Download file pathology and biology of human germ cell tumors pdf. Germ cell tumors arise from the germ egg cells of the ovary and primarily occur in adolescents and young women. Pdf germ cell tumors of the ovary an update researchgate. There are germ cell tumors that grow outside of the gonads very rare. Extragonadal germ cell tumors treatment pdqpatient. Germ cell tumors gcts are a heterogeneous group of malignant and non. 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. Most germ cell tumors were more in males than females except for neurohy pophyseal localization where the numbers were almost equal p, 0.
The two main types of nonepithelial ovarian cancer, germ cell tumors and sex cordstromal tumors, collectively. Diagnosis of germ cell tumors requires many diagnostic procedures. Birch r, williams s, cone a, einhorn l, roark p, turner s, greco fa. Cooperative universitybased cancer study comprising 27 participating institutions. Primary extragonadal germ cell tumors gct are rare and account for only 1% to 5% of all germ cell malignancies 1, 2. Germ cell tumors can spread metastasize to other parts of the body. Ovarian germ cell tumor treatment ovarian teratoma treatment.
Yolk sac tumors, also called endodermal sinus tumors are usually cancer. Doctors perform physical examination, imaging studies xray, ct scan and mri, blood tests and biopsy of the tumor. About 900 children and adolescents are diagnosed with germ cell tumors in the u. Nonseminomatous germ cell tumors are cancer and are found in the brain. In adults, the most common sites of primary extragonadal germ cell tumors are, in descending order, the mediastinum, retroperitoneum, and cranium. Germ cell tumors is a neoplasm which obtained from germ cells. Among these, a common form is teratoma with endodermal sinus tumor. Metastatic igcccg good prognosis intermediate prognosis poor prognosis. Highly specific for germ cell tumors but also seen in ovarian germ cell neoplasms. Comparison of criteria for assigning germ cell tumor patients to good risk and poor risk studies. Germ cell tumors begin in the reproductive cells egg or sperm of the body. Bifocal intracranial tumors of nongerminomatous germ cell etiology.
Germcell tumors in childhood and adolescence annals of oncology. Although they generally have high cure rates, metastases, resistance to cisplatinbased therapy, and late toxicities still represent a lethal threat, arguing for the need of new therapeutic options. Nonrandom prospective trial with endpoints of tumor response, results of repeat surgical exploration, progressionfree interval, and survival. They can destroy the testes or ovaries and can spread to other parts of the body. 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. Management of the primary malignant mediastinal germ cell. The most common germ cell tumors occurring in the perinatal period in order of rank are teratoma, yolk sac tumor, choriocarcinoma, and gonadoblastoma.
Protocol for the examination of resection specimens. Pediatric germ cell tumor is the term used to describe malignant cancers of germline cells in patients aged 018 years. Pathology and biology of human germ cell tumors pdf. The clinical manifestations, diagnosis, and staging are presented separately, as are more. The most common sites for metastasis are the lungs. The germ cells which are present outside the gonads are the birth defects during the development of embryo. Ovarian germ cell tumor is a disease in which malignant cancer cells form in the germ egg cells of the ovary. A number of recommendations emerged from the conference, including that lymphovascular invasion lvi should always be reported and no. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by. Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. To evaluate the efficacy of cisplatinbased combination chemotherapy in patients with advanced ovarian germcell tumors.
Gcts are thought to originate from primordial germ cells, which migrate to the primitive gonadal glands in the urogenital ridge. Ve reuter pathogenesis of testicular germ cell tumours. Most chapters are focused on testicular germ cell tumors whose incidence has been increasing in young males. Germ cell tumors account for about 2 to 4 percent of all cancers in children and adolescents younger than age 20. 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. A teratoma is a tumor that may contain several different types of tissue, such as hair, muscle, and bone. The drugs that are commonly used for treating germ cell tumors include bleomycin blenoxane, cisplatin platinol, and etoposide vepesid, toposar. It deals with the clinical presentations, treatment modalities, the biology and genetics of germ cell tumors in children and adults. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. Age and gender can affect the risk of extragonadal germ cell tumors.
Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. 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. Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. Biopsy is essential for final confirmation and it provides with full data regarding tumor s pathohistological type. Most germ cell tumors that are cancerous occur as cancer of the testicles testicular cancer or cancer of the ovaries ovarian cancer. Benign germ cell tumors include certain kinds of teratomas. Cisplatin, vinblastine, and bleomycin in advanced and. Extragonadal germ cell tumors are usually seen in children or young adults and typically arise in midline locations. Intracranial seminoma commonest histology 6070% of all.
The name of a germ cell tumor came from the word germinate, which means to begin to grow. 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. Doctors use many tests to find, or diagnose, a tumor. When germ cell tumors are found mixed with teratomas, one can infer a transformation between the different cell types i. Germ cell tumors in children usually form in the gonads, but can migrate to other areas. It can be characterized into cancerous or noncancerous cell. Malignant germ cell tumors include several types of cancer, such as immature teratoma, yolk sac tumor and choriocarcinoma. An overview of therapy for men with testicular gcts is presented here. Germ cell tumors health encyclopedia university of.
Histologically, approximately 65% of cns germ cell tumors are pure germinomas, and most of the remaining ones are either pure nongerminomatous germ cell tumors nggct such as embryonal carcinoma, endodermal sinus or. Germ cell tumors 20% of all ovarian tumors 23% of ovarian malignancies presentation at young age tumor markers hcg. The book aims to provide an overview of current knowledge regarding germ cell tumors. Handbook for children with germ cell tumors american pediatric.
Germ cell tumors gcts are the most common solid malignancies found in young men. 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. Approximately 90% of germ cell tumors arise in the testicles, though extragonadal primary tumors of. The survey results were used to initiate discussions, but decisions were made by expert consensus rather than voting.
Germ cell tumors are growths that form from reproductive cells. Bajorin d, katz a, chan e, geller n, vogelzang n, bosl gj. Some germ cell tumors occur in other areas of the body, such as the abdomen, brain and chest, though its not clear why. Ovarian germ cell tumors consider the ovary, fallopian tube, peritoneum protocol. Prognostic factors for favorable outcome in disseminated germ cell tumors. A number of different cell types can be found in the testis germ cells. Ovarian germ cell tumors ogcts are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad, which accounts for about 2. There are four main types of ogcts, namely dysgerminomas, yolk sac tumor, teratoma, and choriocarcinoma dygerminomas are the most common type and are particularly prominent in patients diagnosed with. Signs and symptoms of extragonadal germ cell tumors include breathing problems and chest pain. 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.
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. To determine the efficacy of the addition of ifosfamide to cisplatin plus etoposide vip, or vinblastine veip, in patients with recurrent germ cell tumors. Within the ovaries, germ cell tumors can usually be distinguished from ovarian cysts, which are much more common, using ultrasound. For detailed discussion please refer the articles listed at the end of this page. Each component and the proportion of the tumor it represents should be indicated in the pathology report, as this may influence treatment and prognosis. 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 form from developing sperm or egg cells that travel from the gonads to other parts of the body.
In teenaged boys, testicular tumors may be more difficult to detect if parents and teens are uncomfortable discussing such intimate matters. 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. The field of ovarian germ cell tumors ogcts has remained relatively unchanged in the last 2 decades. Germ cell tumors in the testes of an adolescent male commonly present as an enlarging, solid mass, which may be painful. Extragonadal germinal cell syndromes are rare tumors that predominantly affect young males. Successful management of pediatric germ cell tumors is highly dependent on adherence to sound surgical principles, which can vary depending on the nature and. Management options for stage 1 nonseminomatous germ cell tumours surveillance rplnd adjuvant chemotherapy patients should be made aware of the different management options deciding which option depends upon histology, prognosis, geography, personality, reliability, bias of the doctor, but most importantly the patient. The ovary, in the current classification, has three anlagen.
About 95 percent of testicular cancers begin in germ cells, specialized cells in the testicles that make sperm. 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. Initial therapy of early stage testicular germ cell tumors gcts is based on histology and tumor extent. During fetus development, germ cells migrate to become the eggs in the ovary or the sperms in the testicles. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts. You will find a list of common tests, procedures, and scans that doctors use to find the cause of the medical problem.
The most common extragonadal sites are the mediastinum and retroperitoneum. Germ cell tumors can spread to lymph nodes, lung, liver, and brain. 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. Reporting and staging of testicular germ cell tumors. Embryonic germ cell tumors include teratoma, and extraembryonic germ cell tumors include choriocarcinoma and yolk sac tumor.