Some people with a germ cell tumor can be treated with surgery alone, including boys with a testicular germ cell tumor and girls with an ovarian germ cell tumor that is completely removed by surgery. Germ cell cancer is a rare tumor that affects children, teens, and adults. Childhood extracranial germ cell tumors treatment pdq. This free online tool allows to combine multiple pdf or image files into a single pdf document. However, the childrens oncology group cog developed the following system for staging germ cell tumors. Highly specific for germ cell tumors but also seen in ovarian germ cell neoplasms. The management of malignant ovarian germ cell tumors is predicated on the histologic identity of the tumor see fig. Extragonadal germ cell tumors treatment pdqpatient.
The most common site of extragonadal germ cell tumors is the mediastinum 5070%. Sep 10, 2018 extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Overall, approximately 5% to 10% of all germ cell cancers arise in nongonadal sites, particularly in the mediastinum and retroperitoneum. But in adults, the vast majority of these tumors affect men. Extragonadal germ cell tumors treatment depends on the type and can include surgery, chemotherapy, radiation therapy, and stem cell transplant. Germ cell tumors danafarberboston childrens cancer and. In addition, having an undescended testicle increases the odds of a male developing a testicular germ cell tumor.
Germ cell tumors starting within the brain are discussed further under brain tumors. Patients with clinical stage ia dysgerminoma and those with clinical stage iaib grade i immature teratoma require surgical staging to determine the need for adjuvant chemotherapy fig. Dieses kostenlose tool erlaubt es mehrere pdfs oder bilddateien miteinander zu einem pdfdokument zu verbinden. Germ cells normally occur inside the gonads ovary and testis. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. Potential strategies for future treatment of malignant extracranial germ cell tumors on the basis of risk stratification from malignant germ cell international collaborative magic pediatric data set and adult germ cell studies. Enlarge extragonadal germ cell tumors form in parts of the body other than the gonads testicles or ovaries. Revised risk classification for pediatric extracranial germ cell.
Most testicular tumors, in fact, are germ cell tumors. The surgery may be performed by a surgical oncologist, a doctor who specializes in treating tumors using surgery. Children with germ cell brain tumors are treated at danafarberboston childrens through the brain tumor center, one of the largest and most experienced pediatric. It is intended as a resource to inform and assist clinicians who care for cancer patients. Germ cell tumors can spread metastasize to other parts of the body. The term germ cell comes from the term geminate, which means giving life. In humans, germ cells produce specialized cells needed for reproduction. This includes the pineal gland in the brain, the mediastinum area between the lungs, and retroperitoneum the back wall of the abdomen. Types include germinoma, yolk sac tumor, teratoma, embryonal carcinoma, and some types of choriocarcinoma. Because germ cell tumors can vary greatly, and can arise in different parts of the body, they can be difficult to stage. The degree and direction of the differentiation determine the histologic subtype of the gcts, fig 2.
The tumor may then require a biopsy to determine its exact pathology. There are many kinds of germ cell tumors and only some. Intracranial germ cell tumors make up approximately 0. Totipotent stem cells in an undifferentiated state give rise to embryonal carcinomas. Germ cell tumor definition of germ cell tumor by medical. Current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic. They occur in a variety of sites, both gonadal and extragonadal, the latter in midline locations as the sacrococcygeal, retroperitoneal, mediastinal, cervical, and pineal regions tables 2. 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. Pediatric germ cell tumor is the term used to describe malignant cancers of germline cells in patients aged 018 years. Childhood germ cell tumors md anderson cancer center. Germ cell tumors of the brain most commonly involve or are found near the pituitary and pineal glands in the brain.
Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Ovarian germ cell tumors usually affect one ovary and are most common in girls ages 10 to 14. Authors have also contributed articles on the genetics and epigenetics of germ cell tumor development in humans and in the mouse model system. In males, choriocarcinoma is generally considered a nonseminomatous germ cell tumor, which represents less than 5% of all germ cell tumors in males. Between may 2002 and august 2010, a total of 38 patients, who were clinically diagnosed as, but without surgical biopsy confirmation, intracranial germ cell tumors gcts were treated at our. Ovarian germcell tumors represent 1520% of all ovarian tumors. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. Germ cell brain tumor treatment at danafarberboston childrens. Germ cell tumors gct are neoplasms that originate predominately in the ovary and testis. This type of cancer is rare, constituting just 2 percent of all ovarian cancers.
Extragonadal germ cell tumors hollandfrei cancer medicine. The origin of testicular tumors is thought to be atypical cell proliferation, which is termed intratubular germ cell neoplasia, 5, 6. In a growing fetus, germ cells typically move from a site near the middle of the body to the ovaries or testes gonads. Human germ cell tumors are developmental cancers repub. The cells of each type of tumor look different under a microscope. A germ cell tumor gct is a neoplasm derived from germ cells. Extragonadal germ cell tumors are made up of cells that form during the early development of a fetus unborn baby. Most cancerous ovarian germ cell tumors are a type called dysgerminoma, which can spread to other parts of the body.
Therefore, onethird of the tumors have mixed patterns, and tumors arising from germ cells at different stages of their development should exhibit different features 47. Extragonadal germ cell tumorspatient version national. For earlystage testicular cancer we usually recommend radical orchiectomy, a surgery in which we remove the tumor along with the testicle. Testicular germ cell tumours tgcts are the most frequent solid malignant tumour in men 2040 years of age and the most frequent cause of death from solid tumours in this age group.
Primary intracranial germ cell tumors are rare icgcts and usually localized in the pineal and suprasellar regions of the brain. There are different stage descriptions for different types of tumors. Because a marker chromosome interpreted as isochromosome 12p is present in most testicular tumors of germ cell origin, peltomaki et al. Seminomas account for 3040% of these tumors, and nonseminomatous germ cell tumors nsgcts account for 6070%.
We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. Group united kingdom between 1985 and 2009 were merged. Two of the most common germ cell tumors are seminoma and nonseminoma. A teratoma is a tumor that may contain several different types of tissue, such as hair, muscle, and bone. Pediatric and adolescent extracranial germ cell tumors. Diagnosis of germ cell tumors begins with a thorough health history and a comprehensive physical examination. Consider testicularsparing approach for prepubertal boys with likely benign lesions negative tumor markers. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac tumor, and malignant mixed germ cell tumors mmgcts. Germ cell tumours list of high impact articles ppts. 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. A family history of germ cell tumors also increases the chance that someone will develop a germ cell tumor.
If you just found out that you or your child has a germ cell tumor, you may have a lot of questions about what it means and what comes next. A germ cell tumor may arise within the gonads in the ovary or testis. Germ cell tumors can cause a variety of symptoms in children based primarily on the size and location of the tumor. Keep in mind that the symptoms of a germ cell tumor may resemble other more common conditions or medical problems. Extragonadal germ cell tumors treatment general information about extragonadal germ cell tumors extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. In comparison with normal dna, tumor dna of 18 patients showed increased. Germ cell tumors also may arise in extragonadal sites, reflecting the fact that germ cells travel to diverse areas of the body, such as the chest, abdomen, and brain. In some cases, extragonadal choriocarcinoma occurs in midline locations, such as the mediastinum.
The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system. Handbook for children with germ cell tumors american pediatric. There are 2 types of staging for germ cell tumors, based on whether the patient has had surgery yet. Although not as difficult to deal with as malignant tumors, benign germ cell tumors can cause problems because of their size. Introduction primary germ cell tumors gcts arise by the malignant transformation of primordial germ cells primary gcts of testes constitute 95% of all testicular tumors infrequently, gcts arise from an extragonadal site. 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.
Germ cell tumors of the brain danafarberboston childrens. Treatment for children with extracranial germ cell tumors gct may involve surgical resection followed by monitoring or chemotherapy before. Benign germ cell tumors include certain kinds of teratomas. Some testing will be required and will include blood work cbc, afp, hcg, an xray, an ultrasound, a ct scan, an mri, and a bone scan. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. In rare cases, childhood germ cell tumors can be passed down from one generation to the next. Extragonadal germ cell tumors treatment pdq patients.
Germ cell tumors may develop in the brain, the testes, the ovaries, or elsewhere in the body. Pdf zusammenfugen online pdf dateien zusammenfugen. Extragonadal germ cell tumors form in parts of the body outside the gonads. Extragonadal germinal cell tumors eggcts are rare tumors that predominantly affect young males. Intracranial germ cell tumors radiology reference article. To risk stratify malignant extracranial pediatric germ cell tumors gcts.
Germ cell tumors arise predominantly from within the testis, but an important subset of germ cell tumors are extragonadal in origin. Germ cell tumors gcts are masses of tissue formed by the type of immature cells which, in a healthy body, would develop into mature eggs in a female or sperm in a male. These germ cells travel into the pelvis as ovarian cells or into the scrotal sac as testicular cells. While these tumors typically start in the testicles they also occasionally arise in the abdomen, chest, or other areas of the body, even if theres no evidence of cancer in or near the testicles. Tumors of germ cell origin only very uncommonly arise in extragonadal sites. Extragonadal germ cell tumors can be benign noncancer or malignant. Egcts are classified as seminomas or nonseminomas of which there are several subtypes. The tumor has been entirely removed, and tumor markers are normal. When combined with lifelong monitoring, this can be the only treatment needed for earlystage germ cell tumors that havent spread beyond the testicle. We have diagnosed ten primary malignant extragonadal gct arising in the mediastinum, retroperitoneum, liver, and sacrococcygeal region by fine. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patients prognosis, which is the chance of recovery. Germ cell tumors are a varied group of benign and malignant neoplasms derived from primordial germ cells 110, 1215 table 2.
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