Primary Bilateral Multiple Ovarian Leiomyomata in a Patient with Gorlin-Goltz Syndrome: Would Intraoperative Frozen Section Have Prevented Surgical Menopause?

Abstract

Benign ovarian tumours are a diagnostic criterion for Gorlin-Goltz syndrome (GGS), especially when bilateral. We report this rare case of bilateral multiple ovarian fibroids that posed a diagnostic dilemma in a patient with Gorlin-Goltz syndrome. She is a 31-year-old nullipara, primarily infertile, referred with a 13-year history of lower abdominal swelling, a 22-week size pelvic mass, which was hard and slightly mobile, with no ascites. Tumour markers were normal. Abdominopelvic CT revealed multiple small bowel calcifications likely metastatic. Intraoperative findings were those of bilateral huge, extremely hard, craggy, multi-lobulated ovarian tumour measuring 28cmx12cm on the right and 20cmx10cm on the left. No grossly normal ovarian tissue identified. The appearance and consistency of the tumour raised a suspicion for malignancy. As there was no facility for intraoperative frozen section and patient was not consented for hysterectomy, bilateral salpingo-oophorectomy was done following second and third opinions. Histology revealed bilateral ovarian leiomyomata. During follow up, she was discovered to have major and minor criteria for Gorlin-Goltz syndrome. Patient is currently on hormone replacement therapy for menopausal symptoms and regular with follow up.