Extragonadal means outside of the gonads sex organs. Germ cells are responsible for producing eggs in women and sperm in men. Extragonadal germ cell tumor cancer information ohc. 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. Start here to find information on extragonadal germ cell tumors treatment. These symptoms include fever, constipation, abnormal bleeding in vagina and miss menstruation in females, a lump in the testes in males, lumps along the midline of the body, include coccyx, neck, and abdomen. For detailed discussion please refer the articles listed at the end of this page. Patients and methods of an unselected population of 635 consecutive patients treated from 1975 through 1996 at 11 cancer centers, 341 patients 54% had primary. 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. The metastatic tumor is the same type of tumor as the primary tumor. Extragonadal germ cell tumors missouri cancer associates. A treatment clinical trial is a research study meant to help improve current treatments or obtain information. Metastatic urinary bladder tumor from extragonadal germ cell.
You can also get a germ cell tumor in the brain, chest, belly. Patients with ks also have a higher risk of developing malignant mediastinal germ cell tumors, and at least 8% of male patients with primary mediastinal tumors have ks. Extragonadal germ cell tumors have a variable clinical course, with the potential for aggressive behavior and widespread metastases. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Blood tests, physical exams and scans of the abdomen, chest and pelvis, are used to provide doctors with more information about the tumor. Get detailed information about the treatment of newly diagnosed and recurrent extragonadal gcts in this summary for clinicians. An extracranial germcell tumor egct occurs in the abnormal growth of germ cells in the gonads testes or ovaries and the areas other than the brain via tissue, lymphatic system, or circulatory system. There are different types of treatment for patients with extragonadal germ cell tumors. The initial theory regarding the genesis of these tumors was that they represented metastasis from an occult gonadal primary. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. This hypothesis explains the anatomical distribution of extragonadal gcts as a result of the migration of primor dial germ cells pgcs from the yolk sac, i.
Microscopically, the tumor was composed of seminoma. Incidence and mortality extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. The treatment of the extragonadal germ cell tumor is based on the stage, type, and location of the tumor. May 19, 2012 extragonadal localization of germ cell tumors gcts is rare. Imaging and blood tests are used to detect find and. They do know that tumors form when germ cells, which eventually become sperm in males and eggs in females, exit the gonads reproductive glands and grow out of control and clump together in another area of the body. Some treatments are standard the currently used treatment, and some are being tested in clinical trials. We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. For example, if an extragonadal germ cell tumor spreads to the lung, the tumor cells in the lung are actually cancerous germ cells.
Related summaries ovarian germ cell tumors treatment. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Germcell tumours arise from molecular defects in early germline progenitors known as primordial germ cells. They occur in a variety of sites, both gonadal and extragonadal, the latter in midline locations as the sacrococcygeal, retroperitoneal, mediastinal, cervical, and pineal regions tables 2.
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. However, the true incidence of these tumors may conceivably be higher than originally thought because of failure to diagnose them properly. The disease is metastatic extragonadal germ cell tumor, not lung cancer. One of these cases had complete tumor necrosis caused by chemotherapeutic effects, and the second case could not be confirmed histologically. A clinical trial of highdose chemotherapy with stem cell transplant. 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.
Extragonadal germ cell tumors treatment pdqpatient. Sep 10, 2018 retroperitoneal germ cell tumor diagnosed by endoscopic ultrasoundguided fine needle aspiration. Surgical removal of the tumor if it is benign teratoma. Germ cell tumors tend to respond to treatment and many can be cured, even when diagnosed at a late stage. Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. Extragonadal germ cell tumors can take on a variety of tumor types. Although germ cell tumors rarely turn into cancer, when they do, they can cause ovarian cancer and travel throughout the body to form other types of cancers.
Germ cell tumors that occur in places other than the testicles and ovaries extragonadal germ cell tumors are very rare. Extragonadal germ cell tumors egct represent only 25% of adult germ cell malignancies. Retroperitoneal germ cell tumor diagnosed by endoscopic ultrasoundguided fine needle aspiration. Extragonadal germ cell tumors treatment pdqpatient version.
Researchers have not yet determined what causes extragonadal germ cell tumors. While mixed germ cell tumor mgct is the second most common germ cell tumor, after seminoma, and occurs in gonads of young men, extragonadal mgct is rare, involves midline from pineal gland to coccyx, and occurs in different age groups. There are signs and symptoms of extracranial germ cell tumor that can be seen in children, adolescents, or young adults. Extragonadal germ cell tumors gct treatment depends on the type and can include surgery, radiation, chemotherapy, and stem cell transplant. Testicular cancer resource centers extragonadal germ cell. Why human extragonadal germ cell tumours occur in the midline of.
Blood tests, physical exams and scans of the abdomen, chest and pelvis, are used to. The imaging characteristics of these tumors are nonspecific, and the definitive diagnosis of extragonadal germ cell tumor requires a biopsy. Cytologic features of extragonadal germ cell tumors. Origin of adult male mediastinal germ cell tumours. Sometimes, a group of germ cells grows in a way thats not normal. Extragonadal germ cell tumors occur much more commonly in. Extragonadal germ cell tumors library navigating care. Primary retroperitoneal germ cell tumors are more commonly associated with an occult testicular primary site, especially when the tumor is not midline in origin. Primary extragonadal germ cell tumors egcts are rare, accounting for % of all gcts, of which 60% are seminomas. When the cells move to other parts of the body and form tumors, it is called extragonadal germ cell tumor. Among these, a common form is teratoma with endodermal sinus tumor. Extragonadal germ cell tumor symptoms, diagnosis and. Different types of treatments are available for patients with extragonadal germ cell tumors. Stages of extragonadal germ cell tumors navigating care.
Extragonadal germ cell tumors egct, which are defined as germ cell tumors without a primary tumor in the testes are very rare. Because they are rare and biologically distinct from testis cancer, their natural history and optimal. 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. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. Extragonadal germ cell tumorspatient version national. We report the molecular cytogenetic studies performed in 4 young males with mediastinal germ cell tumors. Mediastinal tumors a general article on all mediastinal tumors, not just extragonadal germ cell tumors. The most common germ cell tumors occurring in the perinatal period in order of rank are teratoma, yolk sac tumor, choriocarcinoma, and gonadoblastoma. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. The incidence of this syndrome among patients with mediastinal germ cell neoplasms is. This case summary is recommended for reporting extragonadal germ cell tumors, but is not required for accreditation purposes.
Protocol for the examination of specimens from patients. The recommendations contained in this guideline are a consensus of the alberta. Extragonadal germ cell tumors treatment pdq patients. The diagnosis of extragonadal germ cell tumor was made based on a lymph node biopsy. Cap approved pediatric extragonadal germ cell tumor germcell 3. Since this is a rare type of tumor they must also differentiate it from other types of tumors. We report here the case history of a patient with an advanced undifferentiated pulmonary tumor, the germ cell origin of which was not initially recognized because of its unusual location. Klinefelter syndrome has been associated with other malignancies such as extragonadal germ cell tumors. When cells that are meant to form sperm in the testicles or eggs in the ovaries travel. Following chemotherapy and retroperitoneal lymphoadenectomy, at outpatient followup.
Rnabinding protein lin28 is a marker for primary extragonadal germ cell tumors. Extragonadal mixed germ cell tumor of the right arm. In the evaluation of an extragonadal germ cell tumor, a histological examination should be included since ultrasonography is not sufficient to detect cis or minute lesions of the testis. Germ cell tumor, unclassified 2 cases in 2 cases, a cytologic diagnosis of gct was made. May 28, 2018 extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. 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. Jude childrens research hospital, the chance of children who are under. How does an extragonadal germ cell tumor affect the body. Extragonadal germ cell tumors hollandfrei cancer medicine. Purpose to characterize the clinical and biologic features of extragonadal germ cell tumor egct and to determine the overall outcome with currently available treatment strategies. Testicular seminoma after the complete remission of. From the cleveland clinic teratomas and other germ cell tumors of the mediastinum an in depth article from medscape primary germ cell tumors of the thorax this is basically a library of information from the armenian medical network. Overall, approximately 5% to 10% of all germ cell cancers arise in nongonadal sites, particularly in the mediastinum and retroperitoneum. Germ cells are primitive cells within the body that normally mature into ova egg or sperm cells.
Extragonadal germ cell tumor genetic and rare diseases. Klinefelter syndrome ks is a well documented abnormality of sex differentiation occurring in 1 in 600 new born males. Klinefelter syndrome 47 xxy is the only known predisposing factor. Paediatric extracranial germcell tumours sciencedirect. The possibility of curative treatment was therefore. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Extragonadal germ cell tumors are often associated with. Malignant extragonadal germ cell tumors may cause signs and symptoms as they grow into nearby areas. 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. Signs and symptoms of extragonadal germ cell tumors include breathing problems and chest pain. We report the case of a 37yearold man who presented with a primary malignant mixed nonseminomatous gct teratocarcinoma variety in the right arm, treated by a combination of cisplatinbased chemotherapy. Extragonadal malignant germ cell tumor of the lung the. Primary extragonadal germ cell tumors are rare, and 60% of such cases are seminomas.
We herein report a case of metachronous occurrence of an extragonadal and gonadal germ cell tumor. The following prognostic groups are used for extragonadal germ cell tumors. Germ cell tumors are a varied group of benign and malignant neoplasms derived from primordial germ cells 110, 1215 table 2. 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. The latter group can be divided into seminoma and nonseminoma germ cell tumors, which include the following. Extragonadal germ cell tumors represent 25% of all germ cell tumor 16 with an incidence around 11,000,000 population 17.
Bgc occur in adults as well as children, with the age ranging from. Germ cell tumors arise predominantly from within the testis, but an important subset of germ cell tumors are extragonadal in origin. The most common site of extragonadal germ cell tumors is the mediastinum 50 70% followed by the retroperitoneum 3040%, the pineal. Feb 05, 2020 extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. The pluripotency gene lin28 is expressed in malignant germcell tumours across all age groups and histologies. The clinical course is variable, with a potential for aggressive behavior and widespread. 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 composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Extragonadal germ cell tumors treatment pdq pubmed health. Most extragonadal mgcts are metastases from primary testicular germ cell tumors. Extragonadal germ cell tumors form in parts of the body outside the gonads.
Extragonadal mixed germ cell tumor arising in adrenal. The hypothetical model of tumorigenesis proposed by teilum 5 postulates that germinomas seminomas in testicular sites and dysgerminomas in ovarian sites arise. The typical clinical feature of seminoma is a progressive increase in testicular size. There are many tumor markers that doctors can use to diagnose an extragonadal germ cell tumor. 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. Risk factors for malignant extragonadal germ cell tumors include the following. He complained of gross hematuria, cystoscopy showed a bladder tumor, and abdominal mri scan showed a right ureteral tumor. Cao d, liu a, wang f, allan rw, mei k, peng y, et al. The tumor can be benign or malignant cancerous by its growth rate. A germ cell tumor gct is a neoplasm derived from germ cells.
Also male patients with carcinoma of unknown primary cup syndrome with undifferentiated hist ology and typical germ cell tumor serum markers, as well as. Egct usually occur in males between the ages of 20 and 35 years, but can occur at any age. There is now general acceptance that extragonadal germ cell tumors represent malignant transformation of germinal elements distributed to these sites without a testicular focus. Age and gender can affect the risk of extragonadal germ cell tumors. Nonproteincoding rnas represent another promising area of investigation in germcell tumours. The broad range of possible germcell tumour histologies is attributed to the totipotent nature of primordial germ cell. Primary mediastinal germ cell tumor with intratubular germ cell neoplasia of the testisfurther support for germ cell origin of these tumors. Of the 8000 annual cases of gct, only 25% are of extragonadal origin. Although malignant extragonadal germ cell tumors of the lung are extremely rare, their recognition is of clinical importance because of their curability. Extracranial, extragonadal germ cell tumors are rare.
We strongly recommend that you talk with a trusted. Extracranial germ cell tumors, including those that occur in the reproductive organs, account for about 3% of all tumors in children younger than 15 and about 14% in adolescents age 15 to 19. Germ cells normally occur inside the gonads ovary and testis. Extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. Malignant tumors can be either seminoma aka germinoma in females or nonseminoma embryonal carcinoma, immature teratoma, endodermal sinus tumor aka yolk sac tumor, choriocarcinoma, and mixed germ cell tumors. Extragonadal germ cell tumors account for 15% of all germ cell tumors. Although their occurrence in the sinonasal tract is exceedingly rare, a number of histologic subtypes have been recognized. Case report extragonadal germ cell tumor in an older patient, not every mass is a lung cancer arnaoutakis, k. Pdf on feb 1, 2002, hj schmoll and others published extragonadal germ cell tumors find, read and cite all the research you need on researchgate. Some germ cell tumors occur in other areas of the body, such as the abdomen, brain and chest, though its not clear why. 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. Seminomas are common germ cell tumors, with a good prognosis and a mortality rate of 0.
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