Objective: The aim of this survey was to analyse published case report
s describing malignancies arising from an extraovarian endometriosis a
nd to list their symptoms, localisations and treatment modalities. Mat
erial and Methods: Seventy-five case reports from the world literature
were reviewed and the main findings summarised. Results: Fifty-eight
tumors were carcinomas and 17 were sarcomas. The most common tumor loc
alisation was the rectovaginal septum. In 20 patients an exogenous (n
= 18) or endogenous (n = 2) oestrogenic stimulation was documented. On
e patient received tamoxifen at the time of diagnosis. Conclusions: Ma
lignancies arising from extraovarian endometriosis are extremely rare.
In patients with a history of a hysterectomy and bilateral oophorecto
my vaginal bleeding or a pelvic mass may represent signs of the malign
ant transformation of an extraovarian endometriosis. This is especiall
y important if the patient's history includes endometriosis. In additi
on to surgical tumour reduction, hormone therapy with progestins is re
commended.