The yolk sac is the first element seen within the gestational sac during pregnancy, usually at 3 days gestation the yolk sac is situated on the front part of the embryo. Histological examination of specimens after subtotal removal revealed a primary mixed germ cell tumor with extensive yolk sac tumor elements, often referred to as an intracranial yolk sac tumor. They are difficult to recognize at other sites outside the gonads, and no case has been reported involving the upper lip. Oct34 is positive in all dysgerminomas but most often negative in yolk sac tumors 9% are positive. Primary malignant extragonadal germ cell tumors of the lung are quite uncommon and pure pulmonary yolk sac tumor is so rare that it is hardly included in the. The treatment generally involves debulking surgery of tumors followed by systemic chemotherapy.
First case of mature teratoma and yolk sac testis tumor associated to inherited men1 syndrome article pdf available in frontiers in endocrinology 10. Comparative morphogenesis of the socalled mesoephroma ovarii schiller and extraembryonic yolk sacallantoic structures of the rats placenta. Yolk sac tumors ysts are a type of malignant germ cell tumor that usually grow in the gonads. Testicular yolk sac tumor radiology reference article. Mixed germ cell tumor with extensive yolk sac tumor elements. Jan 09, 2019 yolk sac tumor of the testis is a type of testicular cancer affecting the germ cells. Endodermal sinus tumor est is a member of the germ cell tumor group of cancers. Pdf primary yolk sac tumor of the lung mohamed nouh.
Yolk sac carcinoma is the most common form of testicular cancer in children. Postoperative pathology confirmed a diagnosis of endometrial yst with metastasis to the greater omentum stage ivb. Pdf first case of mature teratoma and yolk sac testis. In this case, suboccipital craniectomy was performed and the tumor was subtotally removed. Keywords sacrococcygeal teratoma, yolk sac tumor, afp, fnac 1. Magnetic resonance imaging showed a mass occupying most of the vagina that had lobulated outlines and heterogeneous echo texture. In addition to presenting in ovaries and testes, the tumor has been detected at several extragonadal sites such as the retroperitoneum, sacrococcygeal region, pineal gland, and the anterior mediastinum 1. Pdf successful management of a young patient with yolk sac tumour. A yolk sac tumor is a rare, malignant tumor of cells that line the yolk sac of the embryo. Yst usually occurs as a rapidly growing unilateral tumor in young women.
We report on a hepatic yolk sac tumor in a 15monthold girl who presented with acute abdomen. Testicular yolk sac tumor genetic and rare diseases. Malignant germ cell tumors of the ovary follow the same staging system as epithelial ovarian and primary peritoneal cancers. Some variants may occur predominantly in a single site, such as polyvesicular vitelline tumor in the ovary, or in a special clinical situation, such as the parietal or mesenchymelike variants as recurrences of testicular yolk sac tumor after chemotherapy. Yolk sac tumor article about yolk sac tumor by the free. It has been suggested that alphafetoprotein afp can be applied as a feasible tumor marker because its level was elevated in 90% of yst. It is most often found in children before the ages of 1 to 2, but can occur throughout life. Although most gct in children originates in the gonads, the most common primary site for yolk sac tumor yst is the sacrococcygeal region 4. They arise from pluripotent stem cell, occur in gonadal and extragonadal site 8. Yolk sac tumor yst or endodermal sinus tumor is the most common malignant germ cell tumor of the testis in children, and is the second most frequent malignant germ cell tumor of the ovary, being exceeded in frequency by dysgerminoma seminoma. Serum levels of alfafetoprotein were restored to normal and the patient remained free of disease for 15 months. It was first proposed in 1939 by schiller, who discovered an ovarian tumor with a meso. Yolk sac tumor definition of yolk sac tumor by medical. Yolk sac tumor yst, also known as an endodermal sinus tumor, is a rare malignant germ cell tumor.
Pdf yolk sac tumours are rare ovarian malignancies accounting for less than 1% of malignant ovarian germ cell tumours. Ovarian germ cell tumors ogcts are derived from primordial germ cells of the ovary. Yolk sac tumor in the abdominal wall of an 18monthold girl. Other names for this type of cancer include yolk sac tumor, endodermal sinus tumor, infantile embryonal carcinoma or orchidoblastoma. A combination of radical resection and radiation may improve survival and decline recurrence. Pdf yolk sac tumours, synonymous with endodermal sinus tumours, are rare, malignant germ cell neoplasms occurring usually in gonads. Testicular yolk sac tumors also known as endodermal sinus tumor of the testis is the most common childhood testicular tumor 80%, with most cases occurring before the age of two years 1. They are difficult to recognize at other sites outside the gonads, and no case has been reported.
Feb 25, 2014 if the inline pdf is not rendering correctly, you can download the pdf file here. Germ cell tumor gct in children is rare accounting for 3% 4% of childhood malignancies. Germ cells are precursors to sperm cells that will eventually transform into sperms. It is the most common testicular tumor in children under 3, and is also known as infantile embryonal carcinoma. Pdf ovarian yolk sac tumour in a girl case report researchgate. Primary yolk sac tumour of liver with unusual clinical.
J adenocarcinoma with yolk sac tumor of the stomach. A case of yolk sac tumor of the vagina in an infant. We report for the first time, the presentation of a primary yolk sac tumor in the abdominal wall of a small child. Dec 29, 2014 in the current study, ultrasonography and cdfi identified the tumors as solid masses with a rich blood supply. Primary yolk sac tumor originating from the endometrium. Yolk sac tumor is the predominant variant in newborns and younger children. Pineal yolk sac tumors are rare type of extragonadal yolk sac tumors. Primary yolk sac tumor of the lung the annals of thoracic surgery. It is abbreviated yst it may be referred to as endodermal sinus tumour. A 38yearold woman presented with the chief complaint of prolonged menstruation and increased menstrual bleeding with a duration of more than 2 months. Endodermal sinus tumor yolksac carcinoma of the orbit. Introduction ovarian yolk sac tumor oyst, also known as endodermal sinus tumor is a rare tumor comprising 1% of all ovarian malignancies.
Yolk sac carcinomas are so named because their cells look like the yolk sac of an early human embryo. Yolk sac tumors occur most often in the ovary or testicle, but they may also occur in other areas of the body, such as the chest, abdomen, or brain. We used intraoperative analyses of frozensections for routine evaluation of testicular yolk sac tumors to provide evidence for orchiectomy. 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. This is a yolk sac tumor with a reticular microcystic pattern. Afp is variably positive but many times negative in yolk sac tumors and oct34 is invariably negative. Malignant ovarian germ cell tumors mogcts are rare but aggressive, accounting for approximately 1% to 2% of all ovarian malignancies. Introduction among the germ cell gct, sacrococcygeal teratoma sct is the tumors most common congenital neoplasm occurring with a frequency of 1 in 20,000 to 40,000 live births 1 2, although it is also seen in adults 3. We report a case of a vaginal yolk sac tumor in a 5monthold female infant who presented with short history of bleeding per vagina. They make up a small fraction of all intracranial germ cell tumors and an even small fraction of pineal masses overall.
Ysts associated with nongerm cell tumours probably orig inate from malignant pluripotent somatic stem cells. Yolk sac or germ cell tumor cincinnati childrens hospital. Yolk sac tumour is an uncommon germ cell tumour that primarily afflicts infants and young boys it may be seen as part of mixed germ cell tumour. Yolk sac tumor yst of the ovary is a rare germ cell tumor comprising about 1% of all ovarian malignancies. Yolk sac tumors are the most common malignant germ cell tumor in children. A 28 year old woman presented with a cystic liver mass and a markedly raised serum alphafetoprotein concentration. 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. Introduction yolk sac tumors yst, also known as endoder mal sinus tumors, are malignant germ cell tumors gct that usually arise in the gonads, such as the testis or ovaries. Ct should be used for yolk sac tumor staging and identification of metastasis. Finally, yolk sac tumor was diagnosed surgically and histopathological. They tend to grow quickly and spread to other parts of the body if not treated. Primary yolk sac tumour of the liver is exceedingly rare.
Extragondal sites of ysts are reported in mediastinum, vagina, brain, and retroperitoneum but are extremely rarely in omentum. In adults, pure yolk sac tumor is extremely rare, howeve. Pdf yolk sac tumours are rare ovarian malignancies accounting for less than 1 % of malignant ovarian germ cell tumours. The nongerminomatous germ cell tumor occurring in the fourth ventricle is rare. The histological diagnosis was pure yolk sac tumor. Jan 19, 2015 yolk sac tumor yst of the testis is composed of primitive tumor cells, which are relatively small and less pleomorphic relative to those in embryonal carcinoma, another common subtype of malignant germ cell tumor of the testis. Yolk sac tumors, also known as endodermal sinus tumors are one of the common malignant germ cell tumors which account for 3% of all. Csf biochemistry may demonstrate elevated levels of alp. Yolk sac tumor yst is one of the rare malignant germ cell tumor and usually occurs in gonad. Yolk sac tumor yst is an extremely rare tumor of the liver.
Yolk sac carcinoma testicular cancer awareness foundation. She underwent a partial hepatectomy for a suspected hepatocellular carcinoma. Chemotherapy of endodermal sinus tumour yolk sac tumour. Definition of yolk sac tumor nci dictionary of cancer terms. Radiology of primary intracranial yolksac endodermal sinus. A rare case of primary anterior mediastinal yolk sac tumor in. Yolk sac tumors ysts, also referred to as endodermal sinus tumors, are a type of malignant germ cell tumor that usually grow in the gonads 1. Prepubertal type yolk sac tumor is almost always seen in its pure form contrarily to postpubertal type tumors, which are frequently mixed. Yst cells may form many histologic patterns resembling embryonal structures. An 18monthold white girl underwent resection of a small, round subcutaneous lump 1.
Yolk sac tumor of the testis is a type of testicular cancer affecting the germ cells. Endodermal sinus tumor yolk sac carcinoma is a type of malignant germcell tumor of the ovary and testis which on rare occasion occurs as a primary tumor elsewhere. She was diagnosed initially as intussusception, while ultrasonography and ctscan indicated a liver mass. Ovarian yolk sac tumor oyst, also known as endodermal sinus tumor is a rare tumor comprising 1% of all ovarian malignancies. Yolk sac tumor is a subtype of germ cell tumor that is highly malignant. The preoperative diagnosis of nggcts in unusual age and locations is extremely difficult. It was first proposed in 1939 by schiller, who discovered an ovarian tumor with a mesonephroid origin. Yolk sac tumor yst, which most frequently arises in the gonads as a type of germ cell tumor, is rare in children but is highly malignant.