Mature cystic teratomas of the ovary have an incidence of 1.2-14.2 cases per 100.000
people per year. Malignant transformation occurs in approximately 2% of the cases,
and usually consists of squamous cell carcinoma. The preoperative detection is difficult
and the diagnostic accuracy of ultrasound, magnetic resonance imaging, and computed
tomography is debated. The diagnosis is frequently made in the operating room or on
final histological examination. Standard treatment consists of bilateral salpingo-oophorectomy,
total hysterectomy and comprehensive surgical staging in early disease and optimal
cytoreductive surgery in advanced disease. Paclitaxel/carboplatin- based chemotherapy
is the most used adjuvant treatment, whereas more aggressive regimens can be adopted
in patients with high tumor burden or recurrent disease. The efficacy of radiotherapy
is still unproven. The prognosis is poor when the tumor has spread beyond the ovary.
There are few information to provide commonly accepted guidelines for this malignancy.