Germ cell tumors of the ovary are uncommon, but aggressive, tumors, which are seen most often in young women or adolescent girls. These tumors are frequently unilateral and are generally curable if found and treated early. The use of combination chemotherapy after initial surgery has dramatically improved the prognosis for many women with these tumors.[1,2,3]
There are several types of plasma cell neoplasms. These diseases are all associated with a monoclonal (or myeloma) protein (M protein). They include monoclonal gammopathy of undetermined significance (MGUS), isolated plasmacytoma of the bone, extramedullary plasmacytoma, and multiple myeloma.
(Refer to the Lymphoplasmacytic Lymphoma (Waldenström Macroglobulinemia) section in the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.)
Incidence and Mortality
One series found a 10-year survival rate of 88.6% following conservative surgery for patients with dysgerminoma confined to the ovary; less than 10 cm in size; with an intact, smooth capsule unattached to other organs; and without ascites. A number of patients had one or more successful pregnancies following unilateral salpingo-oophorectomy. Even patients with incompletely resected dysgerminoma can be rendered disease-free following chemotherapy with bleomycin, etoposide, and cisplatin (BEP) or a combination of cisplatin, vinblastine, and bleomycin, also known as PVB.
Other Germ Cell Tumors
A report of 35 cases of germ cell tumors, half of which were advanced stage or recurrent or progressive disease, demonstrated a 97% sustained remission at 10 months to 54 months after the start of a combination of BEP. Two Gynecologic Oncology Group trials reported that 89 of 93 patients with stage I, II, or III disease who had completely resected tumors were disease-free after three cycles of BEP.[1,3]
Endodermal sinus tumors of the ovary are particularly aggressive. A review of the literature in 1979 prior to the widespread use of combination chemotherapy found only 27% of 96 patients with stage I endodermal sinus tumor alive at 2 years after diagnosis. More than 50% of the patients died within a year of diagnosis.
Patients with mature teratomas usually experience long-term survival, but survival for patients with immature teratomas following surgery only is related to the grade of the tumor, especially its neural elements. In a series of 58 patients with immature teratoma treated before the modern chemotherapeutic era, recurrence was reported in 18% of the patients with grade 1 disease, in 37% of the patients with grade 2 disease, and in 70% of the patients with grade 3 disease. Similar findings have been reported by others.