Retroperitoneal extragonadal germ cell tumor without. Primary extragonadal malignant germ cell tumors emgcts are rare and characterized by the location in the midline of the body, including mediastinum, cns, retroperitoneum and coccyx. Autopsy findings in 20 patients with extragonadal germ cell tumors arising in the anterior mediastinum revealed only one case ofa testicular primary and one. Pdf benign and malignant germ cell tumors of the mediastinum. Cureus nonseminomatous extragonadal germ cell tumor. The most common extragonadal sites are the mediastinum and retroperitoneum. Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1,2. Extragonadal germ cell tumors are often associated with klinefelter syndrome. The malignants are divided in two types, nonseminomatous and seminomas. Incidentally discovered primary mediastinal germ cell tumor. They may begin to grow anywhere in the body, but usually form in the pineal gland in the brain, the chest, the lower part of the spine, or the abdomen.
Malignant mediastinal germ cell tumors of various histologies were first described as a distinct clinical entity approximately 50 years ago 1. Gonadal germ cell tumor is generally the most common germ cell tumor type and constitutes 90% of germ cell tumors, whereas the mediastinum is the second most frequently affected area ahead of other extragonadal areas, which include retroperitoneum, the sacrococcygeal area, and the. Among these, a common form is teratoma with endodermal sinus tumor. Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors article pdf available in jnci journal of the national cancer institute 921. Extragonadal germ cell tumors of the lung are among the rarest tumors encountered in pathology. Extragonadal germ cell tumors occur much more commonly in males than in females and are usually seen in young adults. Germ cell tumors commonly occur in the gonads, but the anterosuperior mediastinum is the most common site of extragonadal germ cell tumors in adults.
One new case is diagnosed annually for every 1 million people in the united states. Extragonadal germ cell tumors treatment pdqpatient. Extragonadal germ cell tumors comprise a rare subgroup of germ cell neoplasms, most of which arise in the anterior mediastinum. Primary germ cell tumors in the mediastinum wiley online library. Surgical removal of the tumor if it is benign teratoma. Germ cells normally occur inside the gonads ovary and testis. They account for 25% of all germ cell malignancies,1 but their clinical behaviors are somewhat different. May 01, 2015 primary extragonadal malignant germ cell tumors emgcts are rare and characterized by the location in the midline of the body, including mediastinum, cns, retroperitoneum and coccyx. Extragonadal germ cell tumor surgical pathology criteria. Feb 20, 2015 germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in the testes or ovaries. We strongly recommend that you talk with a trusted. Primary extragonadal germ cell tumors are rare, and 60% of such cases are seminomas. Yolk sac tumor, embryonal carcinoma, choriocarcinoma, and combined nonteratomatous germ cell tumors of the mediastinum a clinicopathologic and immunohistochemical study of 64 cases.
The disease is metastatic extragonadal germ cell tumor, not lung cancer. Mediastinal and extrapulmonary origins must be excluded before a diagnosis of germ cell tumor of the lung can be confirmed. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Extragonadal germ cell tumors of the mediastinum and retroperitoneum. Treatment options for benign extragonadal germ cell tumors is surgery. We present an overview of mediastinal extragonadal germ cell tumors based on our experiences with 17 patients. The exact histogenesis has not been elucidated, but is. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo.
Noncentral nervous system extragonadal gcts are found in a variety of anatomic locations, but most commonly affect the mediastinum. The treatment of the extragonadal germ cell tumor is based on the stage, type, and location of the tumor. May 28, 2018 extragonadal germ cell tumors is a fatal condition that can result in death, if early diagnosis and treatment is not provided. Burkitts leukemia after treatment of primary mediastinal nonseminomatous germ cell tumor. The most common histologic type of mediastinal germ cell tumor is mature teratoma, which is typically asymptomatic and incidentally discovered. Extragonadal germ cell tumors gcts are relatively uncommon, but represent 1% to 5% of all gcts. More than half of mediastinal germ cell tumors are mature teratomas. Extragonadal germ cell tumors are as curable as testicular germ cell tumors when treated with intensive cisplatincontaining combination chemotherapy regimens, and surgical resection when necessary. Einhorn department of medicine, indiana university.
In young children, extragonadal germ cell tumors tend to occur primarily in the presacral area. Germ cell tumors includes pediatric and adult patients with germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, and neck the following should not be reported using this protocol. In adults, extragonadal germ cell tumors tend to be in the mediastinum. Start here to find information on extragonadal germ cell tumors treatment. Mediastinal germ cell tumor an overview sciencedirect topics. The mediastinum is the most common location of extragonadal germ cell tumors. The most common extragonadal site of primary germ cell tumors is the anterior mediastinum. The following prognostic groups are used for extragonadal germ cell tumors. Protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites. Extragonadal germ cell tumours clinical practice guidelines. Germ cell tumors arising in the mediastinum are uncommon primary tumors.
A study of patients with primary mediastinal germ cell. Extragonadal germ cell tumors in children are uncommon and occasionally present with atypical features j indian assoc pediatr surg 2012. Klinefelter syndrome 47 xxy is the only known predisposing factor. Because they are rare and biologically distinct from testis cancer, their natural history and optimal. 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. Extragonadal means outside of the gonads sex organs. Chemotherapy for larger tumors or tumors that have spread. Emgcts present with different clinical and biologic characteristics in different tumor locations. Mediastinal germ cell tumor an overview sciencedirect. 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. Pathology of postchemotherapy residual masses reveals necrosis in 24%, teratoma in 45%, sarcoma in 5%, and viable germ cell cancer in 26%. Klinefelter syndrome and mediastinal germ cell tumors.
At about four weeks, the earliest germ cells in the growing fetus migrate from their point of origin to the gonadal area the area of the sex organs. Extragonadal germ cell tumors can take on a variety of tumor types. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. Extragonadal germ cell tumor symptoms, diagnosis and. Unlike benign germ cell tumors of the mediastinum, malignant mediastinal tumors are usually symptomatic at the time of. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. The imaging characteristics of these tumors are nonspecific, and the definitive diagnosis of extragonadal germ cell tumor requires a biopsy. They most commonly occur in the gonad but occasionally elsewhere. Extragonadal germ cell tumors have a variable clinical course, with the potential for aggressive behavior and widespread metastases. Male germ cell tumors gcts usually arise from the testis. To characterize the clinical and biologic features of extragonadal germ cell tumor egct and to determine the overall outcome with currently available treatment strategies. Sometimes, a group of germ cells grows in a way thats not normal. Pure mediastinal seminomas are usually curable with chemotherapy alone, while mature teratomas are treated with surgery alone.
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. Intraoperative deaths occurred in 6% of patients, 90% of which were due to respiratory failure. Extragonadal germ cell tumors are rare and vary greatly in their clinical presentations. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Malignant tumors can be either seminoma aka germinoma in females or nonseminoma embryonal carcinoma, immature teratoma, endodermal sinus tumor aka yolk sac tumor, choriocarcinoma, and mixed germ cell tumors.
E xtragonadal germ cell tumors egct are rare and have distinct clinicopathologic features. Only about 5% of the gct are extragonadal and it is estimated that approximately 3% of gct are primarily mediastinal. Extragonadal germ cell tumors account for 15% of all germ cell tumors. By continuing to use our website, you are agreeing to our use of cookies. Good prognosis if 14 years old or less case reports. Extragonadal germ cell tumor cancer information ohc. The most widely accepted theory suggests that extragonadal germ cell tumors arise from primordial germ cells misplaced during their migration to gonads. It is a slowgrowing tumor that typically occurs in young patients and is.
Cancers that develop from germ cells in other parts of the body are rare. Extragonadal germ cell tumor of the mediastinum d62. The medical name for germ cell tumours that develop outside of the ovaries or testicles is extragonadal germ cell tumour eggct. They are aggressive neoplasms and can arise virtually anywhere, but typically the site of origin is in the midline mediastinum, retroperitoneum, or pineal gland. Diagnosis, staging, and clinical characteristics of the patient with. Malignant mediastinal germ cell tumor is a rare disease and represents only 1% to 4% of all mediastinal tumors.
Management of the primary malignant mediastinal germ cell. The extragonadal germ cell tumors egcts represent a unique entity, and as such require specialized management. Primary mediastinal germ cell tumors pmgcts are rare, which often makes them difficult to treat. 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. Cap extragonadal germ cell tumor protocol revision history. Most germ cell tumors arise from the testicles and often are selfdiagnosed. The term dermoid cyst is commonly applied due to the frequent expression of the ectodermal component of the teratoma. After a germ cell tumor is diagnosed, doctors need to assess how.
Multimodality treatment of germ cell tumors of the mediastinum. Extragonadal teratomas represent 25% of adult germ cell tumours which can be found in any midline structure including retroperitoneum and mediastinum albany and einhorn 20. The majority of mediastinal germ cell tumors are benign with mature teratoma as the most common histologic type. Their histogenesis is under dispute, but their cellu we use cookies to enhance your experience on our website. Blood tests, physical exams and scans of the abdomen, chest and pelvis, are used to provide doctors with more information about the tumor. Extragonadal germ cell tumors involving the mediastinum. Extragonadal germ cell tumorspatient version national. Treatment of seminoma extragonadal germ cell tumors may include the following. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Mediastinal extragonadal germ cell tumors sciencedirect. The metastatic tumor is the same type of tumor as the primary tumor. Their morphology varies widely and includes mature teratoma, immature teratoma, seminoma, yolk sac tumor, embryonal carcinoma, choriocarcinoma, and mixed gcts.
They are usually found along the midline of the body and are most common in the mediastinum, thymus, retroperitoneal organs, and pineal gland. Extragonadal germ cell tumors form in parts of the body outside the gonads. Extragonadal germ cell tumors are tumors that account for 20% of mediastinal masses. The anterior mediastinal germ cell tumors gcts are the most common egct. Primary malignant germcell tumors of the anterior mediastinum in. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant. Mediastinal extragonadal germinal cell tumors gcts are an uncommon entity in cuba and worldwide. The clinical course is variable, with a potential for aggressive behavior and widespread. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Chemotherapy andor surgery is the mainstay of treatment of these primary mediastinal germ cell tumors. However, aberrant migration of primordial germ cells may permit these tumors to develop in an extragonadal location, most commonly the anterior mediastinum 15% of mediastinal. Adult patients with extragonadal germ cell tumors protocol applies to germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, neck, and intracranial sites.
Since this is a rare type of tumor they must also differentiate it from other types of tumors. Researchers reported a 25year single institution experience of postchemotherapy surgery for 158 patients with mediastinal primary nonseminoma germ cell tumours gcts. Extragonadal germ cell tumors egct represent only 25% of adult germ cell malignancies. Extragonadal germ cell tumor genetic and rare diseases.
Surgery for extragonadal germ cell tumor of mediastinum youtube. Survival outcomes for men with mediastinal germcell. Extragonadal germ cell tumors of the mediastinum and. It is often difficult to determine whether or not the tumors are in fact extragonadal in origin. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12.
Radiation therapy for small tumors in one area, followed by watchful waiting if there is tumor remaining after treatment. The most common germ cell tumor, particularly in children, is benign teratoma. Germ cell tumors occur most frequently in the gonad but can rarely occur in extragonadal locations, usually in or near the midline. Surgery for extragonadal germ cell tumor of mediastinum. Extragonadal germ cell tumors eggcts are uncommon neoplasms with peculiar molecular and pathological features. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. You can also get a germ cell tumor in the brain, chest, belly. Emgcts were found to occur predominantly in males, especially for mediastinal mgcts. There are many tumor markers that doctors can use to diagnose an extragonadal germ cell tumor. Primary germ cell tumors gct of the mediastinum are relatively rare and represent approximately 1015% of mediastinal tumors. Procedure biopsy consider extragonadal germ cell biopsy protocol tumor type testicular germ cell tumors consider the testis protocol. Extragonadal germ cell tumors clinical presentation. Herein, we examined patients with pmgcts who underwent multimodal treatment.
Germ cell tumors are a heterogeneous group of lesions originating from primitive germ cell layers ectoderm, mesoderm, and endoderm and normally affect the gonads. Sep 10, 2018 the most common site of extragonadal germ cell tumors is the mediastinum 5070% followed by the retroperitoneum 3040%, the pineal gland 5%, and the sacrococcygeal area less than 5%. Fossa, jorg beyer, jorg pont, lothar kanz, lawrence einhorn, jorg t. 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.
The mediastinum part of the chest between the breastplate and the spinal column the brain. Extragonadal germ cell tumors treatment pdq patients. Response rates and survival are similar to patients with advancedstage testicular germ cell tumors. Extragonadal gcts are rare and do not have a primary testicular involvement. Germcell tumors of the mediastinum, postmortem findings.
Extragonadal malignant germ cell tumor of the lung the. For example, if an extragonadal germ cell tumor spreads to the lung, the tumor cells in the lung are actually cancerous germ cells. Radiographically, these tumors appear as rounded, often lobulated masses. While the tumors can occur in the mediastinum, thymus, retroperitoneal organs and pineal gland, seminoma originating in the prostate tissue is extremely rare. Protocol for the examination of resection specimens from. Extragonadal gcts typically arise in midline locations, and specific.
A germ cell tumor gct is a neoplasm derived from germ cells. Cytology of fine needle aspirates of primary extragonadal germ cell tumors. Extragonadal germ cell tumors are extremely rare and are typically located in midline structures and, especially, the anterior mediastinum. A study of patients with primary mediastinal germ cell tumors. Mediastinal germ cell tumors are tumors that derive from germ cell rest remnants in the mediastinum.
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