Laparoscopic treatment of sclerosing stromal tumor of the ovary in a woman with Gorlin-Goltz Syndrome: a case report and review of the literature

Gorlin-Goltz syndrome is a rare hereditary multisystemic disease. Multiple basal cell carcinomas, odontogenic keratocysts and skeletal abnormalities are the main clinical manifestations of the syndrome, but several organs can be involved. Moreover this condition is associated with the development of various benign and malignant tumors, even in the genital tract. This report describes a rare association between Gorlin-Goltz syndrome and the sclerosing stromal tumor of the ovary. Since the ultrasounds and magnetic resonance pattern of this tumor can be similar to those of a malignant neoplasm, a prompt surgical intervention and histological confirmation of diagnosis is mandatory. However this is a benign lesion and thus can be approached with a laparoscopic fertility sparing surgery. Gynecologists should be aware of this possible association, in order to provide an appropriate counseling to these women, and to perform a fertility sparing laparoscopic approach, wherever possible.