Conservative treatment of recurrent ovarian fibromas in a young patient affected by Gorlin syndrome

Abstract

The case of recurrent bilateral ovarian fibromas occurring in a 22 year old Italian girl affected by Gorlin syndrome is reported. Ovarian fibromas occur in 75% of female patients with Gorlin syndrome and their recurrence has rarely been reported in the literature. Management is guided by the benign nature of the lesion and consists of surgical removal of the fibroma. Preservation of the normal ovarian tissue is recommended even though there is risk of recurrence of the fibroma.

Clinical Characteristics and Surgical Management Options for Ovarian Fibroma/Fibrothecoma: A Study of 97 Cases

“…Ascites or classic Meigs syndrome with hydrothorax has been reported in 10% of patients with ovarian fibroma, and it has also been observed in 75% patients with Gorlin (Nevoid basal cell carcinoma) syndrome [4,7]. Other genetic syndromes, such as Maffucci syndrome and Sotos syndrome, have been reported to be related to ovarian fibroma in younger women [8,9].…”

Laparoscopic Surgery for Bilateral Multiple Calcified Ovarian Fibromas: A Case Report

“…Gorlin syndrome is consisted of multiple tissue calcification and nevoid basal cell carcinoma. Approximately 75% of the ovarian fibromas occur in bilateral, calcified and multinodular forms in patients with Gorlin syndrome [8]. Sotos syndrome is another syndrome associated with ovarian fibromas and characterized by congenital anomalies such as microcephaly or dolichocephaly.…”

Ovarian preservation in a young patient with Gorlin syndrome and multiple bilateral ovarian masses

“…Time to recurrence after initial surgery ranges from 7 months to 9 years (12,15,16). Although studies do not suggest an increased risk of malignancy in recurrent tumors, reports of ovarian malignancy exist (10,11,15).…”

“…The masses may have also had an unidentified secretory function. Interestingly, another case report described the return of normal menses after surgery (16).…”