Surgical management of ovarian fibromas in young patients with Gorlin syndrome: a case series and review of the literature.

OBJECTIVE

To characterize the presentation and surgical management of ovarian fibromas among a case series of pediatric and adolescent patients with Gorlin syndrome.

DESIGN

Retrospective case series.

SETTING

Tertiary-care hospital.

PATIENTS

Patients aged 18 years and younger with a diagnosis of Gorlin syndrome who underwent surgical care of ovarian fibromas at a single tertiary children's hospital from 1990 to 2022.

EXPOSURE

Surgical management of ovarian fibromas.

MAIN OUTCOME MEASURES

Clinical characteristics, surgical treatment outcomes, ovarian conservation, surveillance imaging, and recurrence after surgical management of ovarian fibromas.

RESULTS

Four patients, aged between 5 and 18 years, underwent surgical resection of one or more ovarian fibromas. Dominant fibromas ranged in size from 4 to 9 cm, and most cases had multiple fibromas. Three patients underwent transverse laparotomies, and one patient had a laparoscopic approach. Unilateral oophorectomy was performed in three patients, two of whom presented with adnexal torsion. Among those with surveillance imaging, two patients with prior oophorectomy had a recurrence in the remaining contralateral ovary, one of whom underwent a second surgical procedure.

CONCLUSIONS

Ovarian fibroma presentation and treatment varied widely among pediatric patients with Gorlin syndrome, and the presence of multiple and bilateral ovarian lesions raises important considerations regarding the optimal surgical approach and surveillance. Ovarian conservation should be prioritized in patients with Gorlin syndrome, because they are at risk of iatrogenic oophorectomy with their initial or repeat surgical management.