Treatment of Malignant Gonadal GCTs
While advanced-stage ovarian dysgerminomas similar to testicular seminomas are highly curable with surgery and radiation therapy, the effects on growth, fertility, and risk of treatment-induced second malignancy in these young patients [19,20] make chemotherapy a more attractive adjunct to surgery.[21,22] Complete tumor resection is the goal for advanced dysgerminomas; platinum-based chemotherapy can be given preoperatively to facilitate resection or postoperatively (after debulking surgery) to avoid mutilating surgical procedures. This approach results in a high rate of cure and the maintenance of menstrual function and fertility in most patients with dysgerminomas.[21,23]
For ovarian malignant GCTs other than dysgerminomas or immature teratomas, treatment generally involves surgical resection and adjuvant chemotherapy.[24,25] Platinum-based chemotherapy regimens such as PEB or JEB have been used successfully in children,[8,9,10,15] and PEB is a common regimen in young women with ovarian GCTs.[26,27] This approach results in a high rate of cure and the maintenance of menstrual function and fertility in most patients with nondysgerminomas.[25,28] A few small studies have suggested that observation after surgery may be an option, but only as part of a clinical trial with strict adherence to surgical guidelines.[10,28]
A multidisciplinary approach is essential for treatment of ovarian GCTs. Various surgical subspecialties and the pediatric oncologist must be involved in clinical decisions. The reproductive surgical approach for pediatric GCTs is often guided by the hope that function can be preserved. In a completed pediatric intergroup trial, pediatric patients with ovarian GCTs (stages I-IV) had excellent survival with PEB and conservative surgery, rather than the strict guidelines proposed originally in the study. The role of laparoscopy in children with ovarian GCTs has not been well studied.
Standard treatment options
Surgery: The role for surgery at diagnosis is age- and site-dependent and must be individualized. Primary resection is appropriate when feasible without undue risk of damage to adjacent structures; otherwise, an appropriate strategy is biopsy for diagnosis followed by subsequent surgery in selected patients who have residual masses following chemotherapy.
Stages I through IV
- Surgery and treatment with four to six courses of standard PEB, with the exception of patients with stage I ovarian GCTs for whom observation is currently being evaluated. These patients have an OS outcome greater than 90% with this regimen, suggesting that a reduction in therapy could be considered.[8,9,29]
- Surgery and treatment with six courses of JEB.[10,29]
Treatment options under clinical evaluation for stages I through III
The following are examples of national and/or institutional clinical trials that are currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
COG-AGCT0132: This Children's Oncology Group trial is currently studying the effect of surgery and decreased chemotherapy for patients with stages II-III (three courses of PEB over 3 days) with the goal of decreasing the duration and cumulative doses of chemotherapy (25% dose reduction) and lessening the cost of treatment. Strict guidelines for the evaluation and follow-up of the observation patients are mandated to ensure that disease recurrence or regrowth are detected early.
- A United Kingdom CCG trial is also studying the reduction of total JEB cycles.