Herlyn-Werner-Wunderlich syndrome (HWWS)-associated gynecological malignancies: A case report and literature review

Abstract

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital urogenital anomaly characterized by uterine didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. We present a very rare case of HWWS-associated cervical cancer in which the presence of a genital anomaly was not noticed until the patient experienced postmenopausal vaginal bleeding. A 74-year-old nulliparous Japanese woman presented with vaginal bleeding. Pre-treatment workup revealed uterine didelphys, obstructed hemivagina/hemicervix, renal agenesis, and cancer development from the remnant-obstructed hemivagina/hemicervix. The patient was diagnosed with HWWS and HWWS-associated vaginal or cervical cancer, treated with radical surgery, and a diagnosis of clear cell carcinoma (CCC) of the uterine cervix was histopathologically confirmed. A literature review revealed an increased incidence of CCC in women with HWWS.

Keywords: Cervical cancer; Clear cell carcinoma; Herlyn-Werner-Wunderlich syndrome.

Fig. 1

Pretreatment imaging results. (A), T2-weighted pelvic MRI (Coronal view) of the right uterus. A 4-cm cystic mass (blue arrow) adjacent to her normal right cervix (white arrow), showing a high signal on T2-weighted imaging, was observed in her left pelvis. As this cyst showed iso/high signal on T1-weighted imaging (C) and being communicating with the right normal cervix, this was proven to be a hematometra of her right uterus that was deviated to the left side. (B), T2-weighted pelvic MRI (Left; Coronal view, Right; Sagittal view) of the left uterus and vagina. Her remnant obstructed left hemivagina/hemicervix was observed in front of her deviated right uterus. In addition to a hematometra and a hematocolpos (blue arrow), a solid tumor exhibiting heterogeneous high signal intensity on T2-weighted image was observed in her left hemivagina/hemicervix (white arrow). (C), Axial views of T1-weighted pelvic MRI (left) and T2-weighted pelvic MRI (Right). In addition to the hematometra of the left (blue arrow) and right uteruses (white arrow), a tubular structure (yellow arrow) showing high signal on T1-weighted image suggesting methemoglobin was observed adjacent to her left hematometra, corresponding to a left ectopic ureter.(D), Abdominal CT imaging demonstrating right renal agenesis. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 2

Histopathological findings from surgically-resected specimens. Pathological examination of the surgical specimen revealed a clear cell carcinoma that developed from the obstructed hemivagina or hemicervix (hematoxylin-eosin staining: left, x1; right, × 40).