Müllerian agenesis is a congenital malformation characterized by absence of the uterus, cervix, and upper vagina. A positive home pregnancy test in a woman with Müllerian agenesis mandated evaluation for malignancy.
A woman with Müllerian agenesis presented with elevated levels of human chorionic gonadotropin (hCG), testosterone, and dehydroepiandrosterone sulfate. Pelvic magnetic resonance imaging (MRI), abdominal and pelvic computed tomography scan, chest computed tomography scan, brain MRI, and body positron emission tomography scan did not identify a malignancy. Human chorionic gonadotropin characterization revealed 74% hyperglycosylated and 1.6% free β-hCG, suggesting a trophoblast-containing tumor. Interventional ovarian venous sampling and repeat pelvic MRI suggested a right adnexal source. After laparoscopic removal of a stage 1C right ovarian dysgerminoma, hCG and testosterone returned to normal.
A dysgerminoma coincident with Müllerian agenesis expressed hCG before detection by MRI. Human chorionic gonadotropin molecular characterization, ovarian vein sampling, and repeat pelvic MRI led to successful treatment.
A dysgerminoma coincident with Müllerian agenesis expresses human chorionic gonadotropin before detection by magnetic resonance imaging.
Department of Obstetrics and Gynecology and the Divisions of Gynecologic Oncology and Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine and Tampa General Hospital, Tampa, Florida.
Corresponding author: Shayne M. Plosker, MD, Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine, South Treatment Center, 2 Tampa General Circle, 6th floor, Tampa, FL 33606; e-mail: firstname.lastname@example.org.
Financial Disclosure The authors did not report any potential conflicts of interest.