Ovarian Dysgerminomas

Ovarian Dysgerminomas

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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 approximately 2% of all ovarian cancers.

Although rare, dysgerminomas are important irrespective of incidence because they most commonly 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 well to therapy, potentially sparing patients from infertility and early mortality.

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, owing to the lack of normal contact inhibition, hence germ cell tumor (GCT) formation. All dysgerminomas are considered malignant, but only one third of dysgerminomas behave aggressively. The exact etiology of dysgerminomas has not been determined, although recent molecular studies have implicated loss of function with potential tumor suppressor gene TRC8/RNF139 as a possible etiology. [1]

Additionally, 5% of all dysgerminomas occur in dysgenetic gonads and may be associated with gonadoblastomas. Genetic disorders of the ovary are associated with karyotypic abnormalities and are discussed in Dysgerminomas in patients with karyotypic abnormalities in Complications.

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 tumors (42%); dysgerminoma and other germ cell tumors (GCTs) (30%); metastatic Krukenberg tumors (14%); and sex cord stromal tumors (ie, Sertoli-Leydig cell tumors) (13%).

International

No data are available.

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.

To date, no racial predilection exists for ovarian germ cell tumors (GCTs).

These tumors mostly occur in women, although the disease also occurs in pseudohermaphrodites and patients with gonadal dysgenesis (see Complications). Testicular seminomas are the male histologic counterparts to dysgerminomas.

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.

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Gershenson DM, Miller AM, Champion VL, et al. Reproductive and sexual function after platinum-based chemotherapy in long-term ovarian germ cell tumor survivors: a Gynecologic Oncology Group Study. J Clin Oncol. 2007 Jul 1. 25(19):2792-7. [Medline].

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Williams SD, Kauderer J, Burnett AF, Lentz SS, Aghajanian C, Armstrong DK. Adjuvant therapy of completely resected dysgerminoma with carboplatin and etoposide: a trial of the Gynecologic Oncology Group. Gynecol Oncol. 2004 Dec. 95(3):496-9. [Medline].

Shah R, Xia C, Krailo M, Amatruda JF, Arul SG, Billmire DF, et al. Is carboplatin-based chemotherapy as effective as cisplatin-based chemotherapy in the treatment of advanced-stage dysgerminoma in children, adolescents and young adults?. Gynecol Oncol. 2018 Aug. 150 (2):253-260. [Medline].

De Palo G, Lattuada A, Kenda R, Musumeci R, Zanini M, Pilotti S, et al. Germ cell tumors of the ovary: the experience of the National Cancer Institute of Milan. I. Dysgerminoma. Int J Radiat Oncol Biol Phys. 1987 Jun. 13(6):853-60. [Medline].

Nasioudis D, Frey MK, Chapman-Davis E, Caputo TA, Holcomb K. Fertility-preserving surgery for advanced stage ovarian germ cell tumors. Gynecol Oncol. 2017 Dec. 147 (3):493-496. [Medline].

Mangili G, Sigismondi C, Lorusso D, et al. Is surgical restaging indicated in apparent stage IA pure ovarian dysgerminoma? The MITO group retrospective experience. Gynecol Oncol. 2011 May 1. 121(2):280-4. [Medline].

Gershenson DM, Copeland LJ, del Junco G. Second-look laparotomy in the management of malignant germ cell tumors of the ovary. Obstet Gynecol. 1986 Jun. 67(6):789-93. [Medline].

Solheim O, Gershenson DM, Trope CG, et al. Prognostic factors in malignant ovarian germ cell tumours (The Surveillance, Epidemiology and End Results experience 1978-2010). Eur J Cancer. 2014 Jul. 50(11):1942-50. [Medline].

American College of Obstetrics and Gynecology. Educational bulletin: Ovarian Cancer. ACOG compendium of selected publications. No 250. 2000:667-675.

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Chad M Michener, MD Associate Professor of Surgery, Cleveland Clinic Lerner College of Medicine; Vice Chair, Department of Obstetrics and Gynecology, Main Campus, Division of Gynecologic Oncology, Obstetrics, Gynecology and Women’s Health Institute, Cleveland Clinic

Chad M Michener, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Society of Gynecologic Oncology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: MediBeacon<br/>investor for: Medasync.

Allan Y Wu, MD Director, The Midwest Women’s Specialty Group; Adjunct Clinical Professor, Department of Molecular Biology, The Terre Haute Center for Medical Education, Indiana University School of Medicine

Allan Y Wu, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Michel E Rivlin, MD Former Professor, Department of Obstetrics and Gynecology, University of Mississippi School of Medicine

Michel E Rivlin, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Medical Association, Mississippi State Medical Association, Royal College of Surgeons of Edinburgh, Royal College of Obstetricians and Gynaecologists

Disclosure: Nothing to disclose.

Warner K Huh, MD Professor, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Senior Scientist, Comprehensive Cancer Center, University of Alabama School of Medicine

Warner K Huh, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Surgeons, Massachusetts Medical Society, Society of Gynecologic Oncology, American Society of Clinical Oncology

Disclosure: I have received consulting fees for: Merck; THEVAX.

Ovarian Dysgerminomas

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