eMedicine Specialties > Obstetrics and Gynecology > Gynecologic Oncology
Ovarian Dysgerminomas
Updated: Oct 7, 2008
Introduction
Background
The 3 major types of ovarian tumors are epithelial, sex cord, and germ cell. Epithelial cell tumors represent the majority of all ovarian neoplasms (82%). Conversely, germ cell tumors (GCTs) are rare, comprising approximately 20% of all ovarian tumors, both benign and malignant. Approximately 3-5% of ovarian GCTs are malignant. The most commonly occurring GCT is the dysgerminoma, which accounts for only 1-5% of all ovarian cancers.
Although rare, dysgerminomas are important irrespective of incidence because they affect women of reproductive age (ie, <30 y). In fact, dysgerminomas make up two thirds of all malignant ovarian neoplasms in women younger than 20 years. Moreover, once diagnosed, dysgerminomas respond highly to the prescribed treatments, rescuing patients from infertility and early mortality.
Pathophysiology
Typically, germ cells are encapsulated at birth within the primordial follicle. If they somehow escape encapsulation, cell death usually occurs. If the germ cells survive, rapid growth ensues because no cellular contexts can provide normal contact inhibition, hence GCT formation. All dysgerminomas are considered malignant, but only one third of dysgerminomas behave aggressively. The exact etiology of dysgerminomas has not been determined.
Frequency
United States
The incidence of dysgerminomas has remained unchanged over the last 30 years.
The frequencies of the most common malignant ovarian neoplasms in women of reproductive age are as follows: epithelial (42%); dysgerminoma and other GCTs (30%); metastatic Krukenberg (14%); and sex cord stromal, ie, Sertoli-Leydig cell tumors ([SLCT] 13%).
International
No data are available.
Mortality/Morbidity
The 5-year survival rate is 96% if the tumor is confined to the ovary and 63% if extension occurs beyond the ovaries. Pregnancy does not alter the prognosis of most ovarian malignancies, but complications such as torsion and rupture may increase the incidence of spontaneous abortion or preterm delivery.
Race
To date, no racial predilection exists for ovarian germ cell tumors.
Sex
These tumors mostly occur in women, although the disease also occurs in pseudohermaphrodites and patients with gonadal dysgenesis (see Medical/Legal Pitfalls). Testicular seminomas are the male histologic counterparts to dysgerminomas.
Age
Although most ovarian cancers occur during the menopausal and perimenopausal years (ie, 50-59 y), dysgerminomas tend to occur frequently in the pediatric population. Dysgerminomas are most commonly observed in younger women. Seventy-five percent of dysgerminomas occur in patients in the third and fourth decades of life, with the mean age being 22 years.
Clinical
History
No specific symptoms are diagnostic of dysgerminoma tumors. Many of the presenting symptoms are universal for any adnexal/ovarian mass.
- Most common presenting symptoms include the following:
- Pelvic fullness
- Pain
- Early satiety
- Urinary frequency
- Dysuria
- Vague abdominal symptoms (eg, dyspepsia, digestive disturbances) are less common.
- These tumors usually present as a unilateral mass and can occur during pregnancy.
Physical
The physical examination should include a thorough abdominal and pelvic examination on a gynecology table with stirrups. The examiner also must perform a careful rectovaginal examination because some enlarged adnexal masses can be detected from this approach.
Causes
The exact etiology for this tumor type has not been elucidated.
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References
American College of Obstetrics and Gynecology. Educational bulletin: Ovarian Cancer. ACOG compendium of selected publications. No 250;2000:667-675.
Cotran RS, Kumar V, Robbins SL. Female genital tract. In: Robbins Pathologic Basis of Disease. Philadelphia, Pa: WB Saunders; 1996:1127-1180.
Disaia PJ, Creasman WT. Clinical Gynecologic Oncology. Mosby-Year Book; 1997:282-374.
Gershenson DM, Copeland LJ, del Junco G. Second-look laparotomy in the management of malignant germ cell tumors of the ovary. Obstet Gynecol. Jun 1986;67(6):789-93. [Medline].
Harvey RA, Champe PC, Mycek MJ. Anticancer drugs. In: Lippincott's Illustrated Reviews: Pharmacology. Baltimore, Md: Lippincott Williams & Wilkins; 1992:337-360.
Hoskins WJ, Perez CA, Young RC. Epithelial ovarian cancer. In: Principles and Practice of Gynecologic Oncology. Baltimore, Md: Lippincott Williams & Wilkins; 1992:715-781.
Isselbacher KJ, Braunwald E, Wilson JD. Principles of cancer therapy. In: Harrison's Principles of Internal Medicine. 13th ed. New York, NY: McGraw-Hill; 1994:1826-1840.
Thompson JD, Rock JA. Surgical treatment of ovarian cancer. In: Telinde's Operative Gynecology. Baltimore, Md: Lippincott Williams & Wilkins; 1992:1303-1328.
Further Reading
Keywords
ovarian dysgerminomas, germ cell tumors, GCT, germinomas, malignant germ cell tumor, primitive germ cells, mixed germ cell tumor, dysgenic gonad, gonadoblastoma, epithelial ovarian tumors, sex cord tumors, metastatic Krukenberg tumors, sex cord stromal tumors, Sertoli-Leydig cell tumors, SLCT, malignant ovarian neoplasms, bilateral salpingo-oophorectomy, hysterectomy
Overview: Ovarian Dysgerminomas