The most common site for germ cell tumors to arise is within the gonadal tissue. The clinical manifestations, diagnosis, and staging are presented separately, as are more detailed discussions of specific clinical scenarios. Malignant ovarian germ cell tumors classification according to the world health. Concurrent mediastinal germcell tumour and haematological malignancy. Mediastinal germ cell tumours develop in germ cells. Aug 18, 2011 primary mediastinal germ cell tumor is a rare subgroup of germ cell tumors reported to account for less than 5% of germ cell malignancies. In poor prognosis nonseminomatous germ cell tumours nsgct, overall survival.
Primary extragonadal germ cell tumors egcts are characterized by their location on the midline structures of the body from the pineal gland to the coccyx, such as central nervous system cns, mediastinum. Handbook for children with germ cell tumors american pediatric. Extragonadal means outside of the gonads sex organs. Primary mediastinal germ cell tumors gcts are rare in children and still represent a challenge for both adult and pediatric oncologists because of their worse outcome compared to their gonadal counterpart. Anterior mediastinal lymph nodes andor diffuse thymic infiltration secondary tolymphoma, especially nodular scle.
More than half of mediastinal germ cell tumors are mature teratomas. Testicular germ cell tumors account for 90% of primary tumors of the testes. It has similar clinical characteristics and histopathologic distributions as its counterparts in gonads moran et al. Primary mediastinal germ cell tumor pmgct is a rare cancer that accounts for less than 5% of germ cell malignancies and only 14% of all mediastinal tumors 1, 2. For detailed discussion please refer the articles listed at the end of this page. Men with this genetic condition are born with an extra x chromosome. Management of the primary malignant mediastinal germ cell. Testicular germ cell tumors consider the testis protocol. The next section in this guide is symptoms and signs. We present a series of 3 cases of primary germ cell tumor of the mediastinum, which after chemotherapy, fulfilled the criteria for growing teratoma syndrome and were managed with surgical excision. Nichols patientwithmarkerpositivenonsmallcelllungcancer.
He underwent 4 rounds of chemotherapy followed by surgery to remove the remainder of the tumor. Extension and initial remission rate are independent prognostic factors. Therapeutic challenges for cisplatinresistant ovarian germ cell. Testicular germ cell tumors radiology reference article. The mediastinum is the area in the centre of the chest, between the lungs. Gcts are believed to originate from tissue stem cells and arise from the germinal epithelium of the seminiferous tubules owing to atypical cell proliferation known as testicular intratubular germ cell neoplasia unclassified itgcnu. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. However, there is now abundant clinical evidence to substantiate. Although the histomorphological features of mediastinal gcts are similar to those of gonadal gcts, the biological behavior, clinical.
Resection of primary mediastinal nonseminomatous germ cell tumors. In adults, 30% of ovarian tumors are of germ cell origin. Mediastinal and other extragonadal germ cell tumors were initially thought to represent isolated metastases from an inapparent gonadal primary site. Extragonadal germ cell tumors treatment pdq patients.
For example, if an extragonadal germ cell tumor spreads to the lung, the tumor cells in the lung are actually cancerous germ cells. These germ cells travel into the pelvis as ovarian cells or into the scrotal sac as testicular cells. The most common site for an extragonadal germ cell tumor is the anterior mediastinum. Perinatal factors, including exposure to endocrine disrupting chemicals, have been suggested to be related to risk. Brain metastasis from nonseminomatous germ cell tumors of. 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. Printable germ cell tumors of the mediastinum thymus. Whenever possible, a tissue diagnosis should be obtained to determine histologic subtype, the presence of nongerm cell elements within the germ cell cancer or the rare mediastinal germ cell tumors craig r. Extragonadal germ cell tumors gcts are mainly midline in location, with mediastinum being the most common site. The disease is metastatic extragonadal germ cell tumor, not lung cancer. Thymic carcinoid tumor alsomayresemble germ cell tumor onct.
Germ cell tumours usually develop in the testicles or ovaries but can grow in other areas of the body, such as the mediastinum pronounced mediastynum. However, in 5% of the cases, they are extragonadal in origin. Origin of adult male mediastinal germ cell tumours. Protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites. Sometimes, a group of germ cells grows in a way thats not normal. A germ cell tumor gct is a neoplasm derived from germ cells. Treatment strategies and prognostic factors of patients with. Successful management of brain metastasis from malignant germ cell tumours with standard induction chemotherapy. Initial therapy of early stage testicular germ cell tumors gcts is based on histology and tumor extent.
Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. Malignant germ cell tumors gct are malignant tumors that are formed by immature cells that begin in the reproductive cells of the testes or ovaries. Gct are infrequent in childhood, occurring at a rate of 2. Primary mediastinal germ cell tumor with intratubular germ cell neoplasia of the testisfurther support for germ cell origin of these tumors. Mediastinal germ cell tumor an overview sciencedirect topics. 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. A total of 54 patients with pmmgct in a followup from 1990 to 2009. Mediastinal germ cell tumors mgcts account for 19%25% of all mediastinal tumors and 3%6% of all gcts in pediatric age 1. Childhood central nervous system germ cell tumors treatment. Prognostic factors in mediastinal germ cell tumors include.
Germ cell tumors commonly occur in the gonads, but the anterosuperior mediastinum is the most common site of extragonadal germ cell tumors in adults. Germ cells normally occur inside the gonads ovary and testis. We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. Testicular tumors most commonly occur in men between 20 and 35 years of age, and are the most common solid malignancy in this group. Diagnosis is made primarily based on palpation and findings on testicular ultrasound. The most common extragonadal site is reported to be in the mediastinum. Fiftytwo patients underwent surgical resections, while. All of the patients had undergone surgery plus wholebrain radiotherapy wbrt, and chemotherapy based on cisplatin.
Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. Intracranial seminoma commonest histology 6070% of all germ cell tumors. Malignant mediastinal germ cell tumors of various histologies were first described as a clinical entity approximately 50 years ago. Furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors.
Stem cell marker oct34 in biology and diagnostics of germ cell. Sections show a nonseminomatous mixed germ cell tumor showing extensive tumor necrosis. Conclusions primary mediastinal germ cell tumor is rare with a dominant frequency in young male patients. They are the most common nonhaematologic malignancy in men 1549 years old. Germ cell tumors danafarberboston childrens cancer and. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Nonetheless, more than 95% of testicular neoplasms are derived from germ cells arrested in their differentiationthe testicular germ cell tumors. In children and adolescents, more than 60% of ovarian neoplasms are of germ. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors. The histopathological characteristics of pmgcts are similar to those of gonadal germ cell tumors gcts. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. The vast majority of testicular tumors are gcts 95%. Primary malignant mediastinal germ cell tumor pmmgct is rare and sometimes the prognosis of the patients with pmmgct is not very satisfactory. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant.
Pdf benign and malignant germ cell tumors of the mediastinum. 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. Malignant germ cell tumors of the mediastinum mayo clinic. These are the cells in the body that develop into sperm and eggs. The most recent classification of cns tumors implements molecular parameters for the first time, in addition to histology, to define brain tumor entities. Dec 10, 2012 yolk sac tumor, second in frequency, occurs alone or in combination with a teratoma. My husband was diagnosed with germ cell cancer in 2007 29 years of age the tumor was the size of his fist and was located in his mediastinum, obstructing his aorta. Chemotherapy combined with local therapy like surgery or radiotherapy is a reasonable treatment strategy recommended. Pineal region tumor germ cell tumor most common pineoblastoma pineocytoma pineal parenchymal tumor of intermediate differentiation meningioma ependymoma central neurocytoma metastasis benign cyst suprasellar region tumor pituitary adenoma most common germ cell tumor craniopharyngioma. Germ cell tumor the name comes from the fact that these tumors occur in a germ cell, one that germinates life such as in the testes or ovaries in an organism that reproduces sexually. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males.
The diagnosis of gonadal germ cell tumors usually requires immunohistochemical verification. However, there are a few factors that increase the risk failed testicular descent. However, a small percentage of germ cell tumors arise in extragonadal sites. The metastatic tumor is the same type of tumor as the primary tumor. Thislesionmayappear asawell circumscribed massormayobliterate contiguous fatplanes. In these cases, the mediastinal germ cell tumor develops before or concomitantly with but not after acute megakaryoblastic leukemia. Klinefelters syndrome is connected to a higher risk of a germ cell tumor in the chest. 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. Association between mediastinal germ cell tumors and hematologic malignancies. Teratomas and other germ cell tumors of the mediastinum. Yolk sac tumor, second in frequency, occurs alone or in combination with a teratoma.
Diagnostic staging further includes an abdominopelvic and chest ct, determination of. Biopsy consider extragonadal germ cell biopsy protocol. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. A mature teratoma component is present in the majority of the primary tumors 86%. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. Germ cell tumours are embryologically derived from reproductive cells, and they originate mostly from the gonads. Prospectively collected data concerning patients enrolled in the italian association of pediatric haematology and oncology study on malignant gcts aieop. The mediastinum is the most common extragonadal primary tumor site 4.
Germ cell tumors occur most frequently in the gonad but can rarely occur in extragonadal locations, usually in or near the midline. Germ cell tumors danafarberboston childrens cancer. You can also get a germ cell tumor in the brain, chest, belly. The most common extragonadal site of primary germ cell tumors is the anterior mediastinum. Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1,2. Pure seminoma is sensitive to radiotherapy and the prognosis is good.
Hematologic neoplasia associated with primary mediastinal germcell tumors. Some genetic syndromes caused by extra or missing sex chromosomes can cause incomplete or abnormal development of the reproductive system. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Primary mediastinal malignant gct pmmgct is a rare cancer contributing tumors. Etiologic factors in testicular germ cell tumors ncbi. Successfully treating your childs germ cell tumor depends on where the tumor is located and whether it is benign or malignant. Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. Germ cell tumors can spread to lymph nodes, lung, liver, and brain.
Also, a v ats appr oach can be use d if the tumor is not readily. Germ cell and stromal tumors of the ovary university of kentucky. Rosti g, secondino s, necchi a, fornarini g, pedrazzoli p. Sep 10, 2018 growing teratoma syndrome is the increase in tumor size during or after chemotherapy for mediastinal germ cell tumors mgct or retroperitoneal germ cell tumors rgct in which histologic analysis of the resected tumor specimen shows only mature teratoma. Although yolk sac tumor of the cervix or vagina is the subject of a few case reports, yolk sac tumor of the infant testis is more prevalent and is the most common germ cell tumor arising from this organ 2, 4, 8, 20.
Seminoma is the second most common mediastinal germ cell tumor. 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. Os within a clinical trial depends on the number of patients treated at the. Primary brain tumors, including germ cell tumors gcts, are a diverse group of diseases that together constitute the most common solid tumor of childhood. The following prognostic groups are used for extragonadal germ cell tumors. Three types will be considered, namely, leydig cell tumors, sertoli cell tumors, and gonadoblastomas. An overview of therapy for men with testicular gcts is presented here. The anterior mediastinum is the most common extragonadal primary site of germ cell tumors. Mediastinal germ cell tumor an overview sciencedirect.
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. Mediastinal germ cell tumor medical condition youtube. Extragonadal germ cell tumors clinical presentation. These tumors account for approximately 10% of all primary mediastinal neoplasms. Stem cell marker oct34 in tumor biology and germ cell tumor diagnostics. Surgical management of primary mediastinal germ cell tumors. A teratoma of lung is the most common type of germ cell tumor of the lung. The following factor may raise a persons risk of developing an extracranial, extragonadal germ cell tumor. Case presentation an 18 yearold hispanic man with a history of a benign leg tumor presented with two week history of progressive sob and hemoptysis. Extragonadal germ cell tumors treatment pdqpatient. Sarkaria is, bains ms, sood s, sima cs, reuter ve, flores rm, et al. Mediastinal germ cell tumors in pediatric patients.
With this, the neoplasm can metastasize and affect other areas of the body. 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. A study of patients with primary mediastinal germ cell. Metachronous germ cell tumors of the mediastinum sciencedirect. Nonseminomatous germ cell tumor, which is a rare entity, representing less than 0.
Most often, patients present with a painless nodule or swelling of the testis. Vinblastine plus ifosfamide plus cisplatin as initial salvage therapy in recurrent germ cell tumor. A study of patients with primary mediastinal germ cell tumors. The most common extragonadal sites are the mediastinum and retroperitoneum.
After three cycles of chemotherapy for the mediastinal nonseminomatous germ cell tumor, the serum. A germcell tumor gct is a neoplasm derived from germ cells. The three most common genetic aberrations in the bone marrow cells of these individuals representing 65% of all cases are inversions in the long arm of chromosome 12, trisomy 8, and an extra x chromosome. Germ cell tumors are one of the causes of anterior mediastinal mass, and any of the germ cell histologies may be identified. The exact causes of testicular germ cell tumours are unknown. Testicular tumors knowledge for medical students and. Approximately 56% of all testis tumors are non germ cell tumors of the testis. Primary mediastinal nonseminomatous germ cell tumour is a rare entity, and it accounts for 5% of all germ cell tumours. May 01, 2015 primary extragonadal malignant germ cell tumors emgcts are rare and account for only 25% of the malignant germ cell tumors mgcts. Germ cell cancer is a malignant neoplasm that originates from germ cells.
Clinical and epidemiological characteristics of children with germ. Symptoms, risk factors and treatments of mediastinal germ cell tumor medical condition mediastinal germ cell tumors are tumors that derive from germ cell rest remnants in the mediastinum this. The percentage of viable tumor is estimated at 20%. However, the lung is a very uncommon site for such a tumor, and hence, the origin of the teratoma from other common sites such as gonads should be eliminated prior to establishing a diagnosis of primary teratoma of lung. Although yolk sac tumor of the cervix or vagina is the subject of a few case reports, yolk sac tumor of the infant testis is more prevalent and is the most common germ cell tumor arising from this organ 2. We evaluated the role of the surgical treatment and the effect of multimodality treatment strategy for patients with pmmgct. Germ cells are cells that develop in the embryo which later becomes eggs and sperm in the ovaries and testes respectively. The imaging features of seminomas are usually those of a large, wellmarginated, homogeneous, anterior mediastinal mass with softtissue opacity or attenuation that shows minimal contrast enhancement. Ikdahl t, josefsen d, jakobsen e, delabie j, fossa sd.