Large pelvic mass and ascites complicating pregnancy with Gorlin-Goltz-syndrome

A 19-year old pregnant woman with Gorlin-Goltz-syndrome (Syndrome of nevoid basal cell carcinoma) with high occurrence of ovarian fibromas that rarely become malignant presented with a 10-cm solid retrouterine tumour at 7 wee ks of gestation. Ultrasound-guided punch biopsies revealed benign fibromusc ular tissue. During subsequent weeks the tumour constantly increased in siz e. At 24 weeks of gestation the patient developed massive ascites, pleural effusion and a urinary tract dilatation of II-III degree on the right side. Despite previous benign biopsies and the finding that there were no malign ant cells in the ascites malignancy was still suspected based on rapid tumo ur growth, massive effusion and elevated CA 12-5 levels above 4000 U/ml. At 28 weeks elective Caesarean section was performed after steroid induction of fetal lung maturity via longitudinal incision laparotomy. After delivery of a healthy fetus an extremely vascularised tumour with broad adhesions t o the uterus and intestinum was removed, Histologically the tumour turned o ut to be a 20-cm leiomyoma of high cellularity most likely deriving from th e right ovary. The diagnosis and management of pelvic masses in pregnancy s uspicious of malignancy are discussed on the basis of the presented case.