Herlyn-Werner-Wunderlich syndrome with borderline serous cystadenoma of the ovary: case report and literature review

Abstract

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.

Fig. 1

Coronal high resolution T2 of the pelvis and coronal HASTE of the abdomen demonstrate 2 separate uterine cavities, right ovarian cystic lesion and congenitally absent right kidney.

Fig. 2

Axial T2 and axial nonenhanced T1 fat sat demonstrating 2 separate cervices and vaginas. The right hemivagina is obstructed with internal blood products.

Fig. 3

Axial T2 at the level of the ovaries showing large right cystic lesion with internal frond like projections.

Fig. 4

Postresection histopathology specimen of the resected serous borderline tumor: Left image (Hematoxylin and eosin stain, × 4), right image (Hematoxylin and eosin stain, × 20).