Background: Neuroendocine carcinoma of the gynecologic tract is rare and poses asignificant clinical challenge because of tumor heterogeneity and lack ofstandardized guidelines for treatment. Ovotestis refers to the histology of a gonadthat contains both ovarian follicles and testicular tubular elements. Ovotesticulardisorder of sexual development occurs in fewer than 10% of all disorders of sexualdevelopment. Gonadal tumors with malignant potential occur in 2.6% of all cases ofovotesticular disorder of sexual development.Case: Here we represent a 77-year-old woman with primary amenorrhea, infertilityand 10cm solid mass in left adnex with 46 XY in karyotype with ovotestisneuroendocrine neoplasm in pathology report which was treated with a multimodality manner including surgery and chemotherapy but she came back withpulmonary metastasis after 2 cycles of chemotherapy. For women who present witha stage 1 primary ovarian neuroendocrine tumor the prognosis is excellent withgreater than 90% survival. Neuroendocrine tumor of the ovary represents 3 % of allneuroendocrine tumors. The prevalence of ovotestis is 1/20000 births. For womenwith more advanced disease, the prognosis is poor.
Neuroendocrine carcinoma of theovary is a rare and aggressive tumor commonly associated with other surfaceepithelial and germ cell neoplasms. The prevalence of ovotestis is 1/20000 birthsand gonadal malignancies are the most reported neoplasm affected the ovotestis.Here we report a case of ovotestis which is presented with neuroendocrinecarcinoma and poor prognosis.Conclusion:
Neuroendocrine carcinoma of the ovary is a rare and aggressive tumorcommonly associated with other surface epithelial and germ cell neoplasms. Theprevalence of ovotestis is rare and gonadal malignancies are the most reportedneoplasm affected the ovotestis.