Germ cell tumors

Proper management of germ cell tumors of the ovary is important because many are malignant and occur in young women. It is possible to treat many of these tumors without loss of fertility. The mature cystic teratoma (dermoid cyst) may be the most common ovarian neoplasm while the malignant germ cell tumors are distinctly uncommon: immature teratoma, dysgerminoma, endodermal sinus tumor, and choriocarcinoma.

Teratoma

More than 95% of teratomas are benign mature cystic teratomas, commonly referred to as dermoid cysts. These tumors can occur throughout a woman’s reproductive life and frequently give rise to symptoms of an acute abdomen secondary to either torsion or rupture. Most commonly, dermoid cysts are detected on routine pelvic examination or during the investigation of minor complaints. More than half can be diagnosed by ultrasonography or x-ray through the identification of calcifications, tooth formation, a specific fat-halo sign on x-ray or the characteristic density appearance on ultrasound.

In a young woman, the surgical procedure of choice for a dermoid cyst is cystectomy or enucleation with preservation of as much ovarian tissue as possible. The opposite ovary should also be evaluated by inspection and palpation for the presence of a dermoid tumor. Dermoid cysts have an overt bilateralism rate approaching 15%. However, bilateralism is not always expressed at the time of surgery and may not be detected until months or years later. If the contralateral ovary is normal to inspection and palpation and normal on preoperative ultrasound, no further evaluation is necessary.

Approximately 1% of dermoid cysts will contain a malignancy. Usually these malignancies occur in postmenopausal women and are squamous cell carcinomas. However, a variety of other malignancies also may occur. These tumors usually are found in the solid papilla on the inside of the cyst, the so-called Rokitansky protuberance.

Malignant ovarian teratomas are called “immature teratomas” because their malignant behavior is exhibited by tissues that are embryonic or fetal in appearance. The more immature the tissue, the more malignant its behavior. These immature elements are usually composed of immature neuroepithelial tissue. A microscopic grading system for immature teratomas has been proposed by Norris and seems to correlate well with prognosis.

Most immature teratomas occur in adolescent girls and involve only one ovary. In these patients, the operative treatment consists of unilateral salpingo-oophorectomy. Bilateral extension requires bilateral salpingo-oophorectomy and metastasis requires as much cytoreduction as possible.

A patient with higher grade or metastatic immature teratoma needs additional treatment after surgery with chemotherapy. Radiotherapy has not been proven beneficial and causes sterility. Impressive results have been obtained with combination chemotherapy using bleomycin, etoposide, and cisplatin, or BEP.

Dysgerminoma

The dysgerminoma is the most common malignant germ cell tumor of the ovary. It usually occurs before age 30, often in females with dysgenetic gonads. Most often, it is confined to one ovary at the time of diagnosis. In 15% of cases, the dysgerminoma is not a pure tumor or possesses other germ cell types as well. Therefore, it is important that it is extensively sampled microscopically, particularly in areas of hemorrhage or necrosis, to rule out the presence of a more malignant element.

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