders of sex development (DSD) 46XY variant. Int J ReprodContraceptObstet Gynecol. 2014 Dec;3(4):1163-6.

GhofraniM.Truehermaphroditism.pathologyoutlines.com.website.http://www.pathologyoutlines.com /topic/ovarynontumortruehermaph.html

Donahoe PK, Schnitzer JJ. Evaluation of the infant who has ambiguous genitalia, and principles of operative management. Semin Pediatr Surg. 1996 Feb;5(1):30-40.

Corriden TF. Ovarian neoplasms in a hermaphrodite. N Engl J Med. 1949 Feb 17;240(7):247-9.

Hughes IA, Houk C, Ahmed SF, Lee PA; LWPESConsensusGroup; ESPEConsensusGroup. Consensus statement on management of intersexdisorders. Arch Dis Child.2006 Jul;91(7):554-63. Epub 2006 Apr 19.

Montero M, Méndez R, Valverde D, Fernández JL, Gómez M, Ruíz C.True hermaphroditism and normal male external genitalia: a rare presentation. Acta Paediatr. 1999 Aug;88(8):909-11.


  • There are currently no refbacks.