Ovary tumor
Germ cell tumors
General

Authors: Leena Varughese, M.D. (see Authors page)
Editor: Rajit Malliah, M.D.

Revised: 2 June 2016, last major update November 2013

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PubMed Search: Germ cell tumors [title] ovary
Cite this page: Germ cell tumors - general. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorgermcell.html. Accessed December 9th, 2016.
Definition / 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
Treatment
  • Surgical cytoreduction, followed by chemotherapy using platinum based regimen with bleomycin, etoposide or other options according to current theory