Abstract

Lynch syndrome (LS), also known as hereditary non-polyposis colorectal cancer, is an inherited cancer syndrome which increases the risk of developing colorectal cancer and endometrial cancer. Individuals with LS have an increased risk of cancers of the ovary, urinary tract, stomach, small intestine, pancreas, biliary tract, brain and skin. Cancer risk reduction is recommended through chemoprevention (aspirin), surveillance (colonoscopy, assessment of the endometrium and ovaries via USS, aspiration biopsy and tumour marker monitoring; CA125) or risk reduction surgery, that is, total hysterectomy and bilateral salpingo-oophorectomy.

This is a case of a nulliparous woman in her early 30s with LS and a congenital genital tract malformation. She had a unicornuate (left) uterus and a vestigial (right) uterine horn. There was an inability to obtain a conclusive set of endometrial biopsies in this patient due to the nature of the patient’s congenital uterine abnormality. In this case, surgery was recommended to excise the vestigial horn and fallopian tube in order to optimise surveillance and fertility.