Malignant Germ Cell Tumors of the Ovary

Malignant germ cell tumors of the ovary, which include dysgerminomas, immature teratomas, embryonal carcinomas, endodermal sinus tumors, and ovarian choriocar-cinomas, are uncommon and rare. Surgery is required for the diagnosis and staging of malignant germ cell ovarian tumors. The staging system for malignant ovarian germ cell tumors is identical with that used for epithelial ovarian cancer. In contrast to epithelial ovarian cancer, malignant germ cell tumors occur primarily in girls and young women. These tumors are now curable, mainly as a result of great advances in chemotherapy in the last two decades. Fertility-sparing surgery has increasingly been used in the management of these women. In a review of 281 patients with ovarian germ cell tumors, Kurman and Norris97 first reported that the prognosis of patients with disease grossly confined to one ovary who were treated with unilateral salpingo-oophorectomy was not worse than that of patients undergoing more radical surgery (Table 7-5). Peccatori and colleagues98 identified 129 patients with germ cell tumors, 108 of whom have been treated by fertility-sparing surgery. The overall survival was 96%, and conservative surgery did not affect the recurrence or survival rates. Zanetta and associates99 reported on 169 women with malignant ovarian germ cell tumors. Fertility-sparing surgery was performed in 138 of these patients.

Table 7-5. Comparison of Survival after Unilateral Versus Bilateral Salpingo-oophorectomy in 182 Patients with Tumors Grossly Confined to One Ovary

Tumor No. of Patients Treatment Actuarial Survival (%)

Dysgerminoma 46 SO 91

21 BSO _90

Dysgerminoma 46 SO 91

21 BSO _90

Endodermal sinus tumor

27

SO

91* 22

22

BSO

-i

Immature teratoma

34

SO

16T 76

6

BSO

66 70*

Embryonal carcinoma

7

SO

57

1

BSO

íznt

Mixed germ cell tumors

13

SO

50* 54

5

BSO

40

50*

BSO, bilateral salpingo-oophorectomy; SO, unilateral salpingo-oophorectomy.

From Kurman RJ, Norris HJ: Malignant germ cell tumors of the ovary. Hum Pathol 8:551-564, 1977.

BSO, bilateral salpingo-oophorectomy; SO, unilateral salpingo-oophorectomy.

From Kurman RJ, Norris HJ: Malignant germ cell tumors of the ovary. Hum Pathol 8:551-564, 1977.

Table 7-6. Status of Contralateral Ovary in 191 Patients with Stage I Tumors

Tumor

No. of Patients Stage IA Stage IB

No. of Grossly Normal

Ovaries Examined No. Positive for

Microscopically Occult Tumor

Dysgerminoma

67

11

21

4*

Endodermal sinus tumor

51

0

24

0

Immature teratoma

40

0

6

0

Embryonal carcinoma

9

0

0

0

Mixed germ cell tumor

19

1

5

1+*

*Dysgerminoma developed in two additional patients in a contralateral ovary that appeared normal at the time of operation, 6 and 15 months later.

fDysgerminoma was one of the components of the tumor and was the only type of tumor in the opposite ovary.

Another neoplasm with the same histologic composition developed in the contralateral ovary of an additional patient with a mixed dysgerminoma and endodermal sinus tumor 22 months after the operation.

From Kurman RJ, Norris HJ: Malignant germ cell tumors of the ovary. Hum Pathol 8:551-564, 1977.

*Dysgerminoma developed in two additional patients in a contralateral ovary that appeared normal at the time of operation, 6 and 15 months later.

fDysgerminoma was one of the components of the tumor and was the only type of tumor in the opposite ovary.

Another neoplasm with the same histologic composition developed in the contralateral ovary of an additional patient with a mixed dysgerminoma and endodermal sinus tumor 22 months after the operation.

From Kurman RJ, Norris HJ: Malignant germ cell tumors of the ovary. Hum Pathol 8:551-564, 1977.

The survival rate for women who were treated conservatively was 98% for dysger-minomas, 90% for endodermal sinus tumors, and 100% for either mixed types or immature teratomas. Fifty-five conceptions and 40 term pregnancies were recorded, indicating a marginal effect of treatment on fertility. The contralateral ovary should not be biopsied if it appears grossly normal, unless the tumor is a dysgerminoma or a mixed germ cell tumor with a component of dysgerminoma because the risk of occult contralateral ovarian involvement is up to 20%97 (Table 7-6).

The role of cytoreductive surgery for advanced-stage malignant germ cell tumors of the ovary is less well established. Nevertheless, the same principles of cytoreduc-tive surgery that have been applied for epithelial ovarian cancer are generally recommended with maximum resection to achieve the smallest amount of residual disease. Slayton and colleagues100 examined 76 patients with malignant germ cell tumors of the ovary who received vincristine, dactinomycin, and cyclophosphamide as part of the GOG 44 phase II protocol. Twenty-eight percent of the patients with completely resected disease failed vincristine, adriamycin, cyclophosphamide (VAC) therapy compared with 68% of patients with incompletely resected tumors. Similar findings were recorded in the follow-up GOG 45 study of the combination vinblastine, bleomycin, and cisplatin in which 83% of the patients with no measurable disease at the start of chemotherapy remained disease-free compared with 42% of patients with measurable disease101 (Table 7-7). In their review of 33 patients with malignant germ cell tumors of the ovary, Bafna and associates102

Table 7-7. Disease-Free Survival by Pretreatment Characteristics in Patients with Tumors other than Dysgerminoma

Patient Characteristics No. Disease-Free/Total (%)

Cell type

Patient Characteristics No. Disease-Free/Total (%)

Cell type

Endodermal sinus

16/29 (55)

Embryonal

1/4 (25)

Mixed

14/27 (52)

Immature teratoma

14/26 (54)

Choriocarcinoma

2/3 (67)

Age

<20

23/38 (61)

20-29

15/33 (45)

30-39

8/13 (62)

40-49

0/4 (0)

>50

1/1 (100)

Stage

II

5/5 (1 00)

III

22/37 (60)

IV

5/9 (56)

Recurrent

15/38 (40)

Measurable disease

Yes

12/35 (34)

No

35/54 (65)

Previous therapy

Yes*

14/35 (40)

No

33/54 (61)

Residual disease

Optimal At initial surgery

10/12 (83)

After debulking surgery

17/29 (59)

Suboptimal

20/48 (42)

Markers

Elevated

32/56 (57)

Normal

13/27 (48)

Not done

2/6 (33)

*Chemotherapy, radiation therapy, or both.

From Williams SD, Blessing JA, Moore DH, et ai: Cisplatin, vinblastine, and bleomycin in advanced and recurrent ovarian germ-cell tumors. A trial of the Gynecologic Oncology Group. Ann Intern Med 111:22-27, 1989.

*Chemotherapy, radiation therapy, or both.

From Williams SD, Blessing JA, Moore DH, et ai: Cisplatin, vinblastine, and bleomycin in advanced and recurrent ovarian germ-cell tumors. A trial of the Gynecologic Oncology Group. Ann Intern Med 111:22-27, 1989.

reported that 13 patients had bulky disease (larger than 10 cm) at the start of chemotherapy with bleomycin, etoposide, and cisplatin. Seven of the 13 patients had dysgerminomas, and all of them achieved a sustained complete response, whereas only 3 of the 6 patients with nondysgerminoma tumors achieved a sustained complete response, indicating that aggressive cytoreductive surgery may be more important for nondysgerminomatous tumors.

Despite the success of modern chemotherapy, a small number of patients will present with persistent or recurrent disease. Munkarah and associates103 reported on the M.D. Anderson Cancer Center experience with salvage surgery for malignant ovarian germ cell tumors. Twenty cases were identified, and 16 patients received postoperative chemotherapy following salvage surgery. At the time of analysis, 11 patients were alive and disease-free, 1 was alive with tumor, 6 had died of tumor progression, and 2 had died of treatment-related complications. Survival of patients with immature teratoma who underwent salvage surgery was significantly better than survival of those with other tumor cell types. A case report and review of the literature by Rezk and associates104 again demonstrated that patients with recurrent or persistent immature teratomas might benefit from salvage surgery. Immature tera-tomas may also become mature during chemotherapy, resulting in a growing teratoma syndrome that is surgically unresectable or that may invade and/or obstruct neighboring structures and necessitate prompt surgical intervention.105,106 Finally, unresected mature teratomas can occasionally undergo malignant transformation and tend to be resistant to traditional chemotherapies.107

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