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