Endometrial Cancer Arising in Adenomyosis (EC-AIA): A Systematic Review

SIMPLE SUMMARY: A systematic review of the literature was performed to assess the clinicopathological characteristics and survival outcomes of endometrial cancer arising in adenomyosis (EC-AIA). From our analysis, EC-AIA is a rare disease that mainly affects menopausal women and shows symptoms similar to endometrial cancer, but has a challenging preoperative diagnosis. The higher prevalence of the non-endometrioid histotype, advanced FIGO stages, and p53-signature might be behind the worse prognosis of EC-AIA compared to endometrial cancer. ABSTRACT: Endometrial cancer arising in adenomyosis (EC-AIA) is a rare uterine disease characterized by the malignant transformation of the ectopic endometrium within the adenomyotic foci. Clinicopathological and survival data are mostly limited to case reports and a few cohort studies. We aimed to assess the clinicopathological features and survival outcomes of women with EC-AIA through a systematic review of the literature. Six electronic databases were searched, from 2002 to 2022, for all peer-reviewed studies that reported EC-AIA cases. Thirty-seven EC-AIA patients from 27 case reports and four case series were included in our study. In our analysis, EC-AIA appeared as a rare disease that mainly occurs in menopausal women, shares symptoms with endometrial cancer, and is challenging to diagnose preoperatively. Differently from EC, it shows a higher prevalence of the non-endometrioid histotype, advanced FIGO stages, and p53-signature, which might be responsible for its worse prognosis. Future studies are necessary, to confirm our findings and further investigate this rare condition.