Family history is among the few established risk factors for. Related summaries ovarian germ cell tumors treatment. Germ and stromal cell tumors of the gonads and extragonadal germ cell tumors christian carrie edward c. Sep 10, 2018 retroperitoneal germ cell tumor diagnosed by endoscopic ultrasoundguided fine needle aspiration. Extragonadal germ cell tumor cancer information ohc. Enlarge extragonadal germ cell tumors form in parts of the body other than the gonads testicles or ovaries. Extragonadal germ cell tumors of the lung are among the rarest tumors encountered in pathology.
Extragonadal germ cell tumors have a variable clinical course, with the potential for aggressive behavior and widespread metastases. Incidence and mortality extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. Primary extragonadal germ cell tumors egcts are characterized by their location on the midline structures of the body from the pineal gland to. Protocol for the examination of specimens from patients. 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. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. Extragonadal germ cell tumors hollandfrei cancer medicine. Of extragonadal germ cell tumors, testicular carcinoma in situ cis are present in 3142% of cases, and cis are reported to have low sensitivity to chemotherapy in spite of the various morphology and to have a high likelihood of developing into testicular tumors. The origin is related to abnormal migration of primordial germ cells and tumor can occur along this.
Extragonadal germ cell tumors form in parts of the body outside the gonads. Extragonadal germ cell tumor home conditions extragonadal germ cell tumor. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. May 19, 2012 extragonadal localization of germ cell tumors gcts is rare. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Get detailed information about the treatment of newly diagnosed and recurrent extragonadal gcts in this summary for clinicians. 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 tumors arise when these cells become cancerous. Extragonadal germ cell tumors vanderbiltingram cancer. Gonadal germ cell tumor is generally the most common germ cell tumor type and constitutes 90% of germ cell tumors, whereas the mediastinum is the second most frequently affected area ahead of other extragonadal areas, which include retroperitoneum, the sacrococcygeal area, and the. Firstline highdose chemotherapy for patients with poor. His ct scan chest at presentation was significantfor a right apical mass of 3. Pdf we report a rare case of extragonadal germ cell tumour in a 55yearold man. Overall, approximately 5% to 10% of all germ cell cancers arise in nongonadal sites, particularly in the mediastinum and retroperitoneum.
Firstline highdose chemotherapy for patients with poor prognosis extragonadal germ cell tumors. Seminomas are common germ cell tumors 2, with a good prognosis and a. The following prognostic groups are used for extragonadal germ cell tumors. Case presentation a 69 year old male smoker presented with headache, unsteadiness, disorientation and was found to have a lung mass with multiple brain lesions. The disease is metastatic extragonadal germ cell tumor, not lung cancer. Extragonadal germ cell tumors is a topic covered in the pediatric surgery nat to view the entire topic, please sign in or purchase a subscription apsa pediatric surgery library combines pediatric surgery not a textbook nat with apsa expert, a powerful platform for earning moc cme credits all powered by unbound medicine. Germ cell tumors gcts also occur in the brain in children and young adults.
Blood tests, physical exams and scans of the abdomen, chest and pelvis, are used to. Jun 30, 2017 the true incidence of gonadal germ cell tumors gcts in children is unknown. 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. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. Management of paediatric extracranial germcell tumours carries a unique set of challenges. Surgical treatment of malignant mediastinal nonseminomatous. Case report extragonadal germ cell tumor in an older patient, not every mass is a lung cancer arnaoutakis, k. Primary extragonadal germ cell tumors were diagnosed in 4 patients in whom surgical or postmortem examination of the testis failed to reveal germ cell tumor. Pdf extragonadal germ cell tumors eggcts are uncommon neoplasms, which arise in anatomical locations other than gonads.
Mar 01, 2004 on the other hand, some researchers consider that all extragonadal gcts are metastases from the testes or ovaries, as no primordial germ cells were found in the extragonadal region and the prevalence of extragonadal gcts is only 1%2. 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. View large image view hires image download powerpoint slide. Paediatric extracranial germcell tumours the lancet. Extragonadal gcts are rare and do not have a primary testicular involvement. Testicular seminoma after the complete remission of. Extragonadal germ cell tumors represent a small subset of 25% of all germ cell malignancies. Extragonadal malignant germ cell tumor of the lung the. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant.
Find evidencebased information on extragonadal germ cell tumors treatment. 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. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Rnabinding protein lin28 is a marker for primary extragonadal germ cell tumors. If you have problems viewing pdf files, download the latest version of adobe reader. Stages of extragonadal germ cell tumors navigating care. Should extragonadal germ cell tumors be included in studies. The initial theory regarding the genesis of these tumors was that they represented metastasis from an occult gonadal primary. Noncentral nervous system extragonadal gcts are found in a variety of anatomic locations, but most commonly affect the mediastinum and. 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. A 66yearold man was referred to our hospital for an incidentally detected 40mm mass located at the interaortocaval area around the renal hilum.
Positron emission tomography ct revealed high accumulation suvmax 12. The following three tumor markers are used to detect extragonadal germ cell tumor. When cells that are meant to form sperm in the testicles or eggs in the ovaries. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. The extragonadal germ cell tumors egcts represent a unique entity, and as such require. Researchers have not yet determined what causes extragonadal germ cell tumors. Extragonadal germ cell tumor of retroperitoneal origin. Table of contents expand submenu for table of contents.
Cap approved pediatric extragonadal germ cell tumor germcell 3. 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. Herein, we report a case of extragonadal yst arising in the uterine round ligament. Feb 20, 2015 germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in the testes or ovaries. Nonproteincoding rnas represent another promising area of investigation in germcell tumours. Extragonadal germ cell tumors pediatric surgery nat. Blood tests, physical exams and scans of the abdomen, chest and pelvis, are used to provide doctors with more information about the tumor. Germcell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. If you have problems viewing pdf files, download the latest version of adobe. Germ cells are primitive cells within the body that normally mature into ova egg or sperm cells.
Germ cell tumors commonly occur in the gonads, but the anterosuperior mediastinum is the most common site of extragonadal germ cell tumors in adults. Extracranial, extragonadal germ cell tumors are rare. Pediatric germ cell tumor is the term used to describe malignant cancers of germline cells in patients aged 018 years. Socalled primary extragonadal germ cell tumors in the retroperitoneum are very likely a rare. During extragonadal germ cell tumor diagnosis, magnetic resonance imaging or a ct scan may be performed to determine the tumors size. Protocol for the examination of specimens from patients with extragonadal germ cell tumors version. He presented with a painless mass in right inguinal region, a few days after hernioplasty for right direct inguinal. Mediastinal and extrapulmonary origins must be excluded before a diagnosis of germ cell tumor of the lung can be confirmed.
Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. Age and gender can affect the risk of extragonadal germ cell tumors. Feb 05, 2020 extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Surgery for extragonadal germ cell tumor of mediastinum youtube. Surgical removal of the tumor if it is benign teratoma. However, the true incidence of these tumors may be higher than originally thought because of failure to diagnose them properly. The pluripotency gene lin28 is expressed in malignant germcell tumours across all age groups and histologies. Start here to find information on extragonadal germ cell tumors treatment. Few studies have been published concerning about pediatric gonadal gcts. Bilateral testes were normal by ultrasonography and physical examination, but the serum afp level was increased to 1161 ngml. These cancers may arise in the testis, the ovary, or the extragonadal sites including the sacrococcygeal area and the mediastinum. Management of paediatric extracranial germ cell tumours carries a unique set of challenges. Extragonadal germ cell tumors gct treatment depends on the type and can include surgery, radiation, chemotherapy, and stem cell transplant. Since this is a rare type of tumor they must also differentiate it from other types of tumors.
Male germ cell tumors gcts usually arise from the testis. 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 are rare, and 60% of such. The treatment of the extragonadal germ cell tumor is based on the stage, type, and location of the tumor. Extragonadal germ cell tumors treatment pdqpatient. The extragonadal germ cell tumors egcts represent a unique entity, and as such require specialized management. Extragonadal mixed germ cell tumor of the right arm.
He presented with a painless mass in right inguinal region, a few. Thoracic radiograph a and computed tomographic scan b. Patients with germ cell tumours are managed by a diverse array of specialists. Extragonadal germ cell tumors library navigating care. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. The metastatic tumor is the same type of tumor as the primary tumor. Extragonadal means outside of the gonads sex organs. Extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. Gonadal germ cell tumors in children pubmed central pmc. Should extragonadal germ cell tumors be included in. Blood levels of the tumor markers help determine if the tumor is a seminoma or nonseminoma. Mediastinal germ cell tumor causing superior vena cava tumor thrombosis. Extragonadal malignant germ cell tumor of the lung the journal of.
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 can take on a variety of tumor types. 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. Signs and symptoms of extragonadal germ cell tumors include breathing problems and chest pain. Two cases of primary extragonadal germ cell tumor of retroperiotoneal origin are reported. Retroperitoneal extragonadal germ cell tumors springerlink. The origin of primary extragonadal germ cell tumors is still a matter of debate. Cytologic features of extragonadal germ cell tumors. Extragonadal germ cell tumorshealth professional version. Thus, staging, risk stratification, and treatment approaches for germ cell. Halperin gonadal or extragonadal germ cell tumors gcts represent less than 2% of pediatric malignant tumors. This includes the pineal gland in the brain, the mediastinum area between the lungs, and retroperitoneum the back wall of the abdomen. 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. In adults, the most common sites, in order of frequency, are the anterior mediastinum, retroperitoneum, and the pineal and suprasellar regions.
We report a rare case of extragonadal germ cell tumour in a 55yearold man. Extragonadal germ cell tumors can be benign noncancer or malignant. Extragonadal germ cell tumor symptoms, diagnosis and. Paediatric extracranial germcell tumours sciencedirect.
Management of the primary malignant mediastinal germ cell tumors. There are many tumor markers that doctors can use to diagnose an extragonadal germ cell tumor. An extragonadal yolk sac tumor yst is a rare malignant germ cell tumor that usually occurs in childhood. Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life. May 28, 2018 extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided.
However, the true incidence of these tumors may conceivably be higher than originally thought because of failure to diagnose them properly. There is now general acceptance that extragonadal germ cell tumors represent malignant transformation of germinal elements distributed to these sites without a testicular focus. Between 2% and 5% of malignant germcell tumors in men arise at extragonadal sites. Extragonadal germ cell tumors treatment pdq patients. Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in either the testes or ovaries. Extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. Testicular carcinoma in situ in patients with extragonadal germcell. Retroperitoneal extragonadal germ cell tumor without distant. When cells that are meant to form sperm in the testicles or eggs in the ovaries travel. Extragonadal germ cell tumor eggct represents 510% of germ cell tumors and frequently occurs in mediastinum.
Cureus nonseminomatous extragonadal germ cell tumor. 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 germcell tumors are as curable as testicular germ cell tumors when treated with intensive cisplatincontaining. Malignant mediastinal germ cell tumor is a rare disease and represents only 1% to 4% of all mediastinal tumors. He had a large retroperitoneal tumor with lung, liver and supraclavicular lymph node metastases. Cao d, liu a, wang f, allan rw, mei k, peng y, et al. To determine if the tumor cells have spread beyond the originating site, a bone scan, ct scan of the lungs, chest xray, and bone marrow biopsy or aspirate may be performed. 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. Thus, staging, risk stratification, and treatment approaches for germcell. Patients with germcell tumours are managed by a diverse array of specialists.
The pathogenesis of extragonadal yst remains largely unknown, especially with regards to its cell of origin. They are usually found along the midline of the body and are most common in the mediastinum, thymus, retroperitoneal organs, and pineal gland. 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. Germ cell tumors arise predominantly from within the testis, but an important subset of germ cell tumors are extragonadal in origin. Imaging and blood tests are used to detect find and. The exact histogenesis has not been elucidated, but is. This article is from hereditary cancer in clinical practice, volume 11. Extragonadal gcts typically arise in midline locations, and specific sites vary with age.
An extragonadal yolk sac tumor presumed to be of postmeiotic. Mediastinal germ cell tumor an overview sciencedirect topics. For example, if an extragonadal germ cell tumor spreads to the lung, the tumor cells in the lung are actually cancerous germ cells. Extragonadal germ cell tumor genetic and rare diseases. The imaging characteristics of these tumors are nonspecific, and the definitive diagnosis of extragonadal germ cell tumor requires a biopsy. Extragonadal germ cell tumorspatient version national. He was referred to us after being treated for malignant lymphoma. The behavior of pediatric and adult extragonadal germ cell tumors egcts is quite. 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. The unrecognized extragonadal germ cell cancer syndrome. Extragonadal gcts typically arise in midline locations, and specific.
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