The hallmark of Ovotesticular Disorder of Sexual Development (OT-DSD) is the ovotestis.
Histologic features - the presence of both in the same organ:
(1) presence of ovarian follicles and stroma
(2) presence of seminiferous tubules with Leydig and Sertoli cells
These may be intermixed or separated by connective tissue.
Spermatogenesis is limited to absent.
The patient may also have an ovary or a testis.
The ovotestis is at risk for gonadal neoplasms such as:
(1) gonadoblastoma
(2) seminoma/dysgerminoma
(3) choriocarcinoma
(4) teratoma
(5) yolk sac tumor
