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Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Malignant Gonadal GCTs


General information about clinical trials is also available from the NCI Web site.

Childhood Malignant Ovarian GCT

Most ovarian neoplasms in children and adolescents are of germ cell origin.[1] Ovarian GCTs are very rare in young girls, but the incidence begins to increase in children aged approximately 8 or 9 years, and continues to rise throughout adulthood.[1] Childhood malignant ovarian GCTs can be divided into dysgerminomas (seminomatous) and nonseminomatous malignant GCTs (i.e., immature teratomas, yolk sac carcinomas, mixed GCTs, choriocarcinoma, and embryonal carcinomas). (For information on childhood mature and immature teratomas arising in the ovary, see the Nonsacrococcygeal Teratomas in Children section of this summary. Refer to the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information.)

For stage I ovarian dysgerminomas and immature teratomas, cure can usually be achieved by unilateral salpingo-oophorectomy, conserving the uterus and opposite ovary, and close follow-up observation.[10,15,16,17,18] Chemotherapy can be given if tumor markers do not normalize or if tumors recur.

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.[18] 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]

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