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. Request pdf an overview of testicular germ cell tumors more than 90% of testicular. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. To stage the tumor, doctors will need to know the size of the tumor, if it has spread metastasized and if it has affected lymph nodes and other tissues. Since 1983, chemotherapy has been delivered in japan as an adjuvant therapy in patients with intracranial nongerminomatous germ cell tumors. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. 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. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Differentiation of seminoma from a clear cell malignant sertoli cell tumor may be more difficult on. Treatment may also include chemotherapy or radiation therapy. Germ cell tumors can spread metastasize to other parts of the body. The group of germ cell neoplasms associated with gcnis includes seminoma, embryonal carcinoma, postpubertal type yolk sac tumor, postpubertal type teratoma and choriocarcinoma. If the tumour is benign and can be removed completely then no further treatment is needed. Types of surgery include hysterectomy and removal of one or both ovaries and fallopian tubes bilateral salpingooophorectomy.
The later is then divided further according to histology. The treatment of nonseminomatous germ cell tumors is more controversial. Get a printable copy pdf file of the complete article 3. Surgery is the most common treatment of ovarian germ cell tumor.
A teratoma is a tumor that may contain several different types of tissue, such as hair, muscle, and bone. There are different stage descriptions for different types of tumors. Most chapters are focused on testicular germ cell tumors whose incidence has been increasing in young males. Pdf malignant ovarian germcell tumours researchgate. Germ cell tumors health encyclopedia university of. But germ cells can sometimes be left behind in other parts of the body from when you developed in the womb. 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. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. In teenaged boys, testicular tumors may be more difficult to detect if parents and teens are uncomfortable discussing such intimate matters. Malignant germ cell cancers of the ovary include dysgerminomas and nondysgerminomas, which include immature teratomas, embryonal cell carcinoma, yolk sac tumors, primary ovarian nongestational choriocarcinomas, polyembryoma, and mixed germ cell tumors. Pure seminoma is sensitive to radiotherapy and the prognosis is good.
Germ cell cancer is a rare tumor that affects children, teens, and adults. Childhood central nervous system cns germ cell tumors form from germ cells. Fiftysix of fiftyeight entered patients with measurable and progressive recurrent germ cell tumors of. Pluripotent embryonic cells mismigrate and give rise to gcts. Malignant germ cell tumors include several types of cancer, such as immature teratoma, yolk sac tumor and choriocarcinoma. Germ cell tumors begin in the reproductive cells egg or sperm of the body. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. Ovarian germ cell tumors represent 1520% of all ovarian tumors. The most common sites for metastasis are the lungs. Sometimes, a group of germ cells grows in a way thats not normal. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts.
Dec 10, 2012 germ cell tumors are a varied group of benign and malignant neoplasms derived from primordial germ cells 110, 1215 table 2. However, a germ cell brain tumor can spread to other parts of. This usually involves surgery and sometimes chemotherapy anticancer therapy. To risk stratify malignant extracranial pediatric germ cell tumors gcts. Combined chemotherapy and radiation therapy for cns germ cell. Rnabinding protein lin28 is a marker for primary extragonadal germ cell tumors. After a germ cell tumor is diagnosed, doctors need to assess how advanced it is, which is called staging the tumor. Management of female malignant ovarian germ cell tumours. Hollern,5,32 linghua wang,6,7,32 reanne bowlby,8,32 satish k. Germ cell neoplasms are classified on the basis of the presence of germ cell neoplasia in situ gcnis in the adjacent parenchyma.
This book provides, for the first time, a detailed, uptodate, holistic approach to the pathology and biology of germ cell tumors. You can also get a germ cell tumor in the brain, chest, belly. This includes the pineal gland in the brain, the mediastinum area between the lungs, and retroperitoneum the back wall of the abdomen. Enlarge extragonadal germ cell tumors form in parts of the body other than the gonads testicles or ovaries. Mediastinal germ cell tumor an overview sciencedirect topics. Sertoli cell tumor an overview sciencedirect topics. Most often, patients present with a painless nodule or swelling of the testis. Germ cell tumors gcts are rare in childhood, representing only. Nevertheless, these tumors remain highly significant, as the morbidity and mortality in this age group.
Some germ cell tumors occur in other areas of the body, such as the abdomen, brain and chest, though it. Nov 14, 2018 the american joint committee on cancer ajcc tnm classification and the international federation of gynecology and obstetrics figo staging system for germ cell tumors are listed below see tables 1 and 2. Testicular tumors knowledge for medical students and. Most germ cell tumors gcts arise in the gonads, mainly in the testis. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. Combining gemcitabine, cisplatin, and ifosfamide gip is active in patients with. Hale, carlos rodriguezgalindo, ha dang, thomas olson, matthew j. Cao d, liu a, wang f, allan rw, mei k, peng y, et al.
This document addresses germcell tumours and sex cordgonadal stromal tumours. Sometimes the germ cells travel to or from other parts of the. Mediastinal germ cell tumors are very rare, of these about 90% are seen in the pericardium. The large cell calcifying sertoli cell tumor is a clinically and histologically distinct entity with a higher incidence of multifocality and hormonal activity. Most germ cell tumors form in the testes or ovaries. The germ cells which are present outside the gonads are the birth defects during the development of embryo. While sharing many similarities with male germ cell tumours gcts, a group of. A typical seminoma with clear cells and tubular pattern may mimic a sertoli cell tumor. Malignant germ cell tumors represent 5% of ovarian tumors. Ovarian neoplasms coelomic epithelium germ cell specialized gonadal stroma nonspecific mesenchyme metastatic tumors 5070% 1520% 510% 510% 510%. Many factors also affect the outlook for a good outcome. Belly pain and swelling caused by tumor bathroom troubles a hard time pooping or holding in your pee, if the tumor is near your pelvis breast growth, pubic hair, or vaginal bleeding at an earlier age than normal.
There are 2 types of staging for germ cell tumors, based on whether the patient has had surgery yet. Germ cell tumors in adolescents and young adults jco oncology. Learn about treatment options for ovarian germ cell tumors. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Their imaging characteristics are nonspecific, and extragonadal germ cell tumors should always be included in the differential diagnosis of a midline anterior mediastinal or retroperitoneal mass. Because germ cell tumors can vary greatly, and can arise in different parts. 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. Yolk sac tumors, also called endodermal sinus tumors are usually cancer. Pdf primary intracranial germ cell tumors shagufta. Many factors go into a doctors decision about how to best treat germ cell tumors. Germ cell tumors is a neoplasm which obtained from germ cells. Based on our clinical experience, we undertook a multiinstitutional. Ogcts arise primarily in young women between 10 and 30 years of age and represent 70.
Unlike benign germ cell tumors of the mediastinum, malignant mediastinal tumors are usually symptomatic at the time of diagnosis. Feb 19, 2014 furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors. Combined chemotherapy and radiation therapy for cns germ. Germ cells are special types of cells that are present as the fetus unborn baby develops. Most mediastinal malignant tumors are large and cause symptoms by compressing or invading adjacent structures, including the lungs, pleura, pericardium, and chest wall. The drugs that are commonly used for treating germ cell tumors include bleomycin blenoxane, cisplatin platinol, and etoposide vepesid, toposar. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. Among intracranial germ cell tumors, nongerminomatous tumors have proved refractory to conventional treatment with surgery and irradiation. Management of the primary malignant mediastinal germ cell. Germ cell tumors are growths that form from reproductive cells. Revised risk classification for pediatric extracranial germ. This chapter is based on results of germ cell tumors from the eurocare european cancer registrybased study on survival and care of cancer patients. These groups merged 25 years of clinical data on paediatric. Ovarian germcell tumors represent 1520% of all ovarian tumors.
However, there are a few factors that increase the risk failed testicular descent. Cap approved pediatric extragonadal germ cell tumor germcell 3. When germ cell tumors are found mixed with teratomas, one can infer a transformation between the different cell types i. Extragonadal germ cell college of american pathologists.
These cells usually become sperm in the testicles or unfertilized eggs in the ovaries as the child matures. Ovarian germ cell tumors treatment pdqpatient version. The exact causes of testicular germ cell tumours are unknown. About 23% of all ovarian cancers are germ cell tumors. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Summary of changes this is a major revision to the protocol. Germ cell tumors of the gonads have a better prognosis than germ cell tumors outside the gonads extragonadal. Intracranial seminoma commonest histology 6070% of all germ cell tumors. Testicular germ cell tumor presenting with spontaneous complete or partial regression j ultrasound med 2003. Germ cell tumor is a rare cancer that can affect people at all ages. A germcell tumor gct is a neoplasm derived from germ cells. Germ cell tumors danafarberboston childrens cancer and.
Children with benign germ cell tumors will have surgery to remove the tumor. Expression profile of genes from 12p in testicular germ cell tumors of adolescents and adults associated with i12p and amplification at 12p11. Germ cell tumors are classified into two broad groups. The ovary, in the current classification, has three anlagen. Germ cell brain tumors are tumors, usually cancerous, that arise in the pineal or suprasellar region of the brain. Group united kingdom between 1985 and 2009 were merged. Germ cell tumors are generally located in the gonads but can also appear in the abdomen, pelvis, mediastinum, or brain. 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 3. Benign germ cell tumors include certain kinds of teratomas. Ovarian germ cell tumor treatment ovarian teratoma treatment. The main focus is the complex histopathology of these tumors, but special considerations are also given to their morphologic variations in relation to age, gender and organ.
Extragonadal means outside of the gonads sex organs. Germ cell tumors are composed of a number of histologically different tumor types derived from the. Germ cell tumors starting within the brain are discussed further under brain tumors. These neoplasms comprise approximately 20 to 25 percent of ovarian neoplasms overall, but account for only an approximate 5 percent of all malignant ovarian neoplasms. In women, this type begins in the cells that form eggs in the ovaries, known as germ cells. Most germ cell tumors that are cancerous occur as cancer of the testicles testicular cancer or cancer of the ovaries ovarian cancer.
The germ cell theory hypothesizes malignant transformation of mismigrational primordial germ cells. More than 90 percent of patients with newly diagnosed germ cell tumors are cured, and delay in diagnosis correlates with a higher stage at presentation for treatment. Diagnostic staging further includes an abdominopelvic and chest ct, determination of. The likelihood of a cure can vary with the location of the tumor. These cells may grow in any location but generally settle in the brain brain germ cell tumors, chest chest germ cell tumors, or abdomen abdominal germ cell tumors. Integrated molecular characterization of testicular germ. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both.
Pediatric germ cell tumors gcts are the most important raretumors in childhood and adolescence and are often asymptomatic until theybecome an adnexal mass, compressing neighboring structures. Germ cell tumors are classified as seminoma dysgerminoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma, and teratoma. Germ cell tumors are masses of tissue formed by immature cells that normally would have developed into mature eggs in a female or sperm in a male. The most common intrapericardial germ cell tumors are teratomas followed by yolk sac tumors 3. Mouse models have revealed that abnormalities in pgc speci. These are the cells in the body that develop into sperm and eggs. In men, germ cell tumors occur most commonly after puberty and most of them develop to testicular cancer in the long run. To determine the efficacy of the addition of ifosfamide to cisplatin plus etoposide vip, or vinblastine veip, in patients with recurrent germ cell tumors. Germ cell tumors israel pdf ppt case reports symptoms. Cog in the usa to form the malignant germ cell tumour international consortium. Germ cell tumours the royal marsden nhs foundation trust. Revised risk classification for pediatric extracranial germ cell.
Despite the persistent increase in incidence of germ cell tumors, 1 it remains a rare malignancy compared with other solid tumors and accounts for only 1% of all male malignancies in the united states. Pdf current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic. They can destroy the testes or ovaries and can spread to other parts of the body. The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. Pdf human germ cell tumors are developmental cancers. Based on our clinical experience, we undertook a multi. These tumors start and grow mainly in two areas in the center of the brain. Testicular tumors most commonly occur in men between 20 and 35 years of age, and are the most common solid malignancy in this group.
Pathology and biology of human germ cell tumors pdf. Extragonadal germ cell tumors can be benign noncancer or malignant. It deals with the clinical presentations, treatment modalities, the biology and genetics of germ cell tumors in children and adults. Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life. Germ cell tumours most often develop in the ovary or testicle because this is where most germ cells are. As of 2018, germ cell tumors account for 3% of all cancers in children and adolescents 019 years old. Salvage therapy for patients with germ cell tumor jco. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20.
All these factors may combine in their e ect, leading to the critical event in tgct formationthe abnormal cell. An ovarian germ cell tumor is a growth in a womans ovaries. Jan 31, 2019 treatment recommendations for all other malignant germ cell tumors in patients with embryonal or endodermal sinus tumors, stage iiiv dysgerminoma, or stage 1 grade 23 or stage iiiv immature teratoma, standard postoperative adjuvant chemotherapy for three or four cycles with bleomycinetoposidecisplatin bep is recommended, as follows. Although not as difficult to deal with as malignant tumors, benign germ cell tumors can cause problems because of their size. Extragonadal germ cell tumors treatment pdqpatient. Primary intracranial germ cell tumors pubmed central pmc. Mature teratomas are the most common type of benign germ cell tumor. Feb 10, 2016 terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. Younger women, commonly patients under the age of 21, are more common. Ninety percent of germ cell tumors are gonadal, which means they begin in the reproductive cells of the testes testicular tumors or ovaries ovarian tumors.
Two of the most common germ cell tumors are seminoma and nonseminoma. Germ cells normally occur inside the gonads ovary and testis. Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are 1associated with high cure rates of 85. As with epithelial ovarian cancers, it is a good idea to consult with a gynecologic oncologist for treating malignant germ cell tumors, especially because these are so uncommon. Malignant ovarian germcell tumours account for about 5% of all ovarian malignancies and typically present in the teenage years. Germ cell tumor classification radiology reference. Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. Chromosome 12 abnormalities in malignant ovarian germ cell tumors. The book aims to provide an overview of current knowledge regarding germ cell tumors. Primordial germ cells mismigrate during embryonic development and subsequently undergo malignant transformation. About 900 children and adolescents are diagnosed with germ cell tumors in the u. Those with malignant tumors will first go through a process called staging.
Among these, a common form is teratoma with endodermal sinus tumor. It can be characterized into cancerous or noncancerous cell. First, the clinical stage is based on the results of tests done before surgery, such as a physical examination, ct scans, and mri tests. Testicles contain many specialized cells, including germ cells, which make sperm, and other specialized cells that make testosterone. Retroperitoneal germ cell tumor diagnosed by endoscopic ultrasoundguided fine needle aspiration. Extragonadal germ cell tumors clinical presentation. Most germ cell tumors were more in males than females except for neurohy pophyseal localization where the numbers were almost equal p, 0.
Dec 28, 2018 there are germ cell tumors that grow outside of the gonads very rare. Testicular cancer is the most common carcinoma in young men age 15 to 35 years. Commonly seen as a painless mass in the scrotum the skin that holds the testicles, parents are most likely to notice the development of these tumors in small boys. Different types of these germ cells cause different types of tumors, including the following.
Nonseminomatous germ cell tumors are cancer and are found in the brain. An overview of testicular germ cell tumors request pdf. Staging is a classification system that helps doctors figure out how far the cancer has progressed. Moreover, the interactions of germ cells with an altered interstitial microenvironment, including sertoli and leydig cells that in. The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system.
A germ cell tumor gct is a neoplasm derived from germ cells. They are almost always unilateral and are exquisitely chemosensitive. In women, 30% of ovarian tumors originate from germ cells. The natural history of primary intracranial germcell tumors gcts is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7%. Germinoma, teratoma choriocarcinoma, embryonal carcinoma, yolk sac. Each year, it affects about 8,500 men in the united states, many of them men between the ages of 15 and 35.
Childhood extracranial germ cell tumors treatment pdq. Primary intracranial germ cell tumors are rare icgcts and usually localized in the pineal and suprasellar regions of the brain. The treatment for germ cell tumours depends on the size, position and stage of the tumour. Germ cell tumors most commonly appear in the testes for boys and the ovaries for girls. Germ cell tumors in children usually form in the gonads, but can migrate to other areas. Diagnosis is made primarily based on palpation and findings on testicular ultrasound. Cell reports resource integrated molecular characterization of testicular germ cell tumors hui shen,1,32 juliann shih,2,3,4,32 daniel p. They are rapidly growing neoplasms that arise from primordial germ cells derived from the embryonal gonad. Germ cells migrating to the gonads may not reach that.
1435 1577 352 790 499 1324 297 1439 1178 451 758 307 1398 1211 225 875 1183 301 343 460 349 432 590 546 203 915 1077 936 406 271 253 49 326 44 364 1251 822 848