There have been several published reports of ovarian germ cell cancers affecting more than one family member. Analysis of germ cell tumors of ovary in a tertiary care hospital. 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. The evolution of systemic treatment for ovarian germ cell cancer has paralleled the advances made in the treatment of testicular germ cell cancers because of the biologic similarity between ovarian and testicular germ cell tumors. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. An immunohistologic study of 21 patients with germ cell tumors of the testis with measured serum levels of chorionic gonadotropin hcg and alphafeto protein afp was undertaken to correlate the. Germ cell tumors atlas of genetics and cytogenetics. Germ cell tumors of the ovary textbook of uncommon. In general, all women with malignant germ cell tumors will have the same staging surgery that is done for epithelial ovarian cancer. Ovarian germ cell tumors treatment pdqpatient version. Germ cell and nonepithelial ovarian cancer oncohema key. Most ovarian cancers are a type called epithelial cancer. Ovarian germcell tumors represent 1520% of all ovarian tumors. 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.
Less than 2% of ovarian cancers are germ cell tumors. Ovarian germ cell malignant tumors are uncommon, and account for approximately 5 percent of ovarian cancer cases. Pathology outlines who classification of ovarian neoplasms. More than 95% of gct are benign dermoid cysts mature cystic teratomas. Germ cells normally occur inside the gonads ovary and testis. Nov 14, 2018 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. Malignant germ cell tumors represent 5% of ovarian tumors. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary. For patients with early stage disease, cure rates approach 100%, while for those with advancedstage disease are at least 75%.
Outcome and staging evaluation in malignant germ cell tumors. Mature teratoma is the most common germ cell tumor and accounts for 40. Ovarian germ cell tumors treatment pdqhealth professional. Handbook for children with germ cell tumors american pediatric. Ovarian germ cell tumors union for international cancer. 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 mixed germ cell tumour of ovary an unusual. Germ cell tumors can arise from the ovary or from other locations of the body due to germ cell migration. The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system. It usually manifests as an abdominal mass or pelvic mass occurring at a mean age of 15 years. Germ cell tumors is a neoplasm which obtained from germ cells. Ovarian germ cell tumors gcts are rare tumors affecting predominantly girls or young women.
Staging system for pediatric ovarian germ cell tumors stage i limited to ovary ovaries. Malignant ovarian germ cell tumors spread in the same manner as epithelial ovarian neoplasms but are more likely to involve regional lymph nodes. Although germ cell tumors of the ovary are morphologically similar to testicular germ cell tumors, they may not necessarily have an identical origin. 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. Germ cell tumors gct originate from primordial germ cells and make up 30% of all ovarian tumors. This accounts for 95 percent of all germ cell tumors and is. Pregnancy tests gestational trophoblastic diseases diagnosis and posttreatment care of ectopic pregnancy as a component of triple test. Ovarian germ cell tumor treatment ovarian teratoma treatment. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. 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. Request pdf germ cell tumors of the ovaries the basic classification of ovarian germ cell tumors has largely remained unchanged for several decades.
A modified version of the third who classification 2003 version of germ cell tumors of the ovary is presented in table 2. Germ cells tumors of the ovary account for approximately 1520% of all ovarian tumors. They represent 4050% of all benign ovarian neoplasms. Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are 1associated with high cure rates of 85. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Diseasefree survival rates in these patients are quite high and recurrences can be managed with secondline chemotherapy. Continued pregnancy after ovarian germ cell tumors. Ovarian germ cell tumors ogcts are rare tumors that comprise a diverse group of histologic subtypes that can either be benign or malignant. Some studies have found that size and histology were the major factors determining prognosis for patients with malignant mixed germ cell tumors of the ovary. Germ cell tumours of ovary germ cell tumours predominantly occur in young females.
Vincristine, dactinomycin, and cyclophosphamide in the treatment of malignant germ cell tumors of the ovary. The field of ovarian germ cell tumors ogcts has remained relatively unchanged in the last 2 decades. Ovarian malignant germ cell tumors rsna publications online. There are several types of both benign and malignant germ cell tumors, including the following. The most common sites for metastasis are the lungs. Ovarian germ cell tumors represent 1520% of all ovarian tumors.
In children and adolescents, up to 60% of ovarian tumors can be of germ cell origin. Table 1 subtypes and characteristics of ovarian germ cell tumours data derived from rice, 1999 and john hopkins pathology, 2001. Germ cell tumors germ cell tumors arise from the ovary s germinal elements and comprise a third of all ovarian neoplasms. These tumors affect both adults and children and therefore the recommendations apply to the eml lists for both adults and children regimen distinctions are included in the present briefing. Germ cell tumors include a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. A study of 20 cases zyxwvutsrqp journal of surgical oncology 59. Most germ cell tumors that are cancerous occur as cancer of the testicles testicular cancer or cancer of the ovaries ovarian cancer. Nonepithelial ovarian cancer esmo clinical practice. These neoplasms comprise approximately 20 to 25 percent of ovarian neoplasms overall, but account for only an approximate 5 percent of all malignant ovarian neoplasms. The specific characteristics of each tumor will have a major impact on the treatment prescribed and the prognosis. They are asymptomatic until complications such as torsion or rupture arise. Embryonal carcinoma these cells are malignant and usually are mixed with germ cell tumors. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. 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.
Malignant gct make up 3% and 20% of all ovarian cancers in western countries and asianafrican countries respectively. With the advent of combination chemotherapy, risk of relapse has markedly decreased. Protocol for the examination of specimens from patients with primary tumors of the ovary, fallopian tube, or peritoneum. Overall, they have a good outlook, with more than 9 out of 10 patients surviving at least 5 years after diagnosis. August 2018 includes ptnm requirements from the 8th edition, ajcc staging manual and 2015 figo cancer report. Dysgerminoma most common of the primitive germ cell tumors most common malignant pure ovarian germ cell neoplasm malignant radiosensitive common age germ cells that show morphologic, ultrastructural, and histochemical similarity to primordial germ cells most common presentation is abdominal enlargement and presence of a mass in the lower abdomen, sometimes with abdominal pain and weight loss. Treatment for germ cell tumors of the ovary treating benign germ cell tumors 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. Cell tumor the mediastinum is the most common site of extragonadal germ cell tumors 11, constituting 5070% of all extragonadal germ cell tumors. Of 63 patients felt to have an intact tumor capsule by the surgeon, pathologic results of capsular integrity were table 1. While sharing many similarities with male germ cell tumours gcts, a group of cancers 20 times more common than mogcts. These updated guidelines on nonepithelial ovarian cancer cover prevention, diagnosis, treatment and followup for early and advanced stages and recurrences of germ cell tumours, sex cordstromal tumours and small cell carcinomas of the ovary hypercalcaemic type.
They are rapidly growing neoplasms that arise from primordial germ cells derived from the embryonal gonad. Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are associated with high cure rates of 85. Authors have also contributed articles on the genetics and epigenetics of germ cell tumor development in humans and in the mouse model system. In addition, the majority are unilateral, with bilateral ovarian involvement occurring in 10% to 15% of pure dysgerminoma, in some mixed germ cell tumors in which the predominant component is dysgerminoma, and in occasional cases of. Most of the malignant mixed germ cell tumours consists of dysgerminoma accompanied by endodermal sinus tumours, immature teratoma or choriocarcinoma. Mixed germ cell tumours of the ovary are malignant neoplasms of the ovary comprising of two or more types of germ cell components.
Work up and staging manoeuvres is similar to that for epithelial ovarian cancer. There are four main types of ogcts, namely dysgerminomas, yolk sac tumor, teratoma, and choriocarcinoma. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. These are uncommon neoplasms that correspond to less than 1% of all malignant ovarian tumors. 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 1. Serum markers of malignant ovarian germcell tumors when an ovarian germcell tumor is suspected, these markers should systematically be tested before surgical intervention, and even before any surgery for a pelvic mass in a young woman. While sharing many similarities with male germ cell tumours gcts, a group of. Primitive gcts comprise dysgerminomas and nondysgerminomatous gct, which include yolk sac tumor, embryonal carcinoma, polyembryoma, nongestational choriocarcinoma, and mixed gcts. Jan 31, 2019 surgical staging is the first step in the management of ovarian germ cell cancer. More than half of mediastinal germ cell tumors are mature teratomas. Germ cell tumors include tumors which arise from the ovum and its precursor germ cells. Mixed germ cell malignancies of the ovary contain two or more elements of the tumors described above. It can be characterized into cancerous or noncancerous cell.
Germ cell tumors can spread to lymph nodes, lung, liver, and brain. Seventysix patients with malignant germ cell tumors of the ovary received vincristine, dactinomycin, and cyclophosphamide vac postoperatively. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Dysgerminoma most common of the primitive germ cell tumors most common malignant pure ovarian germ cell neoplasm malignant radiosensitive common age germ cells that show morphologic, ultrastructural, and histochemical similarity to primordial germ cells most common presentation is abdominal enlargement and presence of a mass. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. Fiftyfour were treated after removal of all gross disease.
They can occur in women of any age, but are more often found in young women or adolescent girls. A teratoma is a rare type of tumor that can contain fully developed tissues and organs, including hair, teeth, muscle, and bone. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. In one series, the most common component of a mixed germ cell tumor was dysgerminoma, which occurred in 80%, followed by est in 70%, immature teratoma in 53%, choriocarcinoma in 20%, and embryonal carcinoma in 16%. The germ cells which are present outside the gonads are the birth defects during the development of embryo. For accreditation purposes, this protocol should be used for the following procedures and tumor types.
Germ cell and stromal tumors of the ovary fred ueland, md university of kentucky gynecologic oncology. Management of female malignant ovarian germ cell tumours. Staging includes peritoneal cytology, hysterectomy, bilateral salpingooophorectomy, omentectomy, and exploration of peritoneal surfaces and diaphragm with biopsies. There were three cases of monodermal benign teratoma, four cases of immature teratoma and one case of malignant transformation. Theyre most common in the tailbone, ovaries, and testicles. For women who still want to be able to have children, the cancerous ovary and the fallopian tube on the same side are removed, but the uterus, the ovary, and the fallopian tube on the opposite side are. Germ cell tumors can spread metastasize to other parts of the body. However, the introduction of new stem cell pluripotency. The mature cystic teratoma, also called dermoid cyst, is by far the most common subtype.
Over the past three decades, survival rates for germ cell tumors have dramatically improved, coincident with more aggressive surgical staging and combination chemotherapy. Some germ cell tumors occur in other areas of the body, such as the abdomen, brain and chest, though its. Principles of diagnosis and treatment apply across all histological types. 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. There are many kinds of germ cell tumors and only some. A germ cell tumor gct is a neoplasm derived from germ cells. Germ cell tumors health encyclopedia university of. Malignant ovarian germ cell tumors ogcts historically carried a poor prognosis, especially among those diagnosed with advanced disease. Germ cell tumor gct, dysgerminoma, metastatic ovarian cancer. Prognostic factors include stage, amount of residual tumor, histologic type and raised tumor markers. Malignant germ cell tumors gct of the ovary account for 23% of all ovarian neoplasms and occur mostly in the second and third decade of life. A germ cell tumour is slightly different than a typical ovarian cancer as it starts in the egg producing part of the ovary. However, it represents only 4% of malignant ovarian germ cell tumors in the ovary, and its confusion with choriocarcinoma and ests in the past accounts for its late identification as a distinct entity.
Dysgerminoma is a malignant germ cell tumor of the ovary believed to arise from primordial germ cells, similar to the testicular seminoma counterpart. These tumors frequently affect only one ovary and are generally curable if found and treated early. Disease progression dosage of tumor markers hcg, ldh and. Apr 10, 2018 germ cell tumor of the ovary is an eminently treatable disease and selected patients can be managed with fertilitypreserving surgery. Protocol for the examination of specimens from patients. Immunoassay techniques are used to quantify the presence of. Ovarian stromal tumors fibroma granulosa cell tumors inhibin, ca125 sertolileydig tumors caca125. 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.
The world health organization histological classification for ovarian tumors separates ovarian neoplasms according to the most probable tissue of origin. Germ cell tumors begin in the reproductive cells egg or sperm of the body. Ovarian germ cell tumor is a disease in which malignant cancer cells form in the germ egg cells of the ovary. The concept of germ cell tumors as a specific group of gonadal neoplasms has evolved over the last several decades.
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