Germ cell tumors may develop in the brain, the testes, the ovaries, or elsewhere in the body. After a germ cell tumor is diagnosed, doctors need to assess how advanced it is, which is called staging the tumor. Ovarian neoplasms coelomic epithelium germ cell specialized gonadal stroma nonspecific mesenchyme metastatic tumors 5070% 1520% 510% 510% 510%. Yolk sac tumor yst, embryonal carcinoma ec, choriocarcinoma cc, and combined germ cell tumors cgcts of the mediastinum are uncommon neoplasms. The tumor may then require a biopsy to determine its exact pathology. Germ cell tumors is a neoplasm which obtained from germ cells. Primordial germ cells mismigrate during embryonic development and subsequently undergo malignant transformation. For language access assistance, contact the ncats public information officer.
Another reason is the complex classification system which was used to identify these tumors. Ovarian germ cell tumors treatment pdqpatient version. You can also get a germ cell tumor in the brain, chest, belly. The most common sites for metastasis are the lungs. Patients with germcell tumours are managed by a diverse array of specialists. Prognostic factors for favorable outcome in disseminated germ cell tumors. 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. Mediastinal germ cell tumors are usually silent when small and therefore more difficult to treat. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. Among these, a common form is teratoma with endodermal sinus tumor.
Advanced germ cell tumors potential for cure, especially in poor risk patients notable toxicities and adverse events highly specialized and expensive 1. Many factors also affect the outlook for a good outcome. 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. They originate from cells that were meant to form sex cells i. Ovarian germ cell tumors from routine material and expert consultation files at san cecilio university hospital, granada, spain.
Handbook for children with germ cell tumors american pediatric. The histologic profile of dysgerminoma is stereotypic and identical to that of testicular seminoma and midline germinomas. The germ cells which are present outside the gonads are the birth defects during the development of embryo. Germcell tumors are composed primarily of testicular neoplasms seminomas or nonseminomatous tumors but also i nclude ovarian and extragonadal germcell 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. 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. Ovarian germ cell tumour subtypes were distinct diseases, requiring different management.
For detailed discussion please refer the articles listed at the end of this page. Germ cell tumors can spread metastasize to other parts of the body. Surgery is the most common treatment of ovarian germ cell tumor. Many factors go into a doctors decision about how to best treat germ cell tumors. Most germ cell tumors were more in males than females except for neurohy pophyseal localization where the numbers were almost equal p, 0.
Management of female malignant ovarian germ cell tumours. Comparison of criteria for assigning germ cell tumor patients to good risk and poor risk studies. Pediatric germ cell tumor is the term used to describe malignant cancers of germline cells in patients aged 018 years. The goal of this study was to determine whether the igccc is. Germcell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. A teratoma is a tumor that may contain several different types of tissue, such as hair, muscle, and bone.
Nonseminomatous germ cell tumor genetic and rare diseases. These tumors consist of several histological variations and exhibit several grades of differentiation. Case report coexistence of intracranial germ cell tumor. Germ cell tumors of the ovary an update francisco f. Benign germ cell tumors include certain kinds of teratomas. Germ cell tumors can spread to lymph nodes, lung, liver, and brain. Germcell tumors in childhood and adolescence annals of oncology. This provides a set of candidate biomarkers for risk stratification and potential therapeutic targeting. Thus, staging, risk stratification, and treatment approaches for germcell. The who classifies them into germinomas and nongerminomatous germ cell tumors. 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. Pure seminoma is sensitive to radiotherapy and the prognosis is good. In western countries, intracranial germ cell tumors com. Extragonadal germ cell tumors primarily affect men during the third and fourth decades of life.
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. Although not as difficult to deal with as malignant tumors, benign germ cell tumors can cause problems because of their size. The presence of liver metastases represents an independent poor risk prognostic factor for survival in patients with germ cell tumors. This book provides, for the first time, a detailed, uptodate, holistic approach to the pathology and biology of germ cell tumors. Case report coexistence of intracranial germ cell tumor and. Current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic mutations. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. Table 71 summarizes the frequency of predominantly adult surgical pathology cases seen at the new york neurologic institute.
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. The field of ovarian germ cell tumors ogcts has remained relatively unchanged in the last 2 decades. 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. Types of surgery include hysterectomy and removal of one or both ovaries and fallopian tubes bilateral salpingooophorectomy. Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. Germ cell tumors arise from residual primordial ectoderm, mesoderm, or endoderm, and account for greater than half of the pineal region neoplasms. Terms germ cells a germ cell is any biological cell that gives rise to the gametes of an organism that reproduces sexually. It was recognized in the 1960s that testicular germ cell tumors were curable with chemotherapeutic drugs. Surgery is becoming an increasingly safer procedure for patients with suprasellar and pineal region tumors, and histologic documentation is a prerequisite for optimum therapy. 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. Sometimes, a group of germ cells grows in a way thats not normal.
Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1, 2. Dysgerminomas, immature teratomas and endodermal sinus tumors est are the most common types and comprise over 90% of mogcts 2. The most common extragonadal sites are the mediastinum and retroperitoneum. The most common sites for metastasis are the lungs, liver, lymph nodes, and central nervous system.
Yolk sac tumors, also called endodermal sinus tumors are usually cancer. It can be characterized into cancerous or noncancerous cell. Oct 17, 20 nonseminomatous germ cell tumors are cancerous tumors commonly found in the pineal gland in the brain, in the mediastinum, or in the abdomen. Pdf human germ cell tumors are developmental cancers. Since that time, newer drugs including cisplatin have increased the cure rate of these tumors to over 80%, even in patients with metastatic disease. A multicentre retrospective cohort study of ovarian germ cell tumours. Ovarian germ cell tumor is a disease in which malignant cancer cells form in the germ egg cells of the ovary. The management of germ cell tumors is histology dependent and. University of groningen testicular germ cell tumors gels. Because germ cell tumors can vary greatly, and can arise in different parts.
It deals with the clinical presentations, treatment modalities, the biology and genetics of germ cell tumors in children and adults. 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. Nonseminomatous germ cell tumors are cancer and are found in the brain. Pdf germ cell tumors of the ovary an update researchgate. Malignant ovarian germ cell tumors mogcts are rare and account for around 5% of all ovarian malignancies 1.
Protocol applies to germ cell tumors located in the mediastinum. 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. Germ cell tumors are classified according to their histology, stage, prognosis, and response to chemotherapy. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. Their exact incidence is not known due to the morbidity of surgery in the pineal gland area. Adequate fixation is important for a prima facie diagnosis, but. 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. Intracranial seminoma commonest histology 6070% of all. Download file pathology and biology of human germ cell tumors pdf.
However, the introduction of new stem cell pluripotency markers has provided a new understanding into the. Ve reuter pathogenesis of testicular germ cell tumours. Germ cells normally occur inside the gonads ovary and testis. 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. These extragonadal germ cell tumors histologically contain the same components as their gonadal counterparts, but may have different biologic behaviors, clinical characteristics and. 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 are found widely throughout the body, and encompass a wide range of individual tumors this article does not deal with any specific body locations. Germ cell tumors are composed primarily of testicular neoplasms seminomas or nonseminomatous tumors but also i nclude ovarian and extragonadal germ cell tumors e. Paediatric extracranial germcell tumours the lancet. It is likely that small areas of endodermal sinus tumor. Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are associated with high cure rates of 85. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts. Germ cell and stromal tumors of the ovary university of kentucky. Diagnosis of germ cell tumors begins with a thorough health history and a comprehensive physical examination.
Intracranial germ cell tumors comprise a heterogeneous group of neoplasms. Germcell tumors are classified according to their histology, stage, prognosis, and response to chemotherapy. Primary germ cell tumors of the mediastinum moran 1997. Germ cell tumors are a varied group of benign and malignant neoplasms derived from primordial germ cells 110, 1215 table 2.
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%. Pluripotent embryonic cells mismigrate and give rise to gcts. Pathology and biology of human germ cell tumors pdf. Birch r, williams s, cone a, einhorn l, roark p, turner s, greco fa. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Most chapters are focused on testicular germ cell tumors whose incidence has been increasing in young males. Germ cell tumors background and pathology germ cell tumors are relatively uncommon, constituting 3% to 5% of large institutional pediatric tumor series and 0. The goal of this study was to determine whether the igccc is applicable to a populationbased cohort.
Oct34 regularly shows positivity in dysgerminoma but can also be expressed in ec35 and in some. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. You are advised to consult the publishers version publishers pdf if you wish to cite. Malignant tumors include immature teratomas and other germ cell tumors containing malignant cells. Learn about treatment options for ovarian germ cell tumors. The book aims to provide an overview of current knowledge regarding germ cell tumors.
Furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors. Two of the most common germ cell tumors are seminoma and nonseminoma. Application of the international germ cell consensus. Management of paediatric extracranial germcell tumours carries a unique set of challenges. The likelihood of a cure can vary with the location of the tumor.
Nonseminomatous germ cell tumors are cancerous tumors commonly found in the pineal gland in the brain, in the mediastinum, or in the abdomen. Pdf current high throughput omicsbased data support the model that human malignant germ cell tumors are not initiated by somatic. If you have problems viewing pdf files, download the latest version of adobe reader. They are often large and have a tendency to spread more quickly than the other type of germ cell tumor i. Germ cell tumors health encyclopedia university of. The management of primary intracranial germ cell tumors is changing. 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. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Extragonadal germ cell tumors treatment pdqpatient. Germ cell tumors of the gonads have a better prognosis than germ cell tumors outside the gonads extragonadal. Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body.
Germcell tumors germcell tumors are composed primarily of testicular neoplasms seminomas or nonseminomatous tumors but also include ovarian and extragonadal germcell tumors e. Original article malignant ovarian germ cell tumors in. 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. Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. Treatment may also include chemotherapy or radiation therapy. Germ cell tumours list of high impact articles ppts.
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. Bajorin d, katz a, chan e, geller n, vogelzang n, bosl gj. Multimodality treatment of patients with liver metastases. 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. 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. Only sporadic cases have been documented in the literature.
Childhood extracranial germ cell tumors treatment pdq. 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. Benign teratomas have an excellent prognosis with surgery. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. 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. Management of the primary malignant mediastinal germ cell. A germ cell tumor gct is a neoplasm derived from germ cells. Germ cell tumors israel pdf ppt case reports symptoms.