Management of ovarian germ cell tumors journal of clinical. Germ cell and stromal tumors of the ovary fred ueland, md university of kentucky gynecologic oncology. It is the third most common germ cell malignancy of the ovary accounting for about 20% of. But, we know that they are more common in people with certain inherited disorders passed down through families, such as klinefelter. Teratomas are the most common type of germ cell tumor to develop in extragonadal not in the ovary or testes areas. Ct imaging of ovarian yolk sac tumor with emphasis on differential. Sep 07, 2006 the primary modality used for imaging patients with gct is computed tomography ct but plain film radiography, ultrasound, magnetic resonance imaging mri and positron emission tomography pet may all have roles to play. In adults 95% are benign and consist of mature cystic teratomas dermoid cysts. There are 2 types of staging for germ cell tumors, based on whether the patient has had surgery yet. The role of staging and adjuvant chemotherapy in stage i malignant. Germ cell tumors are rare, about 900 children and adolescents are diagnosed in the united states each year. Pelvic and abdominal ultrasound was performed every 6 months, whereas.
Mar 01, 2014 an unknown percentage of malignant germ cell tumors reported in phenotypic women may, in fact, represent mtgcts seminomas, embryonal carcinomas, mixed germ cell tumors, as they may have originated from the malignant germ cell component of gonadoblatomas present in dysgenetic gonads of patients with an unrecognized y chromosomecontaining genotype 8, often difficult to demonstrate. As with epithelial ovarian cancers, it is a good idea to consult with a gynecologic oncologist for treating malignant germ cell tumors, especially because these are so uncommon. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary. A family history of germ cell tumors also increases the chance that someone will develop a germ cell tumor.
Ovarian germ cell tumors are rare but very curable at all stages of disease. In section, they appear to consist of different lobules, are soft and. Malignant ovarian germ cell tumors occur in the first four decades and are rare thereafter. Ovarian germ cell tumor dysgerminoma cleveland clinic. While the original tumor showed only mature teratoma, there were obviously yolk sac cells that were not detected on the sections taken from the primary tumor. However, these studies were case reports or usually lacked a comparative.
Germ cell tumors include tumors which arise from the ovum and its precursor germ cells. Continued pregnancy after ovarian germ cell tumors. In addition, having an undescended testicle increases the odds of a male developing a testicular germ cell tumor. Malignant germ cell cancers of the ovary include dysgerminomas and nondysgerminomas, which include immature teratomas, embryonal cell carcinoma, yolk sac tumors, primary ovarian nongestational choriocarcinomas, polyembryoma, and mixed germ cell tumors. Mixed germ cell tumours of the ovary are malignant neoplasms of the ovary comprising of two or more types of germ cell components. Clinical profile and classification germ cell tumors. Code the histology as 90653 germ cell tumor nonseminomatous. Women with benign noncancerous germ cell tumors such as mature teratomas dermoid cysts are cured by removing the part of the ovary that has the tumor ovarian cystectomy or by removing the entire ovary. Whether you can get pregnant after a germ cell tumor depends on the type of surgery you have if only one ovary and fallopian tube are removed.
In children and adolescents, up to 60% of ovarian tumors can be of germ cell origin. Color doppler imaging has a complementary role in the evaluation of testicular tumors. Feb 10, 2016 terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. Dysgerminoma is the most common malignant germ cell tumor, accounting for approximately 3040 % of all malignant neoplasms of germ cell origin. Germ cell tumors gct originate from primordial germ cells and make up 30% of all ovarian tumors. So why would the germ cell tumors be in the ovaries and not the fallopian tube or the endometrium. They are the most common malignant germ cell tumors of the ovary and are thought to account for 1% of all ovarian neoplasms 5. Yst is a rare tumor and comprises only 1% of all ovarian cancers. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant.
Jun 15, 2015 yst is a rare tumor and comprises only 1% of all ovarian cancers. A germ cell tumor gct is a neoplasm derived from germ cells. We do not know exactly why germ cell tumors develop. Malignant germ cell cancers of the ovary include dysgerminomas and nondysgerminomas, which include immature teratomas, embryonal cell carcinoma, yolk sac tumors, primary ovarian nongestational choriocarcinomas, polyembryoma, and mixed germ. They represent 4050% of all benign ovarian neoplasms. Recurrent ovarian mixed germ cell tumor with unusual malignant. Ovarian dysgerminomas are a type of germ cell tumor of the ovary. Ct imaging of ovarian yolk sac tumor with emphasis on differential diagnosis. Imaging of ovarian malignant germ cell tumors mri, ct and petct luca guerra dolci carlotta nuclear medicine molecular bioimaging centre san gerardo hospital university of milan bicocca. Determination of the optimal treatment modalities is crucial, as these malignancies mainly affect girls and young women who may wish to preserve their fertility. Yolk sac tumor yst of the ovary, also known as endodermal sinus tumor, is a primitive malignant germ cell tumor gct that occurs in girls and young women. Germ cells are cells that develop in the embryo which later becomes eggs and sperm in the ovaries and testes respectively. While sharing many similarities with male germ cell tumours gcts, a group of.
Cellular classification and clinical and imaging features1 ovarian malignant germ cell tumors omgcts are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad. Germ cells tumors of the ovary account for approximately 1520% of all ovarian tumors. Ovarian germ cell tumor is a disease in which malignant cancer cells form in the germ egg cells of the ovary. First, the clinical stage is based on the results of tests done before surgery, such as a physical examination, ct scans, and mri tests. Most of the malignant mixed germ cell tumours consists of dysgerminoma accompanied by endodermal sinus tumours, immature teratoma or choriocarcinoma. Malignant ovarian germ cell tumors mogcts are rare but. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. Although ovarian tumors have similar clinical and radiologic features, predominant or specific imaging features may be present in some types of ovarian tumors. Germ cell ovarian cancer md anderson cancer center.
Bilateral malignant ovarian germ cell tumors mogcts are rare. If you just found out that you or your child has a germ cell tumor, you may have a lot of questions about what it means and what comes next. Germ cell a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. The earlier the gonadal development program is interrupted by a crucial defect, the less the gonad is able to differentiate into either a testis or. Data from the surveillance, epidemiology and end results program 19782010, investigators found a. An unknown percentage of malignant germ cell tumors reported in phenotypic women may, in fact, represent mtgcts seminomas, embryonal carcinomas, mixed germ cell tumors, as they may have originated from the malignant germ cell component of gonadoblatomas present in dysgenetic gonads of patients with an unrecognized y chromosomecontaining genotype 8, often difficult to.
Management of female malignant ovarian germ cell tumours. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. There are four main types of ogcts, namely dysgerminomas, yolk sac tumor, teratoma, and choriocarcinoma. Full text malignant mixed ovarian germ cell tumor composed of. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. There are many kinds of germ cell tumors and only some.
Germ cell tumors most commonly appear in the gonads sex organs. There have been several published reports of ovarian germ cell cancers affecting more than one family member. Authors have also contributed articles on the genetics and epigenetics of germ cell tumor development in humans and in the mouse model system. Management of bilateral malignant ovarian germ cell tumors. Ovarian germ cell tumors ogcts are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad, which accounts for about 2. Malignant gct make up 3% and 20% of all ovarian cancers in western countries and asianafrican countries respectively. Ct and mr imaging of ovarian tumors with emphasis on. Malignant germ cell tumors account for less than 5% of all ovarian neoplasms 1. Germ cell tumors and their association with pregnancy intechopen.
The mixed solid components are characterized by a soft gray to yellow solid. Germ cell tumours of ovary by dr sreelasya kakarla sri siddhartha medical college and research hospital, tumkur 2. Epidemiology they are rare ovarian tumors that occ. Yolk sac tumor yst of the ovary, also known as endodermal sinus tumor, is a. The nongerm cell tumors include the sertoli cell and the leydig cell tumors. In fact, eighty percent of preadolescent malignant ovarian tumors are gcts. The majority of germ cell tumors show the presence of multiple histologies. Germ cell tumors can spread to lymph nodes, lung, liver, and brain.
So, a germ cell ovarian cancer refers to situations where. Ovarian malignant germ cell tumors omgcts are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad. They make up only 4% of all cancers in children and adolescents. Ovarian germ cell tumors treatment pdqpatient version. Extragonadal germ cell tumors are a group of neoplasms thought to develop from germ cell precursors that become arrested during embryologic migration and survive in ectopic locations. Flamant f, caillou b, pejovic mh, gerardmarchant r, gout m, lemerle j, sarrazin d, zucker jm, schweisguth 0 1978 prognostic factors in malignant germ cell tumors of the ovary in children excluding pure dysgerminoma. Germ cell tumors can arise from the ovary or from other locations of the body due to germ cell migration. Germ cells normally occur inside the gonads ovary and testis. It is commonly seen in the 2235 age group and the patients present with an enlarged scrotum and pain. Dysgerminoma is a malignant germ cell tumor of the ovary believed to arise from primordial germ cells, similar to the testicular seminoma counterpart. Molecular characteristics of malignant ovarian germ cell tumors. The consistency of dysgerminoma varies from firm and rubbery in small and mediumsized tumors to soft in large ones. With this, the neoplasm can metastasize and affect other areas of the body.
These are uncommon neoplasms that correspond to less than 1% of all malignant ovarian tumors. Malignant mixed germ cell tumour of ovary an unusual. Literature suggests that the only known risk factor for extragonadal germ cell tumors egcts is klinefelter syndrome 47xxy, which is associated with mediastinal nonseminomatous germ cell tumors. In children and adolescents, up to 60% of ovarian tumors can be of germ. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Germ cell tumor gct, dysgerminoma, metastatic ovarian cancer.
Germ cell tumors atlas of genetics and cytogenetics. More than 95% of gct are benign dermoid cysts mature cystic teratomas. Determining the diagnosis based on imaging may prove difficult, and combining the imaging findings with the clinical scenario is very helpful in reaching a. It is the third most common germ cell malignancy of the ovary accounting for about 20% of malignant germ cell tumors 5. It is the third most common germ cell malignancy of the ovary. Descriptions of this tumor in radiological literature range from entirely solid to predominantly cystic 1,2,3. 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. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. About 23% of all ovarian cancers are germ cell tumors. Germ cell tumors are growths that occur in children, teens and adults. Extragonadal germ cell tumors treatment pdqpatient.
Ovarian germ cell tumors ogcts are heterogeneous tumors that are derived from the. Germ cell tumors of the ovary radiology reference article. Magnetic resonance imaging mri may provide additional information over ct imaging of the ovaries. They can be reliably diagnosed with ultrasound imaging. Although their occurrence in the sinonasal tract is exceedingly rare, a number of histologic subtypes have been recognized. However, these tumours are rare, occurring annually in. With germ cell ovarian cancer, germ cell refers to the precursor cells that develop into eggs, ovarian refers to ovary which is where the germ cells live, of which there are two that sit on either side of the uterus. Ovarian tumors are classified as epithelial tumors, germ cell tumors, sex cordstromal cell tumors, and metastatic tumors on the basis of tumor origin, table 1, 1. Ovarian germ cell tumours gcts account for approximately five percent of all ovarian cancers and are most commonly diagnosed in young women. Womens imaging 777 ovarian malignant germ cell tumors. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary the ovaries are a pair of organs in the female reproductive system.
Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are 1associated with high cure rates of 85. Malignant ovarian germ cell tumors spread in the same manner as. They make up about 3% of all cases of childhood cancer. Malignant ovarian germ cell tumors mogcts are rare ovarian tumors that. Germ cell tumours tend to affect only one ovary, and most are curable even if diagnosed at an advanced stage. Ovarian germ cell tumors usually occur in teenage girls or young women and. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. In rare cases, childhood germ cell tumors can be passed down from one generation to. Germ cell tumors begin in the reproductive cells egg or sperm of the body. They are asymptomatic until complications such as torsion or rupture arise. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. Silly question, but you can only get germ cell tumors cyctic teratoma, dysgerminoma, endodermal sinus tumor and choriocarcinoma from a fertilized egg after having sex right. Seventyfive percent of dysgerminomas occur between the ages of 10 and 30 years and 5 % occur before the age of 10 years and rarely after age 50 1, 9.
Ovarian germ cell tumor treatment ovarian teratoma treatment. The embryonal variety of nsgct, which has been illustrated here has a rapid and bulky growth and metastasizes early. Germ cell tumors of the ovary are presumed to derive from the pathogenic transformation of ovarian germ cells at different stages of development. The american joint committee on cancer ajcc tnm classification and the international federation of gynecology and obstetrics figo staging system for germ cell tumors are listed below see tables 1 and 2. In general, all women with malignant germ cell tumors will have the same staging surgery that is done for epithelial ovarian cancer. Ct imaging of ovarian yolk sac tumor with emphasis on. While sharing many similarities with male germ cell tumours gcts, a group of cancers 20 times more common than mogcts. Ovarian germ cell tumors ogcts comprise 2025% of all ovarian cancers. A germ cell tumour is slightly different than a typical ovarian cancer as it starts in the egg producing part of the ovary. Most ovarian cancers are a type called epithelial cancer.