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. Pediatric and adolescent extracranial germ cell tumors. Pdf on feb 1, 2011, l leejones and others published ovary. Malignant ovarian germcell tumours account for about 5% of all ovarian malignancies and typically present in the teenage years. 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. Get detailed treatment information for newly diagnosed or recurrent germ cell tumors. Represent 30% of gcts and 70% of neoplastic ovarian masses, being the. Ovarian germ cell tumor treatment ovarian teratoma treatment. Continued pregnancy after ovarian germ cell tumors. Germ cell tumors arise from the primordial germ cells and sex cordstromal derivatives.
Germ cell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. In children and adolescents, more than 60% of ovarian neoplasms are of germ cell origin, and one third are malignant. The college of american pathologists february 2020 release includes 47 revised cancer protocols and one new adult autopsy reporting protocol. The world health organization histological classification for ovarian tumors separates ovarian neoplasms according to the most probable tissue of origin. Treatment of children and adolescents with stage ii testicular and stages i and ii ovarian malignant germ cell tumors. There are four main types of ogcts, namely dysgerminomas, yolk sac tumor, teratoma, and choriocarcinoma. The concept of germ cell tumors as a specific group of gonadal neoplasms has evolved over the last several decades. Paediatric extracranial germcell tumours the lancet. Malignant mixed germ cell tumour of ovary an unusual combination. Gcts that originate outside the gonads may be birth defects resulting from errors during development of the embryo. Ovarian germ cell tumors germ cells usually form the ova or eggs in females and the sperm in males.
In addition, the majority are unilateral, with bilateral ovarian involvement occurring in 10% to 15% of pure dysgerminoma, in some mixed germ cell tumors in which the predominant component is dysgerminoma, and in occasional cases of. Germ cell tumors are rare, about 900 children and adolescents are diagnosed in the united states each year. Ovarian germ cell tumors treatment pdqpatient version. Germ cell tumors and their association with pregnancy intechopen. These tumors frequently affect only one ovary and are generally curable if found and treated early.
They represent 4050% of all benign ovarian neoplasms. Principles of diagnosis and treatment apply across all histological types. As with epithelial ovarian cancers, it is a good idea to consult with a gynecologic oncologist for. Although many reports have been published about germ cell tumors of the ovary in developed countries, there has been no such documentation from nepal. 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. This accounts for 95 percent of all germ cell tumors and is.
A germcell tumor gct is a neoplasm derived from germ cells. Women with benign noncancerous germ cell tumors such as mature teratomas dermoid cysts are cured by removing the part of the ovary that has the tumor ovarian cystectomy or by removing the entire ovary. Yll collected the clinical data and performed the manual microdissection. Whether you can get pregnant after a germ cell tumor depends on the type of surgery you have if only one ovary and fallopian tube are removed. Germ cell tumors are growths that form from reproductive cells. In children and adolescents, up to 60% of ovarian tumors can be of germ cell origin. Ovarian germ cell malignant tumors are uncommon, and account for approximately 5 percent of ovarian cancer cases.
Table 1 subtypes and characteristics of ovarian germ cell tumours data derived from rice, 1999 and john hopkins pathology, 2001. 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 genotype 8, often difficult to. Oct34 regularly shows positivity in dysgerminoma but can also be expressed in ec35 and in some immature neural elements of ovarian teratoma. Germ cell tumors begin in the reproductive cells egg or sperm of the body. Germ cell tumors most commonly appear in the gonads sex organs. Recurrent ovarian mixed germ cell tumor with unusual malignant. Some germ cell tumors occur in other areas of the body, such as the abdomen, brain and chest, though it. Three courses of bep will nearly always prevent recurrence in wellstaged patients with completely resected ovarian germ cell tumors and should be given to all such patients. Ovarian germ cell tumors ogcts comprise 2025% of all ovarian cancers arising from germ cells of ovary. 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. Thus, a patient with a malignant ovarian germ cell tumor discovered early in pregnancy and in need of chemotherapy is faced with a dilemma for which no data are available. This is a rare tumor originating from both testicular and ovarian sex cord cells.
Patients with germ cell tumours are managed by a diverse array of specialists. The gastrointestinal and breast protocols have been updated to reflect the revised who histologic types. Malignant ovarian germ cell tumors occur in the first four decades and are rare thereafter. Germ cell tumors can arise from the ovary or from other locations of the body due to germ cell migration. The gonadal sites ovary and testis account for 40% of cases. Germ cell tumors germ cell tumors arise from the ovary s germinal elements and comprise a third of all ovarian neoplasms. Germ cell tumors constitute the second largest group of ovarian neoplasms after the surface epithelialstromal tumors. Sometimes, a group of germ cells grows in a way thats not normal.
In adults 95% are benign and consist of mature cystic teratomas dermoid cysts. The retrospective study presented here reports the clinicopathologic profile of germ cell tumors of the ovary studied at b. Germ cell tumors also exhibit a unique biology and genetics that distinguish them from other solid tumors and might contribute to their. Colon protocol has been updated requiring reporting of macroscopic evaluation of mesorectum. Msy and crl drafted the manuscript, including the revision. Germ cell tumors of the ovary comprise fewer than 5% of all malignant ovarian tumors. Ovarian germ cell tumors treatment options include surgery, chemotherapy, and radiation therapy.
Germ cell and stromal tumors of the ovary fred ueland, md university of kentucky gynecologic oncology. Germ cells tumors of the ovary account for approximately 1520% of all ovarian tumors. Ovarian germ cell tumors gcts are rare tumors affecting predominantly girls or young women. Cancer protocol templates college of american pathologists.
Germ cell tumors of the ovary textbook of uncommon. You can also get a germ cell tumor in the brain, chest, belly. Germ cell tumor gct, dysgerminoma, metastatic ovarian cancer. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. Ovarian germ cell tumour subtypes were distinct diseases, requiring different management. Mixed germ cell tumours of the ovary are malignant neoplasms of the ovary comprising of two or more types of germ cell components. Germ cell tumors include tumors which arise from the ovum and its precursor germ cells. They can occur in women of any age, but are more often found in young women or adolescent girls. These updated guidelines on nonepithelial ovarian cancer cover prevention, diagnosis, treatment and followup for early and advanced stages and recurrences of germ cell tumours, sex cordstromal tumours and small cell carcinomas of the ovary hypercalcaemic type. This is different from the adult population where epithelial neoplasms predominate. Malignant ovarian germ cell tumors mogcts are rare but curable at all stages of disease. With the advent of combination chemotherapy, risk of relapse has markedly decreased. Pdf germ cell tumors of the ovary an update researchgate.
The field of ovarian germ cell tumors ogcts has remained relatively unchanged in the last 2 decades. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary. Malignant ovarian germ cell tumor an overview sciencedirect. The mature cystic teratoma, also called dermoid cyst, is by far the most common subtype.
Germ cell tumors embrace morphologically heterogeneic neoplasms considered to be originated from germ cells of the gonad and are the most. Pathology outlines who classification of ovarian neoplasms. Germ cell tumors arise from the pleuripotential primordial germ cell and form the commonest group of pediatric ovarian neoplasms comprising between 60 and 80% of all such neoplasms. Malignant ovarian germ cell tumors ogcts historically carried a poor prognosis, especially among those diagnosed with advanced disease. Thus, staging, risk stratification, and treatment approaches for germ cell. Gonadal germ cell tumors in children and adolescents. Get detailed treatment information for newly diagnosed or recurrent germ cell tumors in this summary for clinicians. In the first 2 decades of life, almost 70% of ovarian tumours are of germcell origin, and. Cellular classification and clinical and imaging features1 ovarian malignant germ cell tumors omgcts are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad. Germ cell tumors are composed of a number of histologically different tumor types derived from the primitive germ cells of the embryonic gonad. Primitive gcts comprise dysgerminomas and nondysgerminomatous gct, which include yolk sac tumor, embryonal carcinoma, polyembryoma, nongestational choriocarcinoma, and mixed gcts. They make up only 4% of all cancers in children and adolescents. Female malignant ovarian germ cell tumours mogcts are rare, but early diagnosis and multiagent chemotherapy are 1associated with high cure rates of 85.
Mogcts, which include dysgerminomas, immature teratomas, embryonal tumours and endodermal sinus. Koirala institute of health sciences, dharan, nepal. Ovarian germ cell tumors ogcts are rare tumors that comprise a diverse group of histologic subtypes that can either be benign or malignant. This video mainly focuses on stertoli cell tumor of ovary. While sharing many similarities with male germ cell tumours gcts, a group of.
Most germ cell tumors that are cancerous occur as cancer of the testicles testicular cancer or cancer of the ovaries ovarian cancer. Management of paediatric extracranial germcell tumours carries a unique set of challenges. Nonepithelial ovarian cancer esmo clinical practice. Germ cells normally occur inside the gonads ovary and testis.
Ogcts arise primarily in young women between 10 and 30 years of age and represent 70. Clinical profile and classification germ cell tumors. Because the majority of patients with malignant ovarian germ cell tumors are diagnosed when the disease is in an early. Ovarian neoplasms coelomic epithelium germ cell specialized gonadal stroma nonspecific mesenchyme metastatic tumors 5070% 1520% 510% 510% 510%. Malignant ovarian germcell tumours account for about 5% of all ovarian malignancies and. Caucasian and african american females have similar risk of ovarian and other germ cell tumors. A pediatric intergroup studypediatric oncology group 9048 and childrens cancer group 8891. Since that time, newer drugs including cisplatin have increased the cure rate of these tumors to over 80%, even in patients with metastatic disease. Several new treatment algorithms are featured as well as an extensive summary of. It was recognized in the 1960s that testicular germ cell tumors were curable with chemotherapeutic drugs. Malone and colleagues described a patient with stage ic endodermal sinus tumor.
There have been several published reports of ovarian germ cell cancers affecting more than one family member. Most ovarian germ cell tumors are benign, but some are cancerous and may be life threatening. Eightyone patients with ovarian germ cell malignancies immature teratoma 29, dysgerminoma 26, endodermal sinus tumors 15, mixed germ cell tumor 8, other 3 seen or consulted on at yale. They are almost always unilateral and are exquisitely chemosensitive. Germ cell tumors of the ovary are presumed to derive from the pathogenic transformation of ovarian germ cells at different stages of development. Malignant mixed ovarian germ cell tumor composed of immature teratoma, yolk sac tumor and embryonal carcinoma harboring an egfr.
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