Following chemotherapy or radiation therapy rt for advanced germ cell tumor gct, all patients should undergo imaging, utilizing either highresolution computed tomography ct or magnetic resonance imaging mri. Pdf benign and malignant germ cell tumors of the mediastinum. In adults, approximately % of mediastinal tumors are germ cell tumors 20% of mediastinal tumors and cysts. Report of 5 stage iii cases, abstract among 15 patients with primary mediastinal nonseminomatous germ cell tumors experienced during the past 12 years, 5 were diagnosed with germ cell tumors with somatictype malignancy. Mediastinal and other extragonadal germ cell tumors were initially thought to represent isolated metastases from an inapparent gonadal primary site. Any anterior mediastinal mass figures 1, 2 must be considered suspect for germ cell tumor, especially in a young male. Extragonadal germ cell tumors gcts are mainly midline in location, with mediastinum being the most common site. Feb 19, 2014 furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors. Germ cell cancer is a malignant neoplasm that originates from germ cells.
Primary mediastinal germ cell tumor how is primary mediastinal germ cell tumor abbreviated. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. However, there is now abundant clinical evidence to substantiate. Primary mediastinal malignant gct pmmgct is a rare cancer contributing mediastinal tumors. Please contact me if you are still interested in information. Cytology of mixed germ cell tumor with mediastinal metastasis.
Extragonadal germ cell tumors egct represent only 25% of adult germ cell malignancies. May 24, 2015 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. Also, a v ats appr oach can be use d if the tumor is not readily. Histopathological examination of the orchiectomy material resulted in the diagnosis of mixed germ cell tumor 60% mature teratoma and 40% yolk sac tumor. The recently published igcccg2 prognostic score for relapsed disease includes 7 factors histology.
We evaluated the role of the surgical treatment and the effect of multimodality treatment strategy for patients with pmmgct. Mediastinal germ cell tumor with somatictype malignancy. Successful management of brain metastasis from malignant germ cell tumours with standard induction chemotherapy. All patients received treatment with cisplatincontaining or carboplatincontaining therapy as their. The possibility of metastases from testicular or ovarian primary tumor must be excluded before a. These germ cells travel into the pelvis as ovarian cells or into the scrotal sac as testicular 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.
Nongerminomatous germ cell tumors secrete chemicals into the spinal fluid and bloodstream and require more intensive treatment than pure germ cell tumors. Treatment strategies and prognostic factors of patients with. A mediastinal germ cell tumor mimicking an ectopic pregnancy article pdf available in journal of gynecologic oncology 224. In addition to physical examination and routine laboratory studies, initial evaluation should include ct of the chest and abdomen, and determination of serum levels of hcg and alphafetoprotein. The diagnosis of a mediastinal germ cell tumor should be considered in all young males with a mediastinal mass. Germ cell neoplasms and other malignancies of the mediastinum. Primary mediastinal germ cell tumors pmgcts account for less than 5% of germ cell malignancies and are more common in men but also occur in women. The clinical manifestations, diagnosis, and staging are presented separately, as are more. Because they are rare and biologically distinct from testis cancer, their natural history and optimal. Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1,2. Extension and initial remission rate are independent prognostic factors. Hematologic neoplasia associated with primary mediastinal germcell tumors. Nov 18, 2015 seminoma is the second most common mediastinal germ cell tumor.
Examples of treatable tumors arising in the mediastinum include extragonadal germ cell tumors, malignant thymoma, hodgkins disease, nonhodgkins lymphoma, and poorly differentiated carcinoma. 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. Mediastinal germ cell tumours develop in germ cells. Treatment strategies and prognostic factors of patients. There is an association between mediastinal germ cell tumors and klinefelter syndrome. Extragonadal germ cell tumorshealth professional version. However, a small percentage of germ cell tumors arise in extragonadal sites. I came upon your question as to the survival rate of germ cell cancer. Surgical management of primary mediastinal germ cell tumors. Germ cells normally occur inside the gonads ovary and testis. Germ cell tumors of the brain danafarberboston childrens. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. These are the cells in the body that develop into sperm and eggs.
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. Mediastinal germ cell tumor an overview sciencedirect. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Gct of bone is a benign, but potentially aggressive. Germ cell tumors danafarberboston childrens cancer and. A total of 54 patients with pmmgct in a followup from 1990 to 2009. Malignant mediastinal germ cell tumors of various histologies were first described as a clinical entity approximately 50 years ago. In addition to physical examination and routine laboratory studies, initial evaluation should include ct of the chest and abdomen, and determination. Nonseminomatous germ cell tumor, which is a rare entity, representing less than 0.
For detailed discussion please refer the articles listed at the end of this page. Giant cell tumor gct is a benign neoplasm that usually involves the metaphysis and epiphysis of long bones. Mixed germ cell tumor of mediastinumlung masquerading as. Giant cell tumor of the thoracic spine presenting as a. Pdf ct of primary germcell tumors of the mediastinum. Germ cells are cells that develop in the embryo which later becomes eggs and sperm in the ovaries and testes respectively. Initial therapy of early stage testicular germ cell tumors gcts is based on histology and tumor extent. Pdf a mediastinal germ cell tumor mimicking an ectopic.
However, discrepancies between the genetic alterations and immunoprofiles of mediastinal and testicular seminomas have been reported, raising the question of whether techniques that are useful in the diagnosis of gonadal seminoma are applicable to its mediastinal counterpart. Chemotherapy combined with local therapy like surgery or radiotherapy is a reasonable treatment strategy recommended. The imaging characteristics of these masses differ by whether the primary tumor is a seminoma or a nonseminomatous germ cell. Extragonadal germ cell tumor chest cancer survivors. Pembrolizumab in treating patients with incurable metastatic, recurrent, or platinum refractory germ cell tumors. These tumors account for approximately 10% of all primary mediastinal neoplasms. 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. Printable germ cell tumors of the mediastinum thymus. Mediastinal germ cell tumor an overview sciencedirect topics.
Concurrent mediastinal germcell tumour and haematological malignancy. Technique of mediastinal germ cell tumor resection kenneth a. Three years ago, my son, age 27 was diagnosed with a massive tumor in the chest wall. They are thought to arise from extragonadal germ cells. Mediastinal germ celltumorsmediastinal germ celltumors primary extragonadal germ cell tumors compriseprimary extragonadal germ cell tumors comprise 2% to 5% of all germ cell tumors2% to 5% of all germ cell tumors approximately two thirds of these tumors occur inapproximately two thirds of these tumors occur in the mediastinumthe mediastinum the. Even though metastatic lesions are mostly seen in the posterior mediastinum, our findings reveal that specimens obtained with fnac from the anterior mediastinum bear discohesive, pleomorphic. Mar 15, 20 hi everyoneim a ewbee here and also a cancer survivor of 20 some years now our oldest son has mediastinal germ cell tumors, a rare version of cancer and he has it in the chest cavity as well as in his lymph glandshe is in a cancer treatment centre he had devoloped some fluid around his heart. Survival outcomes for men with mediastinal germcell tumors. The later is then divided further according to histology. 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. Clinical practice guideline gu001 version 7 page 2 of 23 background testicular germ cell tumours gcts account for about 1% of all new cancer cases in men agestandardized incidence rate of 5. Association between mediastinal germ cell tumors and hematologic malignancies. Extragonadal germ cell tumors are rare and account for only a small percentage of all germ cell tumors. Mediastinal germ cell tumors are not related to thymomas.
The majority of this chapter is devoted to a discussion of primary mediastinal germ cell tumors, a unique and important group of highly treatable. 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. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts. Hematologic neoplasia associated with primary mediastinal germ cell tumors.
Fiftytwo patients underwent surgical resections, while. 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. The diagnostic needle core biopsy revealed mixed germ cell tumour with possible combination of embryonal. Delayed effects of whole brain radiotherapy in germ cell tumor patients with central nervous system metastases. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both.
Primary mediastinal germcell tumors pmgcts account for less than 5% of germcell malignancies and are more common in men but also occur in women. With this, the neoplasm can metastasize and affect other areas of the body. An international germ cell tumor prognostic classification has been developed based on a retrospective analysis of 5,202 patients with metastatic nonseminomatous germ cell tumors and 660 patients with metastatic seminomatous germ cell tumors. Pure seminoma is sensitive to radiotherapy and the prognosis is good.
Mediastinal germ cell tumor medical condition youtube. 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. They are much less common than germinal tumors arising in the testes, and account for only 1 to 5% of all germ cell neoplasms. Although the histomorphological features of mediastinal gcts are similar to those of gonadal gcts, the biological behavior, clinical. 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. The condition can produce serious impairment of cardiopulmonary function and is fatal if not treated. Find evidencebased information on extragonadal germ cell tumors treatment. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. The most common site for an extragonadal germ cell tumor is the anterior mediastinum. Germ cell tumor classification radiology reference. Pembrolizumab in treating patients with incurable metastatic.
The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Mar 16, 2020 mediastinal germ cell tumors are not related to thymomas. Management of the primary malignant mediastinal germ cell. Vinblastine plus ifosfamide plus cisplatin as initial salvage therapy in recurrent germ cell tumor. The gctstm reportedly accounts for 11% to 29% of all cases of mediastinal gct in adults 2, 3, 4 and is mostly diagnosed from. Extragonadal germcell tumors comprise a rare subgroup of germcell neoplasms, most of which arise in the anterior mediastinum. Although the spine is the fourth leading location of gct, most of these lesions occur in the sacrum, followed by the thoracic, cervical and lumbar vertebrae 1. Germ cell tumor classification dr jeremy jones and assoc prof frank gaillard et al. They are always associated with the thymus gland and may even be fully contained within it when small. Mediastinal germ cell tumors are tumors that derive from germ cell rest remnants in the mediastinum. Ikdahl t, josefsen d, jakobsen e, delabie j, fossa sd. Malignant germ cell tumors of the mediastinum are uncommon, representing only 3 to 10% of tumors originating in the mediastinum. Eligible subjects must have received initial cisplatinbased combination therapy, such as bleomycinetoposidecisplatin bep, cisplatinetoposide ep, etoposideifosfamidecisplatin vip, or similar regimens and demonstrated progression following the administration of at least one salvage regimen for advanced germ cell neoplasm, such as.
Primary mediastinal germ cell tumor listed as pmgct. Germ cell tumors danafarberboston childrens cancer. Mediastinal germ cell tumors mgcts account for 19%25% of all mediastinal tumors and 3%6% of all gcts in pediatric age 1. Typically divided into seminomas versus nonseminomatous germ cell tumors teratomas mature or. However, the true incidence of these tumors may be higher than originally thought because of failure to diagnose them properly. Germ cell tumors make up about 20% of mediastinal tumors and cysts. There are several subtypes of nongerminomatous germ cell tumor, including teratomas, choriocarcinomas, endodermal sinus tumors yolk sac tumors, embryonal carcinomas and mixed tumors.
In this report, we describe how a mediastinal mixed germ cell tumor was initially mistaken and reported as hemangioma due to the fact that only a minute portion of the 16. These extragonadal germ cell tumors histologically contain the same components as their gonadal counterparts, but may have different biologic behaviors, clinical characteristics and. Pdf primary mediastinal germ cell tumors in children and. The mediastinum is the area in the centre of the chest, between the lungs. Mediastinal germ cell tumors gcts are rare and encompass less than one fifth of all mediastinal neoplasms in adults and children. Germ cell tumors are classified into two broad groups. Prior to this, he reports being in his usual state of good health. An overview of therapy for men with testicular gcts is presented here. Seminoma is the second most common mediastinal germ cell tumor.
Conclusions primary mediastinal germ cell tumor is rare with a dominant frequency in young male patients. Primary malignant mediastinal germ cell tumor pmmgct is rare and sometimes the prognosis of the patients with pmmgct is not very satisfactory. Although all pmnsgct arise in the anterior mediastinal compartment, the exact location, size, and degree of adjacent. Mediastinal definition of mediastinal by medical dictionary. A germ cell tumor gct is a neoplasm derived from germ cells. Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. Kesler, md t he anterior mediastinum represents the second most common site of germ cell tumor origin. We present a case of 16yearold male, who was referred from private centre for dyspnoea, fatigue, and orthopnea. The most common site for germ cell tumors to arise is within the gonadal tissue. The possibility of metastases from testicular or ovarian primary tumor must be excluded before a diagnosis of primary mediastinal germ cell tumor is made. 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. A germ cell tumor with somatictype malignancy gctstm is a germ cell tumor gct accompanied by somatictype malignant stm components, such as sarcoma, carcinoma, or both, according to the most recent histologic classification of the world health organization. The most common extragonadal sites are the mediastinum and retroperitoneum. The most common type of mixed germ cell tumors demonstrates a combination of teratoma, embryonal carcinoma, and yolk sac tumor.
Errors in diagnosis are not uncommon and can result in mismanagement of a potentially curable patient. Successfully treating your childs germ cell tumor depends on where the tumor is located and whether it is benign or malignant. Primary mediastinal germ cell tumor how is primary. Among these, a common form is teratoma with endodermal sinus tumor. Malignant germ cell tumors of the mediastinum mayo clinic. Due to the complexity of the disease of these patients, referral to experienced high volume centers is strongly recommended. Extragonadal germ cell tumors comprise a rare subgroup of germ cell neoplasms, most of which arise in the anterior mediastinum. The clinical manifestations, diagnosis, and staging are presented separately, as are more detailed discussions of specific clinical scenarios.