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. Extragonadal germ cell tumors gct treatment depends on the type and can include surgery, radiation, chemotherapy, and stem cell transplant. Nonseminomatous germ cell tu mors nsgcts include teratoma, embry onal carcinoma, endodermal sinus tumor. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. Klinefelter syndrome with poor risk extragonadal germ cell. Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. The remaining 510% of germ cell tumors arise outside of the gonads. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. Germ cells normally occur inside the gonads ovary and testis.
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 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 tumors account for 15% of all germ cell tumors. Extragonadal germ cell tumorspatient version national.
Extragonadal mixed germ cell tumor arising in adrenal gland. The gcts exhibit various pathologic features according to the totipotentiality of the tumor. Start here to find information on extragonadal germ cell tumors treatment. 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. Bilateral testes were normal by ultrasonography and physical examination, but the serum afp level was increased to 1161 ngml. Germ cell tumors includes pediatric and adult patients with germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, and neck the following should not be reported using this protocol. Bone disease in testicular and extragonadal germ cell tumours. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. 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. Noncentral nervous system extragonadal gcts are found in a variety of anatomic locations, but most commonly affect the mediastinum and. The extent or spread of cancer is usually described as stages. Primary extragonadal germ cell tumors egcts are characterized by their location on the midline structures of the body from the pineal gland to.
Extragonadal germ cell tumor of the prostate associated. The objective of the current study was to evaluate and describe the cytologic features of extragonadal germ cell tumors gcts, both primary and metastasis from gonadal sites, in fineneedle aspiration cytology. Response rates and survival are similar to patients with advancedstage testicular germcell tumors. Extragonadal germ cell tumors annals of oncology oxford. It is intended as a resource to inform and assist clinicians who care for cancer patients. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Protocol for the examination of specimens from pediatric. Thus, staging, risk stratification, and treatment approaches for germcell. Germ cell tumors gcts affect not only the gonads but also extragonadal tissue. Protocol for the examination of resection specimens from.
Pdf cytologic features of extragonadal germ cell tumors. Patient age the behavior of pediatric and adult extragonadal germ cell tumors egcts is quite distinct. Sep 06, 2017 extragonadal germ cell tumours are rare and comprise a heterogeneous group of neoplasms with distinct natural history and responses to different treatment modalities. Patients with germcell tumours are managed by a diverse array of specialists. Find evidencebased information on extragonadal germ cell tumors treatment. The phenomenon of burnout in an extragonadal germ cell tumor is characterizd by regression of testicular tumor. Extragonadal means outside of the gonads sex organs. Overall, approximately 5% to 10% of all germ cell cancers arise in nongonadal sites, particularly in the mediastinum and retroperitoneum. Germ cell tumors can spread metastasize to other parts of the body.
Cap approved pediatric extragonadal germ cell tumor germcell 3. Prognostic factors in children with extragonadal malignant germ cell tumors. While the tumors can occur in the mediastinum, thymus, retroperitoneal organs and pineal gland, seminoma originating in the prostate tissue is extremely rare. While most extragonadal tumors are more challenging to treat, in general, germ cell tumors have an excellent overall prognosis with 5year survival. Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in the testes or ovaries. Retroperitoneal extragonadal germ cell tumor without distant. 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. Germ cell tumors commonly occur in the gonads, but the anterosuperior mediastinum is the most common site of extragonadal germ cell tumors in adults. The exact histogenesis has not been elucidated, but is. A germcell tumor gct is a neoplasm derived from germ cells. Extragonadal germ cell tumors clinical presentation. Extragonadal gcts typically arise in midline locations, and specific sites vary with age. A nonseminoma extragonadal germ cell tumor is in the intermediate prognosis group if. Germcell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour.
The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. We strongly recommend that you talk with a trusted. Childhood extracranial germ cell tumors treatment pdq. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. The imaging characteristics of these tumors are nonspecific, and the definitive diagnosis of extragonadal germ cell tumor requires a biopsy. From head to toe1 learning objectives for test 2 after reading this article and taking the test, the reader will be able to. Retroperitoneal extragonadal germ cell tumor without distant metastasis. Gonadal gcts include testicular gcts in males and ovarian gcts in females. Surgery for extragonadal germ cell tumor of mediastinum. As outlined below, within the pediatric age range, prognosis is worse with increasing age.
Discuss the embryologic origin and clinical features of germ cell tumors in various locations. Germ cell tumors occur predominantly from the testicles. 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. Germ cell tumors are the most common malignancy in the testicle, and the most common malignancy in males 1535 years old. Primary extragonadal germ cell tumors are rare, and 60% of such cases are seminomas. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. There are many kinds of germ cell tumors and only some.
Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life. Cureus nonseminomatous extragonadal germ cell tumor. Although malignant extragonadal germ cell tumors of the lung are extremely rare, their recognition is of clinical importance because of their curability. Because there was no tumor except from the prostate, we considered this case to be a primary extragonadal germ cell tumor of the prostate. Extragonadal germ cell tumors can take on a variety of tumor types. Retroperitoneal extragonadal germ cell tumor without. Summary of changes this is a major revision to the protocol.
Germ cell tumors health encyclopedia university of. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Most extragonadal mgcts are metastases from primary testicular germ cell tumors. However, neither of these two series explain the striking pre. The first classification organized the egcts into two types, gonadal and extragonadal germcell tumor. Extragonadal germ cell tumors form in parts of the body outside the gonads.
For extragonadal germ cell tumors, prognostic groups are used instead of stages. 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. 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. The most widely accepted theory suggests that extragonadal germ cell tumors arise from primordial germ cells misplaced during their migration to gonads. They are aggressive neoplasms and can arise virtually anywhere, but typically the site of origin is in the midline mediastinum. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males.
Procedure biopsy consider extragonadal germ cell biopsy protocol tumor type testicular germ cell tumors consider the testis protocol. Extragonadal germ cell tumors have a variable clinical course, with the potential for aggressive behavior and widespread metastases. Pdf extragonadal germ cell tumors in taiwan tsuyi chao. A pediatric intergroup study neyssa marina, wendy b. Get detailed information about the treatment of newly diagnosed and recurrent extragonadal gcts in this summary for clinicians. After an extragonadal germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. 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.
Table 3 most frequent presenting symptoms by site of disease site of disease no. Extragonadal germ cell tumor genetic and rare diseases. However, the true incidence of these tumors may be higher than originally thought because of failure to diagnose them properly. There is no american joint committee on cancer ajccinternational union. Cap extragonadal germ cell tumor protocol revision history. Male germ cell tumors gcts usually arise from the testis. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Prognostic factors in children with extragonadal malignant. It does not give formal guidelines or recommendations for making decisions about health care.
A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for extragonadal germ cell tumor. Mediastinal germ cell tumors are a heterogeneous group of benign and malignant neoplasms that originate from primitive germ cells left in the mediastinum during early embryogenesis. 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. It is meant to inform and help patients, families, and caregivers. Background documentation pediatric extragonadal germ cell tumor 4. Surgical removal of the tumor if it is benign teratoma.
Mediastinum n 27 chest pain 12 shortness of breath 15 cough 12. In some cases, extragonadal choriocarcinoma occurs in midline locations, such as the mediastinum. Extragonadal malignant germ cell tumor of the lung the. Extragonadal germ cell tumorshealth professional version. 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 pathology based on transduodenal needle biopsy demonstrated a yolk sac tumor. Mediastinal germ cell tumor an overview sciencedirect. Also male patients with carcinoma of unknown primary cup syndrome with undifferentiated hist ology and typical germ cell tumor serum markers, as well as. We report a rare case of extragonadal germ cell tumour in a 55yearold man. Extracranial extragonadal gcts are tumor cells that had been spread out through tissue, lymph system or blood to other areas of the body other than the gonads.
The first classification organized the egcts into two types, gonadal and extragonadal germ cell tumor. Extragonadal germ cell tumors hollandfrei cancer medicine. This pdq cancer information summary has current information about the treatment of extragonadal germ cell tumors. The possibility of curative treatment was therefore. Describe the differences in pathologic and radiologic features between seminomas and nonseminomatous.
Extragonadal germ cell tumors treatment pdq pubmed health. What are the appropriate management strategies for patients with primary mediastinal or retroperitoneal germ cell tumours. Extragonadal germ cell tumors definition germ cells are primitive cells within the body that normally mature into ova egg or sperm cells. Approximately 90% of germ cell tumors arise in the testicles, though extragonadal primary tumors of the retroperitoneum, mediastinum, and pineal gland do occur. Researchers reported a 25year single institution experience of postchemotherapy surgery for 158 patients with mediastinal primary nonseminoma germ cell tumours gcts. Autopsy findings in 20 patients with extragonadal germ cell tumors arising in the anterior mediastinum revealed only one case ofa testicular primary and one. Male extragonadal germ cell tumors egcts are characterized by a malignant transformation of germ cells without the presence of a gonadal primary tumor. Due to old age, the potential toxicity of systemic chemotherapy, and no significant signs of invasion to adjacent organs, we performed surgical resection. Extragonadal tumors arise when these cells become cancerous. Protocol for the examination of biopsy specimens from.
The prostate tumor responded to systemic chemotherapy with cisplatin, vinblastine and bleomycin and elevated lactate dehydrogenase and fetoprotein levels normalized. Adverse effects of chemotherapy, including gastrointestinal, hepatobiliary, genitourinary, and hematologic toxicity. Extragonadal gcts are rare and do not have a primary testicular involvement. The treatment of the extragonadal germ cell tumor is based on the stage, type, and location of the tumor. 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. Patients with extragonadal germ cell tumors version. Extragonadal germcell tumors are as curable as testicular germcell tumors when treated with. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell. Protocol for the examination of specimens from pediatric and adult patients with extragonadal germ cell tumors this protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites. In males, choriocarcinoma is generally considered a nonseminomatous germ cell tumor, which represents less than 5% of all germ cell tumors in males. Mediastinal germ cell tumor an overview sciencedirect topics.
Management of paediatric extracranial germcell tumours carries a unique set of challenges. Extragonadal germ cell tumors treatment pdqpatient. Germ cell tumors arise predominantly from within the testis, but an important subset of germ cell tumors are extragonadal in origin. Germ cell tumor is a neoplasm that takes origin from rests of primitive germ cells that normally migrate from the yolk sac to the urogenital ridge during early embryogenesis. 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. The most common sites for metastasis are the lungs. Extragonadal germ cell tumors treatment pdqpatient version. Extragonadal germ cell tumors occur much more commonly in males than in females 1 and are usually seen in young adults. Paediatric extracranial germcell tumours the lancet. The most frequent site of extragonadal germ cell tumours is the anterior mediastinum 1.
The testes and ovaries are the most common sites where gcts occur. Extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. 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. However, this report presents a case of a young patient who was found to have a large pericardial effusion and early signs of pericardial tamponade, which have not previously been reported as complications of extragonadal germ cell tumors, to the best of our knowledge. However, 25% of all germ cell tumors in adult men have extargonadal localization. Gcts are thought to originate from primordial germ cells, which migrate to the primitive gonadal glands in the urogenital ridge. The anterior mediastinum is the most common extragonadal primary site of germ cell tumors.
1353 129 1141 275 1132 1094 250 1581 1571 1103 596 1103 1608 1631 354 1504 1227 112 1518 1134 1618 869 136 1579 1442 596 508 146 1545 230 80 201 18 1201 1325 634 342 915 775 405 1035 519 248 1180 121