The tumor may then require a biopsy to determine its exact pathology. They originate from cells that were meant to form sex cells i. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. The likelihood of a cure can vary with the location of the tumor. Germcell tumors germcell tumors are composed primarily of testicular neoplasms seminomas or nonseminomatous tumors but also include ovarian and extragonadal germcell tumors e. Germ cell tumors arise from residual primordial ectoderm, mesoderm, or endoderm, and account for greater than half of the pineal region neoplasms. Pdf current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic. Most chapters are focused on testicular germ cell tumors whose incidence has been increasing in young males. 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. These tumors consist of several histological variations and exhibit several grades of differentiation. Germ cell tumors are composed primarily of testicular neoplasms seminomas or nonseminomatous tumors but also i nclude ovarian and extragonadal germ cell tumors e.
Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Germcell tumors are composed primarily of testicular neoplasms seminomas or nonseminomatous tumors but also i nclude ovarian and extragonadal germcell tumors e. Thus, staging, risk stratification, and treatment approaches for germcell. Management of paediatric extracranial germcell tumours carries a unique set of challenges.
Most germ cell tumors were more in males than females except for neurohy pophyseal localization where the numbers were almost equal p, 0. The most common sites for metastasis are the lungs. Germ cell tumors of the ovary an update francisco f. Only sporadic cases have been documented in the literature. Ovarian germ cell tumor is a disease in which malignant cancer cells form in the germ egg cells of the ovary. Prognostic factors for favorable outcome in disseminated germ cell tumors. Two of the most common germ cell tumors are seminoma and nonseminoma. Their exact incidence is not known due to the morbidity of surgery in the pineal gland area.
For language access assistance, contact the ncats public information officer. The goal of this study was to determine whether the igccc is. Germ cell tumors are classified according to their histology, stage, prognosis, and response to chemotherapy. Intracranial seminoma commonest histology 6070% of all. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. Current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic mutations. 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.
Germcell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. The goal of this study was to determine whether the igccc is applicable to a populationbased cohort. Germ cell tumors of the gonads have a better prognosis than germ cell tumors outside the gonads extragonadal. They are often large and have a tendency to spread more quickly than the other type of germ cell tumor i. 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.
These extragonadal germ cell tumors histologically contain the same components as their gonadal counterparts, but may have different biologic behaviors, clinical characteristics and. Yolk sac tumor yst, embryonal carcinoma ec, choriocarcinoma cc, and combined germ cell tumors cgcts of the mediastinum are uncommon neoplasms. Germ cell tumors is a neoplasm which obtained from germ cells. Learn about treatment options for ovarian germ cell tumors. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. Germ cell tumors can spread to lymph nodes, lung, liver, and brain. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. Malignant ovarian germ cell tumors mogcts are rare and account for around 5% of all ovarian malignancies 1. 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. Protocol applies to germ cell tumors located in the mediastinum.
Table 71 summarizes the frequency of predominantly adult surgical pathology cases seen at the new york neurologic institute. After a germ cell tumor is diagnosed, doctors need to assess how advanced it is, which is called staging the tumor. Benign germ cell tumors include certain kinds of teratomas. The management of primary intracranial germ cell tumors is changing. 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. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary the ovaries are a pair of organs in the female reproductive system. Pluripotent embryonic cells mismigrate and give rise to gcts. Download file pathology and biology of human germ cell tumors pdf. Benign teratomas have an excellent prognosis with surgery. Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1, 2. Malignant tumors include immature teratomas and other germ cell tumors containing malignant cells. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Pediatric germ cell tumor is the term used to describe malignant cancers of germline cells in patients aged 018 years.
This provides a set of candidate biomarkers for risk stratification and potential therapeutic targeting. Germ cell tumors health encyclopedia university of. Germ cells normally occur inside the gonads ovary and testis. The management of germ cell tumors is histology dependent and. Germcell tumors in childhood and adolescence annals of oncology. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body.
Paediatric extracranial germcell tumours the lancet. Ovarian germ cell tumors treatment pdqpatient version. Nonseminomatous germ cell tumors are cancer and are found in the brain. If you have problems viewing pdf files, download the latest version of adobe reader. Furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. Nonseminomatous germ cell tumors are cancerous tumors commonly found in the pineal gland in the brain, in the mediastinum, or in the abdomen. Germ cell tumors also exhibit a unique biology and genetics that distinguish them from other solid tumors and might contribute to their. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. Although not as difficult to deal with as malignant tumors, benign germ cell tumors can cause problems because of their size. Birch r, williams s, cone a, einhorn l, roark p, turner s, greco fa. Extragonadal germ cell tumors treatment pdqpatient. Advanced germ cell tumors potential for cure, especially in poor risk patients notable toxicities and adverse events highly specialized and expensive 1. Germ cell tumors may develop in the brain, the testes, the ovaries, or elsewhere in the body.
This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. 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. The most common extragonadal sites are the mediastinum and retroperitoneum. The book aims to provide an overview of current knowledge regarding germ cell tumors. Germ cell tumors background and pathology germ cell tumors are relatively uncommon, constituting 3% to 5% of large institutional pediatric tumor series and 0. Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are associated with high cure rates of 85. Germcell tumors are classified according to their histology, stage, prognosis, and response to chemotherapy. University of groningen testicular germ cell tumors gels. 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. 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. Pure seminoma is sensitive to radiotherapy and the prognosis is good. In western countries, intracranial germ cell tumors com. Types of surgery include hysterectomy and removal of one or both ovaries and fallopian tubes bilateral salpingooophorectomy.
Pathology and biology of human germ cell tumors pdf. Many factors also affect the outlook for a good outcome. The presence of liver metastases represents an independent poor risk prognostic factor for survival in patients with germ cell tumors. Germ cell tumors are a varied group of benign and malignant neoplasms derived from primordial germ cells 110, 1215 table 2. 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. It is likely that small areas of endodermal sinus tumor.
Since that time, newer drugs including cisplatin have increased the cure rate of these tumors to over 80%, even in patients with metastatic disease. Germ cell tumours list of high impact articles ppts. Case report coexistence of intracranial germ cell tumor. Application of the international germ cell consensus. Germ cell tumors gain in sequences of 12 p most often i 12p tumors including itgcn aneuploid seen in 95% of germ cell tumors gain chromosome 9 in spermatocytic seminoma intratubular germ cell neoplasia edge of invasive tumor infertility 1% bxs approximately 50% progress in 5 years cryptorchid testis 28%. Sometimes, a group of germ cells grows in a way thats not normal. A teratoma is a tumor that may contain several different types of tissue, such as hair, muscle, and bone. Ovarian neoplasms coelomic epithelium germ cell specialized gonadal stroma nonspecific mesenchyme metastatic tumors 5070% 1520% 510% 510% 510%. Management of the primary malignant mediastinal germ cell. Nonseminomatous germ cell tumor genetic and rare diseases. Ovarian germ cell tumors from routine material and expert consultation files at san cecilio university hospital, granada, spain. Germ cell and stromal tumors of the ovary university of kentucky. For detailed discussion please refer the articles listed at the end of this page.
The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system. Many factors go into a doctors decision about how to best treat germ cell tumors. Because germ cell tumors can vary greatly, and can arise in different parts. It was recognized in the 1960s that testicular germ cell tumors were curable with chemotherapeutic drugs. 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. The who classifies them into germinomas and nongerminomatous germ cell tumors. Oct34 regularly shows positivity in dysgerminoma but can also be expressed in ec35 and in some. Germ cell tumors begin in the reproductive cells egg or sperm of the body. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life. Patients with germcell tumours are managed by a diverse array of specialists. Mediastinal germ cell tumors are usually silent when small and therefore more difficult to treat. Surgery is becoming an increasingly safer procedure for patients with suprasellar and pineal region tumors, and histologic documentation is a prerequisite for optimum therapy.
Most teratomatous elements in the testis occur as a component of mixed germ cell tumors, which represent about onethird of all testicular germ cell tumors and contain teratoma in about 50% of the cases. The histologic profile of dysgerminoma is stereotypic and identical to that of testicular seminoma and midline germinomas. 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. An unknown percentage of malignant germ cell tumors reported in phenotypic women may, in fact, represent mtgcts seminomas, embryonal carcinomas, mixed germ cell tumors, as they may have originated from the malignant germ cell component of gonadoblatomas present in dysgenetic gonads of patients with an unrecognized y chromosomecontaining. Intracranial germ cell tumors comprise a heterogeneous group of neoplasms. Large, clear, neoplastic, primitivetype germ cells are consistently associated with a variable cytotoxic tcell lymphocytic response 20 that helps to identify the tumor, even in cases with complex histology. 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. Surgery is the most common treatment of ovarian germ cell tumor.
You can also get a germ cell tumor in the brain, chest, belly. Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. 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. You are advised to consult the publishers version publishers pdf if you wish to cite. Childhood extracranial germ cell tumors treatment pdq. Bajorin d, katz a, chan e, geller n, vogelzang n, bosl gj. The international germ cell consensus classification igccc is the internationally accepted, clinically based prognostic classification used to assist in the management and research of metastatic germ cell tumours gcts. Another reason is the complex classification system which was used to identify these tumors. Germ cell tumors israel pdf ppt case reports symptoms.
The germ cells which are present outside the gonads are the birth defects during the development of embryo. Germ cell tumors can spread metastasize to other parts of the body. Original article malignant ovarian germ cell tumors in. Pdf germ cell tumors of the ovary an update researchgate. However, the introduction of new stem cell pluripotency markers has provided a new understanding into the. Primordial germ cells mismigrate during embryonic development and subsequently undergo malignant transformation. Dysgerminomas, immature teratomas and endodermal sinus tumors est are the most common types and comprise over 90% of mogcts 2. Primary germ cell tumors of the mediastinum moran 1997.
Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. Treatment may also include chemotherapy or radiation therapy. Ovarian germ cell tumour subtypes were distinct diseases, requiring different management. Pdf human germ cell tumors are developmental cancers. It deals with the clinical presentations, treatment modalities, the biology and genetics of germ cell tumors in children and adults. 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. The field of ovarian germ cell tumors ogcts has remained relatively unchanged in the last 2 decades. Case report coexistence of intracranial germ cell tumor and. Extragonadal means outside of the gonads sex organs.
Handbook for children with germ cell tumors american pediatric. Comparison of criteria for assigning germ cell tumor patients to good risk and poor risk studies. Among these, a common form is teratoma with endodermal sinus tumor. Adequate fixation is important for a prima facie diagnosis, but. A germ cell tumor gct is a neoplasm derived from germ cells. Ve reuter pathogenesis of testicular germ cell tumours.
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