Patients with germcell tumours are managed by a diverse array of specialists. Germ cell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. Extragonadal germ cell tumors can be benign noncancer or malignant. Egct usually occur in males between the ages of 20 and 35 years, but can occur at any age. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby.
Thus, staging, risk stratification, and treatment approaches for germcell. Treatment options for extragonadal germ cell tumors. Overall, approximately 5% to 10% of all germ cell cancers arise in nongonadal sites, particularly in the mediastinum and retroperitoneum. Extragonadal germ cell tumors hollandfrei cancer medicine. Extragonadal germ cell tumors have a variable clinical course, with the potential for aggressive behavior and widespread metastases. Pdf extragonadal germ cell tumor coexisted with renal. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Yolk sac tumor yst, endodermal sinus tumors is a rare germ cell neoplasm occurring primarily in the gonads. The latter group can be divided into seminoma and nonseminoma germ cell tumors, which include the following. Patients with germ cell tumours are managed by a diverse array of specialists. Primary or secondary extragonadal germ cell tumors. A germ cell tumor is a neoplasm that is comprised of germ cells found in the ovaries and testes.
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. Extragonadal gcts typical it seems to us that you have your javascript disabled on your browser. Malignant extragonadal germ cell tumors are divided into two types, nonseminoma and seminoma. Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in the testes or ovaries. Germ cell tumors occur predominantly from the testicles. Primary extragonadal germ cell tumors egcts are characterized by their location on the midline structures of the body from the pineal gland to. Extragonadal means outside of the gonads sex organs. This includes the pineal gland in the brain, the mediastinum area between the lungs, and retroperitoneum the back wall of the abdomen. Management of paediatric extracranial germ cell tumours carries a unique set of challenges. Management of paediatric extracranial germcell tumours carries a unique set of challenges. Extragonadal yolk sac tumor is even rarer and has only been described in case. Extragonadal germ cell tumors are the same type of tumors, just located somewhere outside of the ovaries and testis. Extragonadal germ cell tumor presenting in a woman with.
Extragonadal localization of germ cell tumors gcts is rare. The world health organization 2016 classification of. May 28, 2018 extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. Branching tubules lined by cylindrical epithelium with prominent vacuolation displaying morular aggregates with cells exhibiting optically clear nuclei 11. This pdq cancer information summary has current information about the treatment of extragonadal germ cell tumors. 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. They originate from cells that were meant to form sex cells i. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. 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. This case report describes a 28yearold malaysian malay woman with lupus nephritis who complained of headache and blurring of vision. Germ cell tumors gcts are classified as extragonadal. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. 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.
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. Extragonadal germ cell tumor genetic and rare diseases. Extragonadal germ cell tumors can take on a variety of tumor types. Active surveillance may help doctors to monitor subjects with low risk germ cell tumors after their tumor is removed.
The initial theory regarding the genesis of these tumors was that they represented metastasis from an occult gonadal primary. Gastrointestinal stromal tumor gist germ cell tumor. Extragonadal germ cell tumors occur much more commonly in. It does not give formal guidelines or recommendations for making decisions about health care. Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in either the testes or ovaries. When cells that are meant to form sperm in the testicles or eggs in the ovaries. Mediastinal tumors a general article on all mediastinal tumors, not just extragonadal germ cell tumors. Paediatric extracranial germcell tumours the lancet.
Surgical removal of the tumor if it is benign teratoma. Extragonadal germ cell tumors occur much more commonly in males than in females 1 and are usually seen in young adults. Start here to find information on extragonadal germ cell tumors treatment. Treatment of nonseminoma extragonadal germ cell tumors may include the following. Extragonadal germ cell tumors treatment pdqpatient version. If a larger tumor remains after treatment, surgery or watchful waiting follow. May 19, 2012 extragonadal localization of germ cell tumors gcts is rare.
Benign extragonadal germ cell tumors are called benign teratomas. Treatment clinical trials for extragonadal germ cell tumor. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Germcell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. Extragonadal germ cell tumorspatient version national. Extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. 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. Cap extragonadal germ cell tumor protocol revision history. Residues compatible with an occult testicular primary, overlooked at the pretreatment examination, were found in 10 of 12 patients with retroperitoneal germ cell tumors, whereas the testes in all 4 patients with mediastinal germ cell tumors showed no. There are many kinds of germ cell tumors and only some. Extragonadal germ cell tumors can be benign noncancer or malignant cancer. Extragonadal germ cell tumors form in parts of the body outside the gonads.
Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. 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. Although their occurrence in the sinonasal tract is exceedingly rare, a number of histologic subtypes have been recognized. 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. 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 mixed germ cell tumor of the right arm. Extragonadal tumors arise when these cells become cancerous. A valu able imaging sign is that a primary extragonadal. Combination chemotherapy followed by surgery to remove any remaining tumor. Extragonadal germ cell tumor home conditions extragonadal germ cell tumor. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. In some cases, however, an apparent extragonadal tumor turns out to be a metastatic testicular tumor where the primary tumor has burnt out or died on its own.
Extragonadal gcts typically arise in it seems to us that you have your javascript disabled on your browser. In males, choriocarcinoma is generally considered a nonseminomatous germ cell tumor, which represents less than 5% of all germ cell tumors in males. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant. They are found in people of all ages, but are primarily seen in young males. Extragonadal germ cell tumor symptoms, diagnosis and. Extragonadal germ cell tumors differential diagnoses. Get a printable copy pdf file of the complete article 1. Extragonadal germ cell tumors treatment pdq pubmed health. Case report computed tomography imaging features of primary. Extragonadal yolk sac tumor in pelvic localization. Extragonadal germ cell tumor eggct represents 510% of germ cell tumors and frequently occurs in mediastinum.
Germ cells normally occur inside the gonads ovary and testis. Extragonadal germ cell tumors definition germ cells are primitive cells within the body that normally mature into ova egg or sperm cells. In some cases, extragonadal choriocarcinoma occurs in midline locations, such as the mediastinum. Extragonadal germ cell tumors in taiwan request pdf. Germ cell tumors are a varied group of benign and malignant neoplasms derived from primordial germ cells 110, 1215 table 2. They are aggressive neoplasms and can arise virtually anywhere, but typically the site of origin is in the midline mediastinum, retroperitoneum, or pineal gland. After initial treatment for extragonadal germ cell tumors, blood levels of afp and other tumor markers continue to be checked to find out how well the treatment is working. 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.
He presented with a painless mass in right inguinal region, a few. Pdf extragonadal germ cell tumor coexisted with renal cell. The imaging characteristics of these tumors are nonspecific, and the definitive diagnosis of extragonadal germ cell tumor requires a biopsy. Extragonadal germ cell tumors treatment pdq patients. Paediatric extracranial germcell tumours the lancet oncology. Germ cell tumors of miscellaneous extragonadal sites. However, 25% of all germ cell tumors in adult men have extargonadal localization.
Enlarge extragonadal germ cell tumors form in parts of the body other than the gonads testicles or ovaries. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell. We strongly recommend that you talk with a trusted. 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. Incidence and mortality extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. However, the true incidence of these tumors may conceivably be higher than originally thought because of failure to diagnose them properly. Germ cell tumors gcts may arise in the testis, the ovary, or a variety of midline extragonadal sites. These are more common than malignant extragonadal germ cell tumors and often are very large. The treatment of the extragonadal germ cell tumor is based on the stage, type, and location of the tumor. Germ cell tumors arise predominantly from within the testis, but an important subset of germ cell tumors are extragonadal in origin. Familial aggregations of tgct have been well described, suggesting the existence of a hereditary tgct subset.
There have been several published reports of ovarian germ cell cancers affecting more than one family member. Treatment and outcome of patients with extragonadal germ cell tumoursthe. If a tumor smaller than 3 centimeters remains after chemotherapy, watchful waiting follows. Drugs used in chemotherapy, such as bleomycin, carboplatin, etoposide, and cisplatin, work in different ways to stop the growth of tumor cells, either by killing the cells, by stopping them from dividing, or by stopping them from spreading. Nonseminomatous germ cell tumor genetic and rare diseases. We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype.
Ct features of primary extragonadal seminoma 12652 int j clin exp med 2017. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for extragonadal germ cell tumor. Thus, staging, risk stratification, and treatment approaches for germ cell. Testicular germ cell tumor tgct is the most common malignancy in males aging 1535. Chronic myelogenous leukemia chronic myeloproliferative disorders colon cancer cutaneous tcell lymphoma d desmoplastic small round cell tumor e endometrial cancer ependymoma esophageal cancer ewings sarcoma in the ewing family of tumors extracranial germ cell tumor, childhood extragonadal germ cell tumor extrahepatic bile duct cancer. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. A germ cell tumor gct is a neoplasm derived from germ cells. Although it is the second most common malignant germ cell tumor of the ovary, extragonadal ysts represent an exceedingly rare malignancy in women. The latter include the central nervous system, mediastinum, retroperitoneum, and sacral region. Background yolk sac tumor endodermal sinus tumor is a rare malignant germ cell tumor arising in the testis or ovary.
In adults, the most common sites, in order of frequency, are the anterior mediastinum, retroperitoneum, and the pineal and suprasellar regions. Patients with extragonadal germ cell tumors version. In these cases, the mediastinal germ cell tumor develops before or concomitantly with but not after acute megakaryoblastic leukemia. Extragonadal germ cell tumors egct, which are defined as germ cell tumors without a primary tumor in the testes are very rare. Extragonadal germ cell tumor cancer information ohc. Retroperitoneal extragonadal germ cell tumors springerlink. Pdf we report a rare case of extragonadal germ cell tumour in a 55yearold man.
Protocol for the examination of resection specimens from. Related summaries ovarian germ cell tumors treatment. Extragonadal germ cell tumor coexisted with renal cell carcinoma article pdf available in nippon hinyokika gakkai zasshi. Surgery for extragonadal germ cell tumor of mediastinum. To characterize the clinical and biologic features of extragonadal germ cell tumor egct and to determine the overall outcome. Should extragonadal germ cell tumors be included in. 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.
Extragonadal germ cell tumors can be benign teratoma or malignant. Germ cell tumor of the pituitary gland is a very rare occurrence. Testicular cancer resource centers extragonadal germ cell. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. Case report computed tomography imaging features of. She was later found to have a pituitary germ cell tumor. Extragonadal germ cell tumors treatment pdqpatient. Pituitary germ cell tumor is considered as a type of extragonadal germ cell tumor. Treatment and outcome of patients with extragonadal germ cell. The remaining 510% of germ cell tumors arise outside of the gonads. Extragonadal germ cell tumors of the mediastinum and. Handbook for children with germ cell tumors american pediatric.
It is meant to inform and help patients, families, and caregivers. 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 gcts are classified as extragonadal if there is no evidence of a primary tumor in either the testes or the ovaries. Feb 20, 2015 germ cell tumors occur predominantly from the testicles. They are often large and have a tendency to spread more quickly than the other type of germ cell tumor i. Extragonadal gcts typically arise in midline locations, and specific sites vary with age.
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