The tumors also occur along the midline path and can be found in the head, chest, abdomen, pelvis, and sacrococcygeal lower back area. Gcts are thought to originate from primordial germ cells, which migrate to the primitive gonadal glands in the urogenital ridge. The anterior mediastinal germ cell tumors gcts are the most common egct. Cap extragonadal germ cell tumor protocol revision history. Patients with germ cell 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 account for fewer than 10% of all germ cell malignancies. The therapeutic outcome for patients with extragonadal germcell tumors is now similar to that of pa tients with very advanced testicular cancer when considered. Benign extragonadal germ cell tumors are called benign teratomas. Primary mediastinal germ cell tumorsthe university of. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body.
Thirteen patients with extragonadal nonseminomatous germ cell tumors were. Protocol for the examination of specimens from pediatric. Abstract extragonadal germ cell tumors eggcts are uncommon neoplasms, which arise in anatomical locations other than gonads. One case demonstrated features of a mature teratoma on aspiration cytology. Pdf retroperitoneal extragonadal nonseminomatous germ. The hypothesis to explain the correlation between extragonadal germ cell tumors and ks could either be an incomplete or a miss migration of germ cells from the yolk sac toward the genital ridge. In adults, the most common sites, in order of frequency, are the anterior mediastinum, retroperitoneum, and the pineal and suprasellar regions. Cryptorchidism is a certain risk factor for testicular gcts 3. Cytologic features of extragonadal germ cell tumors. Primordial germ cells mismigrate during embryonic development and subsequently undergo malignant transformation. 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. Unusual imaging findings associated with germ cell tumors. Extragonadal germ cell tumors egct represent only 25% of adult germ cell malignancies.
Most women diagnosed with malignant ovarian germ cell tumors have curable disease and experience excellent survival with manageable treatmentassociated morbidity, related both to tumor biology and improvements in treatment over the last 4 decades. To characterize the clinical and biologic features of extragonadal germ cell tumor egct and to determine the overall outcome with currently available treatment strategies. Germ cell tumor, extr agonadal, azoospermia introduction germ cell tumors are the most common malignant tumors in young men between 25 and 29 years of age. It is known that about 5% of the germ cell tumors are extragonadal. Some publications from asia have reported inferior treatment outcomes compared to data from an international study group. The pathogenesis of these neoplasms is still poorly understood and it is a matter of debate if they really represent extragondal primary neoplasms or rather extragondal metastasis from occult gonadal neoplasms. Extragonadal germ cell tumors in japan request pdf. Germ cells normally occur inside the gonads ovary and testis.
The latter group can be divided into seminoma and nonseminoma germ cell tumors, which include the following. Jude childrens research hospital, the chance of children who are under. 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. Feb 05, 2021 extragonadal germ cell tumor egct is a relatively rare condition, reportedly representing 37% of all germ cell tumors.
Extragonadal means outside of the gonads sex organs. Extragonadal germ cell tumors egct, which are defined as germ cell tumors without a primary tumor in the testes are very rare. Germ cell tumors that form outside the testes or ovaries are known as extragonadal germ cell tumors. Gonadal origin should be excluded by careful testicular. The extragonadal germ cell tumors egcts represent a unique entity, and as such require specialized management. Germ cell tumors are the most common malignancy of the testicle, with a peak incidence in males between 15 and 35years old. Results of modern therapy for patients with mediastinal nonseminomatous germ cell tumors. A large number of egct have been described in association with ks, most often located in the mediastinum with a relative risk of at least 50. If untreated, malignant extragonadal germ cell tumors may spread to the lungs, lymph nodes, bones, liver, or other parts of the body. Germ cell tumor gct is a neoplasm derived from germ cells. These are more common than malignant extragonadal germ cell tumors and often are very large. Prognostic factors in children with extragonadal malignant. To ascertain whether this is generally the case, here we analyze treatment outcomes for 30 japanese patients with egct. Of an unselected population of 635 consecutive patients treated from 1975 through 1996 at 11 cancer centers, 341 patients 54% had primary mediastinal egct, and 283 patients 45% had.
Retroperitoneal extragonadal germ cell tumor presenting as a. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. 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. Extragonadal germ cell tumors of the head and neck region. Mixed germ cell tumor 16 cases of the 16 mixed gcts, aspiration smears showed features of more than 1 gct component in 6 cases. After the formation of germ cells, it migrates from the gonad to the rest of the body which form the extracranial extragonadal germ cell tumors. Approximately 90% of germ cell tumors arise in the testicles, though extragonadal primary tumors of the retroperitoneum, mediastinum, and pineal gland do occur. Sep 30, 2020 several case reports show an incidence of extragonadal germ cells tumors egct of 1. Primary extragonadal germ cell tumors are rare and their histogenetic origin is not clear. Despite the unknown causing factor of egcts, researchers suggested the tumor cells begin from the germ cells which locate in the testes in male and ovaries in female. Survival of patients with extragonadal nonseminomatous germ cell tumors remains inferior to that of patients with advanced testicular cancer, although treatment is often the same for both conditions. The abnormal hormonal influence could also increase the malignant potential 18. We report a rare case of a retroperitoneal eggct presenting as a bulky pelvic mass located between the left internal and external iliac arteries in a 29.
Expression profile of genes from 12p in testicular germ cell tumors of adolescents and adults associated with i12p and amplification at 12p11. Extragonadal germ cell tumors clinical presentation. May 24, 2016 extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Thus, staging, risk stratification, and treatment approaches for germ cell.
Pdf retroperitoneal extragonadal nonseminomatous germ cell. Therefore, onethird of the tumors have mixed patterns, and tumors arising from germ cells at different stages of their development should exhibit different features 47. Extragonadal germ cell tumor genetic and rare diseases. Palifosfamide in treating patients with recurrent germ. They are aggressive neoplasms and can arise virtually anywhere, but typically the site of origin is in the midline mediastinum, retroperitoneum, or pineal gland. Schmoll, extragonadal germ cell tumors, annals of metastasis rate has been reported to be 27% in mediastinal oncology, vol. Germ cell tumors are the most common malignancy of the testicle, with a peak incidence in males between 15 and 35 years old.
Rnabinding protein lin28 is a marker for primary extragonadal germ cell tumors. Tumor sites outside the gonad are called extragonadal sites. Ovarian germ cell tumors union for international cancer. By continuing to browse this site you are agreeing to our use of cookies. Management of paediatric extracranial germcell tumours carries a unique set of challenges. A 21 and 18 yearsold patients presented retroperitoneal choriocarcinoma and yolk sac tumor, respectively. No ajccuicc tnm staging system the childrens oncology group staging recommendations are included. In pediatric patients, extragonadal germ cell tumors are often found intracranially and in the pelvis, mediastinum, and retroperitoneum, from highest to lowest rate of prevalence, as well as in other sites 2,10. Background documentation pediatric extragonadal germ cell tumor 4. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. We describe two cases presenting as primary retroperitoneal germ cell tumors without clinical evidence of testicular tumor. Of the 8000 annual cases of gct, only 25% are of extragonadal origin. The gcts exhibit various pathologic features according to the totipotentiality of the tumor cells.
Why human extragonadal germ cell tumours occur in the midline of. Patients with extragonadal germ cell tumors version. 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. Germ cell tumors atlas of genetics and cytogenetics. Extragonadal germ cell tumors occur much more commonly in. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. G erm cell tumors gcts are common tumors of gonads ie, testis and ovaries. Germ cell tumors health encyclopedia university of. Cytologic features of extragonadal germ cell tumors gupta. Pluripotent embryonic cells mismigrate and give rise to gcts.
Some publications from asia have reported inferior treatment outcomes compared to data from an. Campbell university department of clinical oncology, newcastle general hospital, newcastle upon tyne, uk. Should extragonadal germ cell tumors be included in. Primary extragonadal germ cell tumors of the retroperitoneum. 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. Extragonadal germ cell tumors are usually seen in children or young adults and typically arise in midline locations. Risk factors for malignant extragonadal germ cell tumors include being male, being age 20 or older, and having klinefelter syndrome. Cao d, liu a, wang f, allan rw, mei k, peng y, et al. Primary retroperitoneal extragonadal germ cell tumors.
They usually are large and cause signs and symptoms. Extragonadal germ cell tumors treatment pdq patients. A prospective clinical study will be needed to 246 extragonadal germ cell tumor with high serum levels of dupan2 247 examine the usefulness of dupan2 for the management of cell tumor of mediastinum with high serum level of carcino germ cell tumor. Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in either the testes or ovaries 1.
Protocol for the examination of resection specimens from. Extragonadal mixed germ cell tumor of the right arm. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. All reports of family inheritance focused exclusively in isolated germ cell tumors of testis 4, 6, 7 and the estimation of the contribution of extragonadal germ cell tumor in the familial setting is not known. These tumors affect both adults and children and therefore the recommendations apply to the eml lists for both adults and children regimen distinctions are included in the present briefing. Gcts include seminomas 3571% and nonseminomatous germ cell tumors nsgcts 2. Mar 07, 2019 extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Teratomas are a specific type of germ cell tumor that contain mature elements from all three embryonic germ layersthe ectoderm, mesoderm, and endoderm 1. Case report open access extragonadal mixed germ cell tumor of.
Klinefelter syndrome 47 xxy is the only known predisposing factor. Paediatric extracranial germcell tumours the lancet. Extragonadal germ cell tumors often develop in midline structures fig 1. February 2019 includes the childrens oncology group staging system accreditation requirements the use of this protocol is recommended for clinical care purposes but is. Sep 10, 2018 retroperitoneal germ cell tumor diagnosed by endoscopic ultrasoundguided fine needle aspiration. This process is experimental and the keywords may be updated as the learning algorithm improves. Malignant ovarian germ cell tumors occur predominantly in girls, adolescents. Steele, neoplasm of the testis, in to be 49% in retroperitoneal tumors 4. Signs and symptoms of extragonadal germ cell tumors include breathing problems and chest pain. Egct usually occur in males between the ages of 20 and 35 years, but can occur at any age. In adults, the most common sites of primary extragonadal germ cell tumors are, in descending order, the mediastinum, retroperitoneum, and cranium. Extragonadal germ cell tumors eggcts are uncommon neoplasms, which arise in anatomical locations other than gonads. Extragonadal germ cell tumors account for 15% of all germ cell tumors. Nonseminomas tend to grow and spread more quickly than seminomas.
As outlined below, within the pediatric age range, prognosis is worse with increasing age. Chromosome 12 abnormalities in malignant ovarian germ cell tumors. These tumors are usually diagnosed with imaging such as ct scans combined with blood tests but may also require a surgical biopsy for analysis. Germ cell tumors are also found in extragonadal sites, likely arising from primordial germ cells that fail to migrate to the gonads 1. Extragonadal germ cell college of american pathologists. Survival of patients with malignant extragonadal germ cell tumors patient cohort no. We strongly recommend that you talk with a trusted. Paediatric extracranial germcell tumours the lancet oncology.
Their occurrence at extragonadal sites, either as primary tumors or as metastatic foci, is rare. Extragonadal germ cell tumors can be benign teratoma or malignant. Management of extragonadal germcell tumors and the significance. Poor prognosis of retroperitoneal mixed extragonadal germ. 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.
Extragonadal germ cell tumors of the mediastinum and. Both primary and salvage treatment of these tumors constitute a challenge. Should extragonadal germ cell tumors be included in studies. Hypotheses on the origin and distribution of extragonadal germ cell tumours. Primary extragonadal germ cell tumors pegct are rare neoplasms. The tumor can be benign or malignant cancerous by its growth rate. Gynaecomastia and extragonadal symptoms leading to diagnosis delay of germ cell tumours in young men. Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life. Germ cell tumors gcts in males mainly develop in testicular tissue. Extragonadal germ cell tumors are often associated with. Apr 01, 2006 the hypothesis to explain the correlation between extragonadal germ cell tumors and ks could either be an incomplete or a miss migration of germ cells from the yolk sac toward the genital ridge. There they develop into eggs in females and sperm in males. Abstract extragonadal germ cell tumors eggct are commonly found in the midline of the body.
In the 26 patients, seminomas, 10 nonseminomatous germ cell tumors and two tumors suspicious for a germ cell tumor were found at histological examination of the extragonadal mass. In addition, the prognosis for nonseminomatous tumors has been shown to be worse than for seminoma. Protocol for the examination of biopsy specimens from. Young patients with a germ cell tumor are not routinely tested for klinefelter syndrome. Germ cell tumor embryonal carcinoma granulosa cell tumor immature teratoma struma ovarii these keywords were added by machine and not by the authors.
Extragonadal germ cell tumors overview american association. Protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites. Schmoll published extragonadal germ cell tumors find, read and cite all the research you need on researchgate. Gynaecomastia and extragonadal symptoms leading to diagnosis. Malignant extragonadal germ cell tumors are divided into two types, nonseminoma and seminoma. While most extragonadal tumors are more challenging to treat, in general, germ cell tumors have an excellent overall prognosis with 5year survival. We describe two cases presenting as primary retroperitoneal germ cell tumors without clinical evidence of. The clinical course is variable, with a potential for aggressive behavior and widespread.
Extragonadal germ cell tumors can be benign noncancer or malignant. They have a poor prognosis, different behavior, and natural course compared to their gonadal counterparts. Protocol for the examination of specimens from pediatric and. Extragonadal germ cell tumors occur much more commonly in males than in females and are usually seen in young adults. The primary tumor was on the left side in 10 of 26 patients 38%, on the right side in seven 27% and bilaterally in nine 35%. Age and gender can affect the risk of extragonadal germ cell tumors. Extragonadal germ cell tumors treatment pdqpatient.
Extragonadal germ cell tumors treatment pdq pdq cancer. We recommend complete evaluation of cancer in patients with infertility and azoospermia. The most widely accepted theory suggests that extragonadal germ cell tumors arise from primordial germ cells misplaced during their migration to gonads. The therapeutic outcome for patients with extragonadal germ cell tumors is now similar to that of pa tients with very advanced testicular cancer when considered. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. Pediatric and adult patients with extragonadal germ cell tumors.
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