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Table of Contents
Definition / General | Terminology | Epidemiology | Sites | Clinical Features | Diagnosis | Laboratory | Prognostic Factors | Case Reports | TreatmentDefinition / General
- Ovary is most common site of germ cell tumors in females
- 20 - 30% of ovarian tumors are germ cell tumors
- Germ cell tumor histogenesis varies depending on neoplastic transformation of germ cells into either mature or embryonal (multipotential) cells
Terminology
- Germ cell tumor
- Gonadal or extragonadal
- Types:
- Mature cystic teratoma (dermoid cyst)
- Dysgerminoma
- Yolk sac tumor (endodermal sinus tumor)
- Embryonal carcinoma
- Polyembryoma
- Choriocarcinoma
- Immature teratoma
- Monodermal teratoma (struma ovarii, carcinoid, neuroectodermal tumors, sebaceous tumors)
- Mixed malignant germ cell tumor
Epidemiology
- Usually children and young adults
Sites
- Ovary, testis
- Extragonadal sites include retroperitoneum, mediastinum, midline of CNS
Clinical Features
- Sudden onset abdominal pain, abdominal enlargement
- Most present as large (mean 16 cm) unilateral mass, except for dysgerminoma (15% bilateral)
- Frequency of germ cell tumors that are malignant: 1/3 of childhood tumors, 75% of tumors in women < age 30 years; 3% of tumors in Western countries, 30% of tumors in women of color or Asian ancestry
- 60 - 75% are diagnosed at stage I at diagnosis; 25 - 35% at stage III
- Resemble germ cell tumors in testis
- 95% are dermoid cyst or mature teratoma - rarely contain immature or primitive malignant elements, usually in older patients
- 8 - 10% of malignant germ cell tumors are mixed (2+ types of germ cell tumor) - most common subtype has dysgerminoma and yolk sac tumor (endodermal sinus tumor)
- Survival: 95% disease free survival due to chemotherapy with bleomycin, etoposide and cisplatin
Diagnosis
- Sample tumors extensively
- Record relative amount of microscopic components of each element in a mixed type germ cell tumor
Laboratory
- Elevated hCG in choriocarinoma and tumors containing syncytiotrophoblasts
- Elevated serum AFP in yolk sac tumor
- Elevated hCG and AFP in embryonal carcinoma, polyembryoma
Prognostic Factors
- Type of tumor
- Stage of tumor
- hCG
- AFP or other tumor markers elevated at initial diagnosis
Case Reports
- 12 year old girl with systemic mast cell disease 3 months after chemotherapy (Hum Pathol 1998;29:1546)
Treatment
- Surgical cytoreduction, followed by chemotherapy using platinum based regimen with bleomycin, etoposide or other options according to current theory
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