Non-human Papillomavirus Cervical Mucinous Adenocarcinoma in a Phenotypic Male with Congenital Adrenal Hyperplasia

A majority of cervical cancers are caused by human papillomavirus (HPV); however, HPV-negative cervical cancers exist and, though rare, are more aggressive. No prior reports examine HPV-negative cancer of the cervix in a female pseudohermaphrodite with congenital adrenal hyperplasia (CAH). This is a case of a 78-year-old phenotypic male with hypospadias and absent testicles who presented with urinary retention and urosepsis. He was diagnosed with a pelvic mass on imaging and with a female mosaic karyotype (45,X/47,XXX/46 XX). He was taken to the operating room and found to have a rare form of HPV-negative cervical cancer: gastric-type adenocarcinoma (GAS). This study examines the presentation, management, and outcome of a GAS cervical cancer in a patient with a known lack of HPV exposure secondary to the unique anatomy of female pseudohermaphrodism.