Extragonadal germ cell tumors treatment pdqpatient. Extragonadal germ cell tumors account for 15% of all germ cell tumors. Germ cell tumors gcts are masses of tissue formed by the type of immature cells which, in a healthy body, would develop into mature eggs in a female or sperm in a male. Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life. The pathology based on transduodenal needle biopsy demonstrated a yolk sac tumor. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Researchers reported a 25year single institution experience of postchemotherapy surgery for 158 patients with mediastinal primary nonseminoma germ cell tumours gcts. Germ cell tumors can spread metastasize to other parts of the body. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually.
Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant. Germ cell tumors is a neoplasm which obtained from germ cells. Potential strategies for future treatment of malignant extracranial germ cell tumors on the basis of risk stratification from malignant germ cell international collaborative magic pediatric data set and adult germ cell studies. Their imaging characteristics are nonspecific, and extragonadal germ cell tumors should always be included in the differential diagnosis of a midline anterior mediastinal or retroperitoneal mass. Approximately 90% of germ cell tumors arise in the testicles, though extragonadal primary tumors of the retroperitoneum, mediastinum, and pineal gland do occur. Extragonadal gcts typically arise in midline locations, and specific sites vary with age. Young adults can also develop intracranial germ cell tumours but they are again very rare. Extensive changes have been made throughout the document. Intracranial germ cell tumour the royal marsden nhs. Cap extragonadal germ cell tumor protocol revision history version code the definition of version control and an explanation of version codes can be found at.
A germ cell tumor may arise within the gonads in the ovary or testis. Extragonadal germ cell tumor genetic and rare diseases. Of 297 patients with metastatic testicular and extragonadal germ cell tumours gct, bone involvement was detected clinically in 3% 7251 of those at first presentation and in 9% 446 of relapsed cases. Extragonadal germ cell tumors in taiwan request pdf. Retroperitoneal extragonadal germ cell tumor without distant metastasis. They may begin to grow anywhere in the body, but usually form in the pineal gland in the brain, the chest, the lower part of the spine, or the abdomen.
Germ cell tumors may develop in the brain, the testes, the ovaries, or elsewhere in the body. Germ cell tumors can spread to lymph nodes, lung, liver, and brain. We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. Cureus nonseminomatous extragonadal germ cell tumor. Extragonadal germ cell tumorspatient version national.
Male germ cell tumors gcts usually arise from the testis. Extragonadal means outside of the gonads sex organs. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. The likelihood of a cure can vary with the location of the tumor. Recent findings the anterior mediastinal germ cell tumors gcts are the most common egct. Protocol for the examination of resection specimens from. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. We have diagnosed ten primary malignant extragonadal gct arising in the mediastinum, retroperitoneum, liver, and sacrococcygeal region by fine. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Extragonadal germ cell tumors in japan request pdf. Germ cell cancer is a rare tumor that affects children, teens, and adults. Some publications from asia have reported inferior treatment outcomes compared to data from an international study group.
Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Germ cell tumor treatment at danafarberboston childrens. Although yolk sac tumor of the cervix or vagina is the subject of a few case reports, yolk sac tumor of the infant testis is more prevalent and is the most common germ cell tumor arising from this organ 2, 4, 8, 20. Germ cell tumors most commonly appear in the gonads sex organs. If untreated, malignant extragonadal germ cell tumors may spread to the lungs, lymph nodes, bones, liver, or other parts of the body. To ascertain whether this is generally the case, here we analyze treatment outcomes. Testicular germ cell tumors account for approximately 1% of all newly diagnosed male cancers worldwide, and in 2012, there was estimated to be over 10,000. 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. Human germ cell tumors are developmental cancers repub. Extragonadal germ cell tumors occur much more commonly in males than in females 1 and are usually seen in young adults. A person with this syndrome has a higher risk of developing a gonadoblastoma or other germ cell tumors. Pediatric germ cell tumor is the term used to describe malignant cancers of germline cells in patients aged 018 years.
Primary mediastinal germ cell tumor the mediastinum is the most common site of extragonadal germ cell tumors 11, constituting 5070% of all extragonadal germ cell tumors. Germ cell tumors danafarberboston childrens cancer and. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. 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.
The following factor may raise a persons risk of developing an extracranial, extragonadal germ cell tumor. The pathogenesis of these neoplasms is still poorly understood and it is a matter of debate if they really represent extragondal. The treatment of the extragonadal germ cell tumor is based on the stage, type, and location of the tumor. Germ cell tumors are neoplasms that originate from multi potential germ cells and can be intra or extra gonadal. Most patients with a cancerous germ cell tumor will need chemotherapy. Lack, md during the period from 1928 to 1982, 16 children were treated or seen in consultation at the childrens hospital medical center for extragonadal germ cell tumors. Malignant extragonadal germ cell tumors are divided into two types, nonseminoma and seminoma. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. February 2019 includes the childrens oncology group staging system accreditation requirements the use of this protocol is recommended for clinical care purposes but is not required for accreditation purposes. Primary intracranial germ cell tumors are rare icgcts and usually localized in the pineal and suprasellar regions of the brain. A germ cell tumor gct is a neoplasm derived from germ cells. Mediastinal germ cell tumor an overview sciencedirect. For detailed discussion please refer the articles listed at the end of this page. Find evidencebased information on extragonadal germ cell tumors treatment.
Extragonadal germ cell tumors egct represent only 25% of adult germ cell malignancies. These tumors originate in the anterior mediastinum. Tumors of germ cell origin only very uncommonly arise in extragonadal sites. Authors have also contributed articles on the genetics and epigenetics of germ cell tumor development in humans and in the mouse model system. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors mmgcts.
Discuss the embryologic origin and clinical features of germ cell tumors in various locations. Childhood extracranial germ cell tumors treatment pdq. An increasing level in both markers indicates the sign of diagnosing germ cell tumor. The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system. Yolk sac tumor, second in frequency, occurs alone or in combination with a teratoma.
You are advised to consult the publishers version publishers pdf if you wish to cite. Germ cell tumours list of high impact articles ppts. A patient may receive 1 drug at a time or combinations of different drugs given at the same time. Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in the testes or ovaries. There are many kinds of germ cell tumors and only some. Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. Gonadal and extragonadal germ cell neoplasia of childhood.
Background yolk sac tumor endodermal sinus tumor is a rare malignant germ cell tumor arising in the testis or ovary. Extragonadal germ cell tumors form in parts of the body outside the gonads. In children and adolescents, up to 60% of ovarian tumors can be of germ cell origin. Embryonal carcinoma these cells are malignant and usually are mixed with germ cell tumors. They are aggressive neoplasms and can arise virtually anywhere, but typically the site of origin is in the midline mediastinum. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. It can be characterized into cancerous or noncancerous cell. Pubmed abstract mcintyre a, gilbert d, goddard n, et al genes, chromosomes and the development of testicular germ cell tumors of adolescents and adults. 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. It predicts that with standard cisplatinbased chemotherapy, approximately 10% of goodrisk, 25% of intermediaterisk, and more than 50% of poorrisk patients. Summary of changes this is a major revision to the protocol. Primary intracranial germ cell tumors pubmed central pmc. When combined with lifelong monitoring, this can be the only treatment needed for earlystage germ cell tumors that havent spread beyond the testicle. The clinical course is variable, with a potential for aggressive behavior.
In some cases, extragonadal choriocarcinoma occurs in midline locations, such as the mediastinum. The origin of testicular tumors is thought to be atypical cell proliferation, which is termed intratubular germ cell neoplasia, 5, 6. Mediastinal germ cell tumors are usually silent when small and therefore more difficult to treat. Extragonadal yolk sac tumor is even rarer and has only been described in case. The specific characteristics of each tumor will have a major impact on the treatment prescribed and the prognosis. Nonetheless, more than 95% of testicular neoplasms are derived from germ cells arrested in their differentiationthe testicular germ cell tumors. Extragonadal germ cell tumors treatment general information about extragonadal germ cell tumors extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Objective to identify patients with late relapse of metastatic, nonseminomatous germ cell tumour nsgct and to evaluate the patterns of relapse, treatment and outcome, as such relapse at 2 years. Germ cell tumors of the ovary radiology reference article. Extragonadal gcts are rare and do not have a primary testicular involvement. The term germ cell comes from the term geminate, which means giving life. About half of germ cell brain tumors occur in children and young adults between the ages 11 and 30.
Most testicular tumors, in fact, are germ cell tumors. A mature teratoma component is present in the majority of the primary tumors 86%. Patients with extragonadal germ cell tumors version. Germ cell tumors also may arise in extragonadal sites, reflecting the fact that germ cells travel to diverse areas of the body, such as the chest, abdomen, and brain. Germ cells normally occur inside the gonads ovary and testis. The initial theory regarding the genesis of these tumors was that they represented metastasis from an occult. Retroperitoneal extragonadal germ cell tumor without. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Surgical removal of the tumor if it is benign teratoma. Bone disease in testicular and extragonadal germ cell tumours. The tumor marker test includes two markers, afp and. Germ cell tumors are growths that occur in children, teens and adults. It is a slowgrowing tumor that typically occurs in young patients and is. The most widely accepted theory suggests that extragonadal germ cell tumors arise from primordial germ cells misplaced during their migration to gonads.
Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum 50 to 70% of all extragonadal germ cell tumors. Germ cell tumors are found widely throughout the body, and encompass a wide range of individual tumors. More than half of mediastinal germ cell tumors are mature teratomas. We strongly recommend that you talk with a trusted. Gonadal and extragonadal germ cell tumors in the united.
Totipotent stem cells in an undifferentiated state give rise to embryonal carcinomas. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. The degree and direction of the differentiation determine the histologic subtype of the gcts, fig 2. Germ cell tumors arise predominantly from within the testis, but an important subset of germ cell tumors are extragonadal in origin. Pediatric and adolescent extracranial germ cell tumors. During the period from 1928 to 1982, 16 children were treated or seen in consultation at the childrens hospital medical center for extragonadal germ cell tumors of the head and neck region. In humans, germ cells produce specialized cells needed for reproduction. University of groningen testicular germ cell tumors gels. Current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic. Handbook for children with germ cell tumors american pediatric. However, the true incidence of these tumors may be higher than originally thought because of failure to diagnose them properly.
Adverse effects of chemotherapy, including gastrointestinal, hepatobiliary, genitourinary, and hematologic toxicity. This article does not deal with any specific body locations. This page is focused on extracranial outside of the brain germ cell tumors. Another type of germ cell tumour which can be either benign or malignant is known as a teratoma. Germ cell tumors are rare, about 900 children and adolescents are diagnosed in the united states each year. They are usually found along the midline of the body and are most common in the mediastinum, thymus, retroperitoneal organs, and pineal gland. Extragonadal germ cell tumours clinical practice guidelines. Germ cell tumors of the gonads have a better prognosis than germ cell tumors outside the gonads extragonadal. Extragonadal germ cell tumors eggcts are uncommon neoplasms, which arise in anatomical locations other than gonads. In males, choriocarcinoma is generally considered a nonseminomatous germ cell tumor, which represents less than 5% of all germ cell tumors in males. There are several types of both benign and malignant germ cell tumors, including the following. Afp iuml or ngml is a protein which a very high concentration of afp may lead to germ cell tumor.
The drugs that are commonly used for treating germ cell tumors include bleomycin blenoxane, cisplatin platinol, and etoposide vepesid, toposar. Extragonadal germ cell tumors clinical presentation. They make up only 4% of all cancers in children and adolescents. It is intended as a resource to inform and assist clinicians who care for cancer patients. Review of 16 cases management of extragonadal germ cell tumors and the significance of bilateral testicular biopsies 911 treatment and outcome of patients with extragonadal germ cell tumors. From head to toe1 learning objectives for test 2 after reading this article and taking the test, the reader will be able to. Extragonadal germ cell tumors library navigating care. Germ cell tumors of the brain are a type of extragonadal germ cell tumor, but are treated differently than germ cell tumors in other parts of the body.
Extragonadal germ cell tumors of the head and neck region. Germ cell tumors health encyclopedia university of. For earlystage testicular cancer we usually recommend radical orchiectomy, a surgery in which we remove the tumor along with the testicle. Germ cells tumors of the ovary account for approximately 1520% of all ovarian tumors. Germ cell tumors commonly occur in the gonads, but the anterosuperior mediastinum is the most common site of extragonadal germ cell tumors in adults. Germ cell brain tumors are very rare, accounting for less than 5 percent of all brain tumors in children. The development of the international germ cell cancer collaboration group classification of risk in 1997 7 improved our ability to estimate the risk of failure to 1 stline chemotherapy. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. The full text of this article is available in pdf format. Due to old age, the potential toxicity of systemic chemotherapy, and no significant signs of invasion to adjacent organs, we performed surgical resection. Sep 10, 2018 growing teratoma syndrome is the increase in tumor size during or after chemotherapy for mediastinal germ cell tumors mgct or retroperitoneal germ cell tumors rgct in which histologic analysis of the resected tumor specimen shows only mature teratoma. Klinefelter syndrome 47 xxy is the only known predisposing factor.
Treatment of extragonadal germ cell tumors that are recurrent come back after being treated or refractory do not get better during treatment may include the following. Overall, approximately 5% to 10% of all germ cell cancers arise in nongonadal sites, particularly in the mediastinum and retroperitoneum. The aim of this study was to examine sex and racespecific trends in incidence and survival of gonadal ggcts and extragonadal gcts egcts in the us from 1973 to 2007. Nonseminomas tend to grow and spread more quickly than seminomas. Germ cell tumors gcts most often arise in the gonads but some develop extragonadally.
When cells that are meant to form sperm in the testicles or eggs in the ovaries travel to other parts of the body, they may grow into extragonadal germ cell tumors. Recurrent or refractory extragonadal germ cell tumors. Describe the differences in pathologic and radiologic features between seminomas and nonseminomatous. While most extragonadal tumors are more challenging to treat, in general, germ cell tumors have an excellent overall prognosis with 5year survival.
Extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. Extragonadal germ cell tumorshealth professional version. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. Male extragonadal germ cell tumors egcts are characterized by a malignant transformation of germ cells without the presence of a gonadal primary tumor. Fewer than ten children a year develop an intracranial germ cell tumour in the uk. Germ cell tumors gct are neoplasms that originate predominately in the ovary and testis. Testicular germ cell tumor tgct is the most common tumor which usually occurs in males between 2040 years old 1, 2.
They usually are large and cause signs and symptoms. 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. The germ cells which are present outside the gonads are the birth defects during the development of embryo. You will learn about how doctors describe a tumors growth or spread. May 28, 2018 extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided.
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