The girl was admitted to the hospital with a left side cervical tumor with a quick growth within 3 wk. And what i mean by that is the fact the most common most common ovarian tumor in a girl less than five years of age, the most common testicular tumor in a boy less than five years of age is this. We report on our experience with two vaginal endodermal sinus tumors. Endodermal sinus tumor definition of endodermal sinus tumor. Yolk sac tumor endodermal sinus tumor yolk sac tumors, also called endodermal sinus tumors, are derived from the primitive yolk sac. Age, yrlsex nwml mluml subtype symptoms metastases 211m 1,672 tumor is associated with a somatic epithelial neoplasm serous carcinoma, clear cell carcinoma, endometrioid adenocarcinoma, borderline clear cell adenofibroma, serous tubal intraepithelial carcinoma, large cell neuroendocrine carcinoma histopathology 2016. Endodermal sinus tumor article about endodermal sinus tumor. Integrated 18ffluorodeoxyglucose positron emission tomography and computerized tomography in endodermal sinus tumor. Endodermal sinus tumor and embryonal carcinoma of the. We describe two cases of extensive est treated with a new highdose sequential combination chemotherapy regimen ciscavb followed by radical surgical excision. Therapy of endodermal sinus tumor of the ovary american journal. Six months following resection of the left cerebellar tumor, the patient succumbed to recurrence of the disease, due to acute vomiting and severe headache. Endodermal sinus tumor of the ovary can be differentiated histologically and immunohistochemically from ovarian embryonal cell carcinoma.
Ultrasound and magnetic resonance images of endodermal sinus. Endodermal sinus tumor, or yolk sac tumor, is the most common malignant neoplasm of germ cell origin and usually occurs in infant testes or ovaries. This type of tumor appears most often in the ovaries or. Gastric adenocarcinoma with features of endodermal sinus tumor. Germ cells are the cells in the ovaries and testicles that develop into eggs ova in females and sperm in males. Teratocarcinoma embryonal carcinoma containing elements of immature teratoma. An endodermal sinus tumor of the ovary diagnosed in pregnancy. These tumors usually present in childhood or young adulthood as testicular or ovarian masses. Mediastinal endodermal sinus tumors genetic and rare. Endodermal sinus tumor definition of endodermal sinus. Only seven cases of est involving the vulva are reported. Endodermal sinus tumors est of the lower female genital tract are uncommon malignancies.
Endodermal sinus tumor wikimili, the free encyclopedia. Endodermal sinus tumor in children journal of pediatric surgery. Malignant germ cell tumors are rare tumors of the childhood accounting for less than 3 % of pediatric malignancies. Waikuen ng, in comprehensive cytopathology third edition, 2008. The authors add a third case with the tumor located in the anterior third ventricular suprasellar region to the literature. The incidence of endodermal sinus tumor est or yolk sac tumor yst elements has been studied in two series of testicular germ. Even when the tumor has spread further or if it comes back later, surgery, radiation therapy, andor chemotherapy are effective in controlling or curing the disease in about 90% of patients. It can also appear in the uterus, abdomen, vagina, liver or brain. Endodermal sinus tumor an overview sciencedirect topics.
In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. Yolk sac tumor also termed endodermal sinus tumor malignant develop as a result of differentiation of primitive malignant germ cell elements in the direction of yolk sac or vitelline structures. Endodermal sinus tumor of lie infant vagina, gynecol. Pdf endodermal sinus tumor of the vagina researchgate. The optimal treatment is the surgical resection of the tumor, followed by adjuvant chemotherapy including bleomycin, etoposide and cisplatin 3, however, the results are poor.
Successful treatment of mediastinal germ cellendodermal. Primary endodermal sinus tumor presenting with spinal cord. And what i mean by that is the fact the most common most common ovarian tumor in a girl less than five years of age, the most common testicular tumor in a. The older term endodermal sinus tumor is no longer used because this structure is not a universal finding in yolk sac tumors. Endodermal tumor of the paranasal sinuses is quite rare and symptoms arising from paranasal sinuses may include nasal congestion, difficulty in moving eyes, headache or vision problems. Endodermal sinus tumor is an uncommon malignant germ cell tumor that occurs in both gonadal and extragonadal tissues. He presented with a solid mass in the upper abdomen and a markedly raised serum.
Thirtyfive patients were included in the study, 14 with immature teratoma, 11 with dysgerminoma, eight with endodermal sinus tumor, and two with mixed germ cell tumor. Symptoms will vary depending on the exact location of the tumor. Endodermal sinus tumor yolk sac tumor of the ear jama. The other two layers are the ectoderm outside layer and mesoderm middle layer, with the endoderm being the innermost layer. Malignant germ cell tumors account for about 3% of neoplasms in children, and endodermal sinus tumor est is the most common histological subtype. The tumor was present in a developmentally delayed child with an abnormal temporal bone and exhibited histopathologic and immunocytochemical features identical to those of endodermal sinus tumors of gonadal origin. First, no one should feel bad about being confused by this term, since this name has an obscure historical origin from the days of the tumors discovery. They are alive at 11 and 20 months, respectively, postoperatively. Endodermal sinus tumour of the omentum in a child springerlink. We report a 3yearold boy with an endodermal sinus tumour arising in the greater omentum, which may be the second reported case in the english literature. An endodermal sinus tumor, also called a yolk sac tumor, is a type of germ cell cancer. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
Gk, endon, within, derma, skin pertaining to the inner of the three layers of the embryo and the epithelial lining of the respiratory system, the digestive tract, and other tissues. Germ cell tumors of the ovary archives of pathology. Pdf integrated 18ffluorodeoxyglucose positron emission. Laparoscopic biopsy showed endodermal sinus tumour. Endodermal sinus tumor yolk sac carcinoma, a germinal neoplasm, is rarely primary in the cranial cavity. Jul 28, 2004 endodermal sinus tumour usually arises in a gonad.
Others have considered endodermal sinus tumor and embryonal. Most of these tumors are found in the vagina or in the cervix. Definition of endodermal sinus tumor nci dictionary of. For language access assistance, contact the ncats public information officer. Endodermal sinus tumor yolksac carcinoma of the orbit. Endoderm is one of the three primary germ layers in the very early embryo. Endodermal sinus tumor, also known as yolk sac tumor, is a rare malignant ovarian tumor that usually occurs in the second decade of life. 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. Primary endodermal sinus tumor of the vulva in a 52yearold woman with longterm survival. They can occur in the testis, ovaries, uterus, abdomen, thorax, tailbone region, vagina, liver, retroperitoneum and pineal ventricle of the brain. Endodermal sinus tumor, also known as yolk sac tumor, mainly arises from germ cells of ovary or testes. Endodermal sinus tumor online pathology video online. This type of tumor appears most often in the ovaries or testes.
Endodermal sinus tumor of the ovary in an 86 year old woman. Yolk sac tumor or endodermal sinus tumor is the most common malignant element in sct. Analysis of ascites revealed high levels of afp and ca 125. The new term, primitive endodermal tumors, simplifies the understanding of the complex histology of the yolk sac tumor group, as this terminology encompasses its multiple endodermal differentiations. Endodermal sinus tumor has shown a significant increase p endodermal sinus tumors of the ovary are particularly aggressive.
Some authors have included endodermal sinus tumors in the broader catagory of ovarian teratomas and grouped all teratomatous lesions together in discussing treatment and survival lo. Endodermal sinus tumor yolksac carcinoma is a type of malignant germcell tumor of the ovary and testis which on rare occasion occurs as a primary tumor elsewhere. Endodermal sinus yolk sac tumor elements in testicular. Endodermal sinus tumor and choriocarcinoma resemble extraembryonic tissues, and the embryonal carcinoma appears similar to fetal embryonal tissue. Chorionic tumor patient alphafetoprotein, gonadotropin,t histologic initial no. Endodermal sinus tumor of the parotid gland in a child. Primary orbital endodermal sinus tumor yolk sac tumor jaapos. Primary endodermal sinus tumor est of the mediastinum has been regarded as a rare and rapidly fatal germ cell neoplasm. Endodermal sinus tumor of the vagina a rare entity. Endodermal sinus tumors occur most often in children. They tend to grow quickly and spread to other parts of the body if not treated. Endodermal sinus tumors yolk sac tumor consist of glomeruloid structures composed of a space lined by tumor cells with an invaginated vascular pedicle covered by a. We report what is, to our knowledge, the first known case of an endodermal sinus tumor of the ear. In contrast to the pure form typical of infants, adult endoderma.
Pdf endodermal sinus tumor of the vagina and cervix. Endodermal sinus tumor of the ovary presents as a large complex abdominal mass that can be predominantly cystic and should be considered in the. Treatment with peb regimen induced both tumor regression and. Eosinophilic, paspositive, diastaseresistant hyaline bodies of different sizes are present in most ysts, being most numerous in areas with a reticular or hepatoid pattern. The tumor was present in a developmentally delayed. Primary yolk sac tumor endodermal sinus tumor of the vulva. Intracranial seminoma commonest histology 6070% of all germ cell tumors.
Serumcsf tumor markers bhcg, afp csf cytology biopsy biopsy may not be required when tumor markers are characteristically elevated, however patients will often require surgical intervention due to hydrocephalus pineal tumors or visual field deficits suprasellar tumors. Endodermal sinus tumor est is a member of the germ cell tumor group of cancers. Gastric adenocarcinoma with features of endodermal sinus tumor 4783. Endodermal sinus tumors are the most common malignant germ cell tumor in children. Endodermal sinus tumor yolk sac tumor and choriocarcinoma these very rare tumors typically affect girls and young. Extragonadal yolk sac tumor of the vulva first, it was a rare case of mixed germ cell tumors with components of endodermal sinus tumor and embryonal carcinoma which is the least common 16%. We report a case of a 16monthold girl with a primary endodermal sinus tumor est of the parotid gland.
It is the most common testicular tumor in children under 3, and is also known as infantile embryonal carcinoma. Endodermal sinus tumors, also known as ysts, belong to an inferior class of germ cell tumors gcts with a poor prognosis. In contrast to the pure form typical of infants, adult endodermal sinus tumors are often found in combination. Endodermal sinus tumor est is the most common histologic subtype of malignant gct. Endodermal sinus tumor of the vagina terenziani 2007. Case report 1 cg was a 19yearold, nulligravida white female, who had a normal menstrual. Endodermal definition of endodermal by medical dictionary. Type 2 sacrococcygeal teratoma endodermal sinus tumor. Endodermal sinus tumor of the paranasal sinuses request pdf. Definition of endodermal sinus tumor national cancer institute. Endodermal sinus yolk sac tumor elements in testicular germ.
Feb 10, 2016 sdbs are usually sparse, but when numerous, create a distinctive endodermal sinus pattern. The tumor resolved after chemotherapy, and the patient remained alive without evidence of disease at the time of this writing. Malignant germ cell tumors of the vagina represent a critical site for local treatment. Endodermal sinus 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. Cells migrating inward along the archenteron form the inner layer of the gastrula, which develops into the endoderm. So if you see them, they are pretty much diagnostic of yolk sac tumor, but if you dont see them you still havent ruled it out. Although rare, yolk sac tumor previously denoted as endodermal sinus tumor is the second most common malignant germ cell tumor of the ovary that affects children and adolescents. The final pathology report revealed an endodermal sinus tumor, also known as a yolk sac tumor. Endodermal sinus tumors of the ovary are particularly aggressive. A form of malignant germ cell tumor that occurs mainly in young children. Endodermal sinus tumor and embryonal carcinoma of the ovary.
The case initially presented as a medulloblastoma and the patient was followed up for six months. More than 50% of the patients died within a year of diagnosis. Three of them present special clinical and pathologic features that further substantiate the extraembryonic origin of this teratoid tumor. The chemosensitivity of the component of the endodermal sinus tumor patterns has tended to determine pathological recurrence, and a majority of cases of ovarian endodermal sinus tumor associated with endometrioid or mucinous neoplasms in postmenopausal women have resulted in death secondary to disease nawa et al. Radiology of primary intracranial yolksac endodermal sinus. Request pdf endodermal sinus tumor of the paranasal sinuses we report a rare case of endodermal sinus tumor est of the paranasal sinus in a 59yearold man with a 6week history of nasal. Endodermal sinus tumor of the vagina, the role of primarv chemeotherapv cancer, 1985, 56. Endodermal sinus tumor of the ovary 207 treated cases is then undertaken and indicates that a note of optimism can now be sounded regarding the future outlook for patients with this disease. Nov 29, 2011 yolk sac tumor or endodermal sinus tumor is the most common malignant element in sct. Endodermal sinus tumors of the head and neck, exclusive of the central nervous. A case of vaginal endodermal sinus yolk sac tumor resulting in hydrometra in a 9monthold female infant is reported. Endodermal sinus tumor is an uncommon malignant germcell neoplasm. Endodermal sinus tumor has shown a significant increase p tumors of the vagina represent a critical site for local treatment.
Chemotherapy of endodermal sinus tumour yolk sac tumour. May 19, 2017 so if you see them, they are pretty much diagnostic of yolk sac tumor, but if you dont see them you still havent ruled it out. Diagnosis of an est was established on the hematoxylin and eosinstained. The majority of tumors secrete afp, and there is a good correlation between the levels and the extent of disease.
One patient had a residual lesion that was biopsed twice and no viable tumor cells were ever found. Recently described autoimmune encephalitis due to antibodies against the nmethyldaspartate receptor has become the most frequent. Extragonadal yolk sac tumor of the vulva cases of mixed embryonal carcinoma and endodermal sinus tumor est are rare and reported just in a few case reports 6. If you have problems viewing pdf files, download the latest version of adobe reader. Endodermal sinus tumor symptoms, diagnosis, treatments and. Radiology of primary intracranial yolksac endodermal. We performed this study to determine the imaging characteristics of this tumor. First, no one should feel bad about being confused by this term, since this name has an obscure historical origin from the days of the tumor s discovery. Endodermal sinus tumor of the ovary is a rare complex malignant tumor that occurs in young women.