Purpose: Malignant ovarian germ cell tumors (MOGCTS) are rare but curable at all stages of disease. This review gives an outline of the management of this disease.
Methods: We performed a literature search in the PubMed of almost all relevant articles concerning MOGCTs on pathology, prognostic factors, surgery, post-operative therapy and late effects of therapy. The available literature is mainly composed of retrospective reviews and articles.
Results: Prognostic factors include stage, amount of residual tumor, histologic type and raised tumor markers. For patients with early stage disease, cure rates approach 100%, while for those with advanced-stage disease are at least 75%. Appropriate surgical treatment for patients where fertility needs to be preserved consists in laparotomy with unilateral salpingo-oophorectomy (USO) and resection of all visible disease. For patients with advanced-stage disease, the role and the extent of debulking surgery remain controversial despite its routine use. However, it is suggested a benefit from minimal residual disease at completion of primary surgical cytoreduction with both non-platinum and platinum-based chemotherapy regimens. Second-look surgery clearly is not indicated in patients with early stage non-dysgerminoma or in all patients with dysgerminoma. However, teratoma patients may benefit from secondary cytoreduction. Three courses of bleomycin, etoposide and cisplatin (BEP) is the current standard adjuvant chemotherapy and four courses of BEP are recommended in case of bulky residual tumor after surgery. More evidence is required to show that surveillance is a safe option. There is a hint that high-dose chemotherapy may play a role in relapsed patients. The majority of MOGCTs patients who undergo fertility-sparing surgery and chemotherapy retain their gonadal and reproductive function. There is an increasing concern about life-threatening long-term effects of treatment.
Conclusion: MOGCTs are rare neoplasms that affect girls and young women and have excellent prognosis at all stages of disease with optimal therapy. The majority of MOGCTs patients retain their reproductive function.