ReviewMultidisciplinary management of malignant ovarian germ cell tumours
Research Highlights
►Ovarian germ cell tumours are highly curable. ►Treatment options are limited for recurrent and residual tumours. ►Long term results suggest no significant impact on fertility and menstrual function.
Introduction
Malignant ovarian germ cell tumours (MOGCTs) are tumours derived from primitive germ cells of the embryonic gonad. These are highly malignant and rapidly growing tumours with a peak incidence occurring in adolescent and young women. The incidence varies according to geographic areas; the estimated incidence of MOGCT in the USA is 0.41 cases per 100,000 women [1] compared with 0.2 in the UK [2]. MOGCTs account for less than 5% of all ovarian malignancies in the Western countries, whereas they may represent up to 15% of ovarian cancers in Asians and blacks [3].
MOGCTs are broadly classified into two types: dysgerminomas, which are the ovarian counterpart of seminoma and non-dysgerminoma, which consist of a number of subtypes and are the ovarian counterpart of non-seminoma [3]. Since many of the patients present with early stage disease with cure rate approaching 100% and affect young women, multidisciplinary evaluation and treatment are important.
Section snippets
Staging and evaluation
MOGCTs are staged according to the FIGO staging for ovarian cancer. In general, 60 to 70% of MOGCT are stage I, 25–30% stage III, and stage II and IV are rare. This is in striking contrast to epithelial ovarian tumours where 70–75% are stage III and the remainder are equally divided by stages I, II and IV [4].
Principles of surgery
Surgery is the initial treatment of choice. Staging laparotomy follows the principles applied to epithelial ovarian cancer. Since this disease predominantly affects the young females, conservative surgery to preserve fertility is the norm and the minimal surgery is unilateral oophorectomy [26], [27], [28], [29], [30]. Cystectomy may be an adequate surgical approach however larger studies are needed before it can be considered standard. In one series of 8 patients with stage I immature teratoma
Prognostic factors
The most important prognostic factors are histological type and stage. Dysgerminoma has the best prognosis among all histological types with cure rates of > 95% even in advanced disease [69], [70], [71], [72], though with modern chemotherapy even the traditionally poor prognosis histiotypes are achieving good outcomes. Before the era of cisplatin based chemotherapy overall survival for patients with yolk sac tumours at 3 years was 13% [73]. With modern BEP regimens, a recent retrospective series
Follow-up
90% of relapses in MOGCT occur within 2 years. Dysgerminomas may recur late, up to 10 years after primary treatment. Follow up by serial measurement of tumour markers and radiological and clinical assessment every 3 monthly for the first 2 years, six monthly for three years, then annually to five years is recommended for non-dysgerminomatous tumours, and to 10 years for dysgerminoma. AFP and ß-HCG are routinely measured for all patients even if not initially raised since occasionally recurrences
Management of relapsed and refractory tumours
Approximately 20% of advanced germ cell tumours will be resistant to treatment or relapse at a later stage [57]. For patients failing optimal primary treatment, either with persistent, progressive or relapsed disease, the outlook is poor with long germ survival of 10% [62].
Evidence to guide the management of patients with recurrent ovarian germ cell tumours is sparse and largely extrapolated from experience with testicular cancer. There are some prospective studies in MOGCT [40], [79] and some
Fertility and teratogenesis
Most studies suggest no adverse effect on fertility or teratogenesity following fertility-preserving surgery and chemotherapy for MOGT [28], [29], [30], [86], [118]. In a study of 40 patients who had conservative surgery, 27 (68%) retained regular menses and 83% were having regular menses at the time of follow-up. Sixteen patients attempted to become pregnant, 12 did so and had 22 healthy children over time. No increase in congenital abnormality was reported in the offspring [119]. In the GOG
Conclusion
MOGCTs are rare tumours, the majority of which occur at a critical point in the development of young women. Most patients can be successfully treated with fertility-preserving surgery with or without chemotherapy with preservation of reproductive function. Better risk stratification to reduce treatment in good prognostic groups and improve treatment in poor prognostic or relapsed patients would be of benefit.
Conflict of interest statement
The authors declare that there are no conflicts of interest.
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