Ovarian germ cell cancer dysgerminoma yolk sac tumour immature teratoma mixed germ cell tumour bep. In the united states, the overall gct incidence is 0. Tumor sites outside the gonad are called extragonadal sites. Germ cell tumors are rare, about 900 children and adolescents are diagnosed in the united states each year. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Yolk sac tumor, second in frequency, occurs alone or in combination with a teratoma. To stage the tumor, doctors will need to know the size of the tumor, if it has spread metastasized and if it has affected lymph nodes and other tissues. The drugs that are commonly used for treating germ cell tumors include bleomycin blenoxane, cisplatin platinol, and etoposide vepesid, toposar. It can be characterized into cancerous or noncancerous cell.
Uso if young ow tahbso granulosa cell tumor bep or vac x 34 cycles malignant as above teratoma embryonal as above bep x 34 cycles carcinoma debulk but bep x 34 cycles preserve fertility endodermal sinus tumor bep x 3 cycles if stage iiiv uso staging if possible dysgerminoma. Intracranial germ cell tumors icgct are rare in north america and europe, occurring in 24% of children with brain tumors between birth and 19 years of age 6,44,65. Sometimes, a group of germ cells grows in a way thats not normal. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. I had a mediastinal primary germ cell tumor, diagnosed october 2007 and had an excellent response to therapy. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. More than half of mediastinal germ cell tumors are mature teratomas. In rare cases, childhood germ cell tumors can be passed down from one generation to the next. Germ cells normally occur inside the gonads ovary and testis. Mixed germ cell tumor of the testis is made up of 2 or more different types of germ cell tumors. A biopsy is the removal of a small amount of tissue for examination under a microscope. Germ cell tumors can spread metastasize to other parts of the body. T staging ptx primary tumor cannot be assessed if no radical orchiectomy has been performed, tx is used pt0 no evidence of primary tumor e.
Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. A multicentre retrospective cohort study of ovarian germ cell tumours. And finally, choriocarcinoma is a strictly malignant tumor made of germ cells. The name comes from the fact that these tumors occur in a germ cell, one that germinates life such as in the testes or ovaries in an organism that reproduces sexually. By contrast, icgct is the most common supratentorial brain tumor in japan, taiwan, and korea, accounting for 1115% of brain tumors in children and adolescents 14, 30, 49, 67. Although testicular germ cell tumors are sensitive to radiation therapy, these tumors usually present with widely metastatic disease that is not amenable to radiation treatment. May 31, 2015 intracranial germ cell tumors icgct are rare in north america and europe, occurring in 24% of children with brain tumors between birth and 19 years of age 6,44,65. There are several subtypes of nongerminomatous germ cell tumor, including teratomas, choriocarcinomas, endodermal sinus tumors yolk sac tumors, embryonal carcinomas and mixed tumors. In children and adolescents, up to 60% of ovarian tumors can be of germ cell origin. A family history of germ cell tumors also increases the chance that someone will develop a germ cell tumor. Most germ cell tumors of the fetus and infant are histologically benign and are diagnosed as either mature or immature teratomas table 2. The tumors also occur along the midline path and can be found in the head, chest, abdomen, pelvis and sacrococcygeal lower back area. Most patients with a cancerous germ cell tumor will need chemotherapy.
About 900 children and adolescents are diagnosed with germ cell tumors in the u. There are several types of both benign and malignant germ cell tumors, including the following. Handbook for children with germ cell tumors american pediatric. 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. There have been several published reports of ovarian germ cell cancers affecting more than one family member. Germ cell tumors are growths that form from reproductive cells. Families with multiple cases of testicular cancer are rare and almost all those reported have only 2 affected members. Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain. Germ cell tumors are growths that occur in children, teens and adults. However, in view of the excellent chemosensitivity of germ cell cancers, clinical judgment can be used if surgical management would significantly increase postoperative morbidity. Isbn 9789535104568, pdf isbn 9789535169482, published 20120330.
Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. Bajorin d, katz a, chan e, geller n, vogelzang n, bosl gj. Germ cell tumors danafarberboston childrens cancer. Gcts are thought to originate from primordial germ cells, which migrate to the primitive gonadal glands in the urogenital ridge. In addition, having an undescended testicle increases the odds of a male developing a testicular germ cell tumor. These tumors are most commonly found in the ovary, testes, and sacrococcygeal areas tail bone, or distal end of spinal column. Germ cell tumors include the germinoma, the teratoma, the more aggressive embryonal carcinoma and yolk sac endodermal sinus tumors and the choriocarcinoma. Metastatic testicular germ cell tumors were recognized as curable shortly after the discovery of drugs that kill dividing human cells. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary. In north america, germ cell tumors gcts comprise approximately 1% of primary brain tumors in pediatric and young adult patients with a median age of diagnosis of 16 years 2.
Germ cell tumors can spread to lymph nodes, lung, liver, and brain. Belly pain and swelling caused by tumor bathroom troubles a hard time pooping or holding in your pee, if the tumor is near your pelvis breast growth, pubic hair, or vaginal bleeding at an earlier age than normal. The chemotherapy chemo used to treat germ cell tumors depends on the location of the tumor, the type of tumor and the stage of disease. Among these, a common form is teratoma with endodermal sinus tumor. Germ cell tumors gcts are not tumors caused by germs. 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. Many factors also affect the outlook for a good outcome. Most germ cell tumors that are cancerous occur as cancer of the testicles testicular cancer or cancer of the ovaries ovarian cancer. After a germ cell tumor is diagnosed, doctors need to assess how advanced it is, which is called staging the tumor. Os within a clinical trial depends on the number of patients treated at the. Germ cell cancer is a rare tumor that affects children, teens, and adults. Understanding the treatment strategies of intracranial germ. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound.
Most chapters are focused on testicular germ cell tumors whose incidence has been increasing in young males. Nonseminomatous germ cell tu mors nsgcts include teratoma, embry onal carcinoma, endodermal sinus tumor. Ovarian neoplasms coelomic epithelium germ cell specialized gonadal stroma nonspecific mesenchyme metastatic tumors 5070% 1520% 510% 510% 510%. Literature suggests that the only known risk factor for the germ cell tumors gcts is klinefelter syndrome 47xxy, which is associated with mediastinal nonseminomatous germ cell tumors. However, testicular stromal tumors are often benign not cancerous. Management of germ cell tumors in children open access lmu. Germ cell tumors atlas of genetics and cytogenetics. A germcell tumor gct is a neoplasm derived from germ cells. Pediatric germ cell tumors gcts are the most important raretumors in childhood and adolescence and are often asymptomatic until theybecome an adnexal mass, compressing neighboring structures. Germ cell tumors begin in the reproductive cells egg or sperm of the body. A patient may receive 1 drug at a time or combinations of different drugs given at the same time. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. Stage 4 the tumor has spread to other areas of the body.
This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. Pdf human germ cell tumors are developmental cancers. Ovarian germ cell tumors treatment pdqpatient version. The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system. Endodermal sinus tumor or yolk sac tumors are germ cell tumors that are most often malignant, but may also be benign. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. Because germ cell tumors can vary greatly, and can arise in different parts. Mar 23, 2020 literature suggests that the only known risk factor for the germ cell tumors gcts is klinefelter syndrome 47xxy, which is associated with mediastinal nonseminomatous germ cell tumors. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Germ cell tumors of the gonads have a better prognosis than germ cell tumors outside the gonads extragonadal. Many factors go into a doctors decision about how to best treat germ cell tumors. When possible, the first choice is to try and remove the entire tumor. If disease appears to be limited to the ovaries or pelvis, a pelvic and paraaortic lymphadenectomy should also be performed.
A germ cell is a type of cell that forms as a fetus unborn baby develops. 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. Understanding the treatment strategies of intracranial. For childhood extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests. Ovarian germ cell tumor is a disease in which malignant cancer cells form in the germ egg cells of the ovary. Granulosa cell tumor bep or vac x 34 cycles malignant as above teratoma embryonal as above bep x 34 cycles carcinoma debulk but bep x 34 cycles preserve fertility endodermal sinus tumor bep x 3 cycles if stage iiiv uso staging if possible dysgerminoma. Germ cell tumors most commonly appear in the gonads sex organs.
As the name implies, these tumors arise from various germ cells found in the brain. About 5 percent of testicular cancers start in stromal cells, which make testosterone. Germ cells tumors of the ovary account for approximately 1520% of all ovarian tumors. Childhood germ cell tumors md anderson cancer center. Embryonal carcinoma is a malignant tumor originating from germ cells and represents a mixture of different cell types. Germ cell tumors are found widely throughout the body, and encompass a wide range of individual tumors this article does not deal with any specific body locations. By contrast, icgct is the most common supratentorial brain tumor in japan, taiwan, and korea, accounting for 1115% of brain tumors in children and adolescents 14,30,49,67. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. However, they grow and spread like nonseminomas, and for this reason, they are treated as nonseminomas. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. An attempt is made to remove all germ cell tumors with surgery.
It deals with the clinical presentations, treatment modalities, the biology and genetics of germ cell tumors in children and adults. Although yolk sac tumor of the cervix or vagina is the subject of a few case reports, yolk sac tumor of the infant. Disease progression dosage of tumor markers hcg, ldh and. Dec 10, 2012 most germ cell tumors of the fetus and infant are histologically benign and are diagnosed as either mature or immature teratomas table 2. The book aims to provide an overview of current knowledge regarding germ cell tumors.
Successfully treating your childs germ cell tumor depends on where the tumor is located and whether it is benign or malignant. In poor prognosis nonseminomatous germ cell tumours nsgct, overall survival. It actually originates from the placenta and may soon spread to different organs most commonly the lungs. The specific characteristics of each tumor will have a major impact on the treatment prescribed and the prognosis.
These cells later become sperm in the testicles or eggs in the ovaries this summary is about germ cell tumors that form in parts of the body that are extracranial outside the brain. In rare cases, childhood germ cell tumors can be passed down from one generation to. Prognostic factors for favorable outcome in disseminated germ cell tumors. The drugs that are commonly used for treating germ cell tumors include bleomycin blenoxane. Pdf testicular tumors are the most common tumors in adolescent and young men and germ cell tumors tgcts account for most of all testicular cancers. Germ cell tumors represent about 3 percent of all childhood cancers. However, a germ cell brain tumor can spread to other parts of. Jan 31, 2019 if disease involves the upper abdomen, a surgical effort to remove all visible tumor should be made. Surgery the location of the tumor may influence the need or possibility for surgery. Different types of these germ cells cause different types of tumors, including the following. Surgical staging is the first step in the management of ovarian germ cell cancer. The germ cells which are present outside the gonads are the birth defects during the development of embryo. Cell tumor the mediastinum is the most common site of extragonadal germ cell tumors 11, constituting 5070% of all extragonadal germ cell tumors. They make up only 4% of all cancers in children and adolescents.
Staging includes peritoneal cytology, hysterectomy, bilateral salpingooophorectomy, omentectomy, and exploration of peritoneal surfaces and diaphragm with biopsies. Germ cell tumors israel pdf ppt case reports symptoms. For detailed discussion please refer the articles listed at the end of this page. A gamete is any cell that fuses with another during. The results of earlier medical tests in addition to a physical examination, the following tests may be used to diagnose a germ cell tumor. Germ cell tumors health encyclopedia university of.
The introduction of cisplatinum chemotherapy and current ad vances in the surgical treatment have. Comparison of criteria for assigning germ cell tumor patients to good risk and poor risk studies. The likelihood of a cure can vary with the location of the tumor. Germ cell tumors danafarberboston childrens cancer and. Testicular germ cell tumours alberta health services. Nongerminomatous germ cell tumors secrete chemicals into the spinal fluid and bloodstream and require more intensive treatment than pure germ cell tumors. Pdf current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic.
Feb 05, 2020 extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. A germ cell tumor gct is a neoplasm derived from germ cells. Types of germ cell tumors gct and non germ cell tumors. A stromal cell tumor could also be a cancer that has spread from another part of the body called a secondary cancer. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Childhood extracranial germ cell tumors treatment pdq. Germ cell tumors is a neoplasm which obtained from germ cells. This is commonly characterized by their location on the midline plane of the human body and thus may appear anywhere from the pineal gland to the coccyx. Embryonal carcinoma these cells are malignant and usually are mixed with germ cell tumors. Some germ cell tumors occur in other areas of the body, such as the abdomen, brain and chest, though it. Germ cell brain tumors are tumors, usually cancerous, that arise in the pineal or suprasellar region of the brain.
Chemotherapy when chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body systemic chemotherapy. Birch r, williams s, cone a, einhorn l, roark p, turner s, greco fa. These tumors start and grow mainly in two areas in the center of the brain. Germ cell tumors of the brain danafarberboston childrens. Treating germ cell tumors there are two primary ways to treat germ cell tumors. The most common sites for metastasis are the lungs. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts.
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