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Germ Cell (Ovary)

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Description, Classification & Epidemiology
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Treatment
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Malignant germ cell tumors of the ovary occur principally in girls and young women (teens or 20s). In certain percentage of cases these tumors are associated with pregnancy.

Ovarian Germ Cell Tumors include:
1. Dysgerminoma
---This is the female equivalent of seminoma
---Majority of pts present with Stage I. But 10% will have bilateral disease.
---Spread to retroperitoneal lymph node is common
---This type of tumor is very radiosensitive (vs non-dysgerminoma)
2. Tumors other than dysgerminoma
Note that many patients will have elements of multiple cell types present.
Embryonal Carcinoma
.........aggressive
Endodermal Sinus Tumor
.........aggressive
Choriocarcinoma
.........aggressive
Mature Teratoma
Immature Teratoma (Grades I, II, III or high-grade vs low-grade)
---Mixed Germ cell Tumors

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DIAGNOSIS
Laparotomy provides the best avenue to properly diagnose, stage, & treat patients with ovarian germ cell tumors.

Dysgerminoma
--diagnosis requires a normal AFP
--increased HCG could be seen

ENDODERMAL SINUS TUMOR
--AFP increased

CHORIOCARCINOMA
--HCG increased


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MANAGEMENT

1. SURGERY. Initial treatment (also diagnostic)
In most patients unilateral salpingo-oophorectomy with preservation of contralateral ovary & the uterus can be performed. Therefore, fertility can be preserved.
In the case of pure dysgerminoma, biopsy of the contralateral ovary should be obtained.
If benign cystic teratoma, then ovarian cystectomy with preservation of normal ovarian tissue is recommended.
If metastatic disease is encountered at initial surgery then it should be treated the same way as advanced epithelial ovarian cancer.
........SECOND-LOOK LAPAROTOMY. Not necessary in patients with tumor completely resected or in those whose tumor was incompletely resected but did not contain any teratoma. If teratoma was present then second-look laparotomy may be beneficial.
2. ADJUVANT CHEMOTHERAPY. Germ cell tumors are very chemosensitive. Stage II & III patients are treated with chemotherapy.
Patients with advanced ovarian germ cell tumors should receive 4 cycles of chemotherapy in full dose & on schedule. Treatment is given regardless of hematologic parameters on shceduled day of treatment.
Patients with high stage or recurrent dysgerminoma should receive BEP just like patients with non-dysgerminomatous tumors.
Patients with completely resected endodermal sinus tumor, embryonal carcinoma, or mixed tumors containing these elements have a very high risk or recurrence after surgery. These patients should receive adjuvant chemotherapy given as soon as possible after initial surgical treatment. Treatment should be started within 7-10 days since they can recur rapidly. For completely resected stage I (grade II or III) immature teratoma, similar approach is recommended.
........BEP (preferred regimen)
........VeIP (persistant disease but platinum sensitive)
........? HDT with stem cell rescue (persistant disease & platinum resistant)
3. RADIATION THERAPY. Patients with DYSGERMINOMA stage I can be treated with adjuvant radiation therapy. However, because of the likely effects on fertility, some patients have been observed after surgery. Therefore, well-staged stage IA dysgerminoma can be observed after unilateral salpingo-oopherectomy regardless of the primary tumor size.

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LATE EFFECTS OF TREATMENT
1. ETOPOSIDE is leukemogenic. However, the incidence of second neoplasm is low in patients who receive the low cumulative etoposide doses that is generally required for all but a small minority of patients.
2. GONADAL FUNCTION & FERTILITY. Although gonadal dysfunction is a risk of chemotherapy, most survivors can anticipate normal menstrual & reproductive function.
3. OTHER LATE EFFECTS. Includes renal dysfunction, neurotoxicity, Raynaud's phenomenon, & ischemic heart disease.