Herlyn-Werner-Wunderlich syndrome, is a rare urogenital congenital anomaly. Coexisting Mullerian ducts anomalies and ovarian neoplasms are rarely reported. We present the first case of Herlyn-Werner-Wunderlich syndrome with borderline serous neoplasm of the ovary. A 29-year-old married female with primary infertility and elevated level of cancer antigen 125 (CA-125), underwent pelvic magnetic resonance imaging for evaluation which revealed uterus didelphys, obstructed right hemivagina, right renal agenesis as well as right ovarian cystic lesion. Ovary preserving laparoscopic cystectomy was performed with a pathological diagnosis of a serous borderline tumor. Although rarely reported, Mullerian ducts anomalies and uterine or ovarian remnant neoplasms can occur. These few case reports may suggest an underlying, yet to be discovered, genetic association of Mullerian ducts anomalies and development of ovarian neoplasms of various pathological subtypes.
Keywords: Congenital anomalies; Kidneys; Ovarian neoplasms.
© 2020 The Authors. Published by Elsevier Inc. on behalf of University of Washington.