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. Extragonadal germ cell tumors gcts are mainly midline in location, with mediastinum being the most common site. Primary brain tumors, including germ cell tumors gcts, are a diverse group of diseases that together constitute the most common solid tumor of childhood. 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. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors. However, a small percentage of germ cell tumors arise in extragonadal sites. Approximately 56% of all testis tumors are non germ cell tumors of the testis. With this, the neoplasm can metastasize and affect other areas of the body. Surgical management of primary mediastinal germ cell tumors. Os within a clinical trial depends on the number of patients treated at the.
Ikdahl t, josefsen d, jakobsen e, delabie j, fossa sd. Initial therapy of early stage testicular germ cell tumors gcts is based on histology and tumor extent. A mature teratoma component is present in the majority of the primary tumors 86%. 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. You can also get a germ cell tumor in the brain, chest, belly. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Most often, patients present with a painless nodule or swelling of the testis. Germ cells are cells that develop in the embryo which later becomes eggs and sperm in the ovaries and testes respectively.
Mediastinal germ cell tumors in pediatric patients. Malignant mediastinal germ cell tumors of various histologies were first described as a clinical entity approximately 50 years ago. After three cycles of chemotherapy for the mediastinal nonseminomatous germ cell tumor, the serum. 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. Rosti g, secondino s, necchi a, fornarini g, pedrazzoli p. Mediastinal germ cell tumours develop in germ cells. 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. 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. 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. Conclusions primary mediastinal germ cell tumor is rare with a dominant frequency in young male patients. Vinblastine plus ifosfamide plus cisplatin as initial salvage therapy in recurrent germ cell tumor. Pure seminoma is sensitive to radiotherapy and the prognosis is good. Anterior mediastinal lymph nodes andor diffuse thymic infiltration secondary tolymphoma, especially nodular scle. Extragonadal germ cell tumors clinical presentation. Nichols patientwithmarkerpositivenonsmallcelllungcancer. The most common extragonadal sites are the mediastinum and retroperitoneum. The vast majority of testicular tumors are gcts 95%. A teratoma of lung is the most common type of germ cell tumor of the lung. Germ cell and stromal tumors of the ovary university of kentucky.
Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. Malignant germ cell tumors of the mediastinum mayo clinic. They are the most common nonhaematologic malignancy in men 1549 years old. It has similar clinical characteristics and histopathologic distributions as its counterparts in gonads moran et al. Brain metastasis from nonseminomatous germ cell tumors of. Handbook for children with germ cell tumors american pediatric. Thymic carcinoid tumor alsomayresemble germ cell tumor onct. Metachronous germ cell tumors of the mediastinum sciencedirect. Successfully treating your childs germ cell tumor depends on where the tumor is located and whether it is benign or malignant. The clinical manifestations, diagnosis, and staging are presented separately, as are more detailed discussions of specific clinical scenarios.
Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant. 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. Perinatal factors, including exposure to endocrine disrupting chemicals, have been suggested to be related to risk. Malignant ovarian germ cell tumors classification according to the world health. Extension and initial remission rate are independent prognostic factors. Sometimes, a group of germ cells grows in a way thats not normal. Primary malignant mediastinal germ cell tumor pmmgct is rare and sometimes the prognosis of the patients with pmmgct is not very satisfactory.
Prospectively collected data concerning patients enrolled in the italian association of pediatric haematology and oncology study on malignant gcts aieop. Association between mediastinal germ cell tumors and hematologic malignancies. 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. Extragonadal means outside of the gonads sex organs. In adults, 30% of ovarian tumors are of germ cell origin. These germ cells travel into the pelvis as ovarian cells or into the scrotal sac as testicular cells. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Seminoma is the second most common mediastinal germ cell tumor. Germ cell tumours are embryologically derived from reproductive cells, and they originate mostly from the gonads. Primary mediastinal nonseminomatous germ cell tumour is a rare entity, and it accounts for 5% of all germ cell tumours. Delayed effects of whole brain radiotherapy in germ cell tumor patients with central nervous system metastases.
Nonseminomatous germ cell tumor, which is a rare entity, representing less than 0. The mediastinum is the area in the centre of the chest, between the lungs. Concurrent mediastinal germcell tumour and haematological malignancy. The most common site for an extragonadal germ cell tumor is the anterior mediastinum. 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. Diagnosis is made primarily based on palpation and findings on testicular ultrasound.
Klinefelters syndrome is connected to a higher risk of a germ cell tumor in the chest. Testicular germ cell tumors account for 90% of primary tumors of the testes. Testicular germ cell tumors radiology reference article. Mediastinal and other extragonadal germ cell tumors were initially thought to represent isolated metastases from an inapparent gonadal primary site. Germ cells normally occur inside the gonads ovary and testis. Testicular tumors knowledge for medical students and. Yolk sac tumor, second in frequency, occurs alone or in combination with a teratoma. Germ cell tumors occur most frequently in the gonad but can rarely occur in extragonadal locations, usually in or near the midline.
In these cases, the mediastinal germ cell tumor develops before or concomitantly with but not after acute megakaryoblastic leukemia. Mediastinal germ cell tumor an overview sciencedirect. 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. Therapeutic challenges for cisplatinresistant ovarian germ cell. Sections show a nonseminomatous mixed germ cell tumor showing extensive tumor necrosis. Intracranial seminoma commonest histology 6070% of all germ cell tumors.
Nonetheless, more than 95% of testicular neoplasms are derived from germ cells arrested in their differentiationthe testicular germ cell tumors. However, in 5% of the cases, they are extragonadal in origin. Protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites. The next section in this guide is symptoms and signs. 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. Germ cell cancer is a malignant neoplasm that originates from germ cells.
Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. 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. Testicular tumors most commonly occur in men between 20 and 35 years of age, and are the most common solid malignancy in this group. Origin of adult male mediastinal germ cell tumours. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. Biopsy consider extragonadal germ cell biopsy protocol. 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. Pdf benign and malignant germ cell tumors of the mediastinum. Fiftytwo patients underwent surgical resections, while. However, there are a few factors that increase the risk failed testicular descent. Extragonadal germ cell tumors treatment pdqpatient.
The most common extragonadal site is reported to be in the mediastinum. The most recent classification of cns tumors implements molecular parameters for the first time, in addition to histology, to define brain tumor entities. Furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors. Gct are infrequent in childhood, occurring at a rate of 2. Etiologic factors in testicular germ cell tumors ncbi. Some genetic syndromes caused by extra or missing sex chromosomes can cause incomplete or abnormal development of the reproductive system. 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. Thislesionmayappear asawell circumscribed massormayobliterate contiguous fatplanes. These are the cells in the body that develop into sperm and eggs. Although the histomorphological features of mediastinal gcts are similar to those of gonadal gcts, the biological behavior, clinical. The following prognostic groups are used for extragonadal germ cell tumors. 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. Diagnostic staging further includes an abdominopelvic and chest ct, determination of.
A total of 54 patients with pmmgct in a followup from 1990 to 2009. Germ cell tumors danafarberboston childrens cancer. The metastatic tumor is the same type of tumor as the primary tumor. We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. Stem cell marker oct34 in tumor biology and germ cell tumor diagnostics. Mediastinal germ cell tumor medical condition youtube. The diagnosis of gonadal germ cell tumors usually requires immunohistochemical verification. Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. Treatment strategies and prognostic factors of patients with. 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. Prognostic factors in mediastinal germ cell tumors include.
A germ cell tumor gct is a neoplasm derived from germ cells. 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. The most common extragonadal site of primary germ cell tumors is the anterior mediastinum. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. We report a rare case of mediastinal nonseminomatous germ cell tumor arising after 2 complete resections of mediastinal mature teratomas 18 and 10 years prior. The percentage of viable tumor is estimated at 20%. Stem cell marker oct34 in biology and diagnostics of germ cell. For example, if an extragonadal germ cell tumor spreads to the lung, the tumor cells in the lung are actually cancerous germ cells. Printable germ cell tumors of the mediastinum thymus. Protocol for the examination of resection specimens.
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. 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. Resection of primary mediastinal nonseminomatous germ cell tumors. Testicular germ cell tumors consider the testis protocol. The anterior mediastinum is the most common extragonadal primary site of germ cell tumors. We evaluated the role of the surgical treatment and the effect of multimodality treatment strategy for patients with pmmgct. Also, a v ats appr oach can be use d if the tumor is not readily. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. May 01, 2015 primary extragonadal malignant germ cell tumors emgcts are rare and account for only 25% of the malignant germ cell tumors mgcts. Childhood central nervous system germ cell tumors treatment. 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. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males.
Teratomas and other germ cell tumors of the mediastinum. More than half of mediastinal germ cell tumors are mature teratomas. Hematologic neoplasia associated with primary mediastinal germcell tumors. However, there is now abundant clinical evidence to substantiate. A study of patients with primary mediastinal germ cell. Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1,2. Germ cell tumors commonly occur in the gonads, but the anterosuperior mediastinum is the most common site of extragonadal germ cell tumors in adults. Sarkaria is, bains ms, sood s, sima cs, reuter ve, flores rm, et al. 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. Dec 10, 2012 yolk sac tumor, second in frequency, occurs alone or in combination with a teratoma. 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. Primary mediastinal malignant gct pmmgct is a rare cancer contributing tumors.
The histopathological characteristics of pmgcts are similar to those of gonadal germ cell tumors gcts. 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. These tumors account for approximately 10% of all primary mediastinal neoplasms. Successful management of brain metastasis from malignant germ cell tumours with standard induction chemotherapy. 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. Mediastinal germ cell tumor an overview sciencedirect topics. The most common site for germ cell tumors to arise is within the gonadal tissue. Primary mediastinal germ cell tumor with intratubular germ cell neoplasia of the testisfurther support for germ cell origin of these tumors. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. In poor prognosis nonseminomatous germ cell tumours nsgct, overall survival. A study of patients with primary mediastinal germ cell tumors. The following factor may raise a persons risk of developing an extracranial, extragonadal germ cell tumor. A germcell tumor gct is a neoplasm derived from germ cells.
The disease is metastatic extragonadal germ cell tumor, not lung cancer. 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. Extragonadal germ cell tumors treatment pdq patients. 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. The mediastinum is the most common extragonadal primary tumor site 4. For detailed discussion please refer the articles listed at the end of this page. He underwent 4 rounds of chemotherapy followed by surgery to remove the remainder of the tumor. 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.
These extragonadal germ cell tumors histologically contain the same components as their gonadal counterparts, but may have different biologic behaviors, clinical characteristics and. Germ cell tumors can spread to lymph nodes, lung, liver, and brain. All of the patients had undergone surgery plus wholebrain radiotherapy wbrt, and chemotherapy based on cisplatin. Clinical and epidemiological characteristics of children with germ. Germ cell tumors danafarberboston childrens cancer and. Management of the primary malignant mediastinal germ cell.
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