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Therapy of endodermal sinus tumor of the ovary.

Endodermal sinus tumor of the ovary can be differentiated histologically and immunohistochemically from ovarian embryonal cell carcinoma. A case report of a patient with endodermal sinus tumor is presented in which a long-term remission was achieved by unilateral adnexectomy and combination chemotherapy. Review of the current literature indicates that tumor removal followed by combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide is the most effective method of therapy for this highly malignant ovarian neoplasm. The addition of hysterectomy with contralateral ovariectomy or radiation therapy does not appear to significantly improve the survival of patients with this tumor. Serial plasma determinations of alpha fetoprotein provide biochemical monitoring of response to therapy and may be useful in predicting occult tumor recurrence.

Adolescent↗

Endodermal sinus tumor (yolk sac tumor) of the ear.

Endodermal sinus tumors (yolk sac tumors) are malignant germ cell tumors that usually arise in the gonads. We report what is, to our knowledge, the first known case of an endodermal sinus tumor of the ear. 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. The tumor resolved after chemotherapy, and the patient remained alive without evidence of disease at the time of this writing. The purpose of this report is to add a rare tumor to the differential diagnosis of neoplasms of the ear in children and to familiarize otorhinolaryngologists and head and neck surgeons with its pathologic features and clinical management.

Antineoplastic Combined Chemotherapy Protocols↗

Primary endodermal sinus tumor of the omentum.

Endodermal sinus tumor (EST) is a rare neoplasm which usually arises in the testis or ovary. But extragonadal EST is well recognized and may arise in a wide array of sites such as the mediastinum, vagina, and brain. We report a case of primary EST of the omentum and to our knowledge it is the first case of omental EST in the literature. A 45-year-old woman with a history of abdominal distension was treated with total abdominal hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy, followed by four cycles of BEP (bleomycin, etoposide, cisplatin) combination chemotherapy. Microscopically, the tumor exhibited typical patterns of EST and stained for alpha-fetoprotein and cytokeratin. She has remained free of disease for 10 months of follow-up. According to previous studies, debulking surgery and BEP chemotherapy were used as primary therapy with good results. The subject of extragonadal EST is reviewed, and the possible histogenesis of this tumor at such a rare site is discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Endodermal germ cell carcinoma (endodermal sinus tumor) of the vagina in infant girls.

An endodermal sinus tumor (endodermal germ cell carcinoma) was diagnosed in a 1-year-old girl in the vagina after hemorrhage; the tumor was completely removed by radical abdominal surgery. Postoperative polychemotherapy was performed for two years with Actinomycin D, Adriamycin, Vincristin, and Cyclophosphamide. The infant is now tumor-free for 26 months, showing almost normal somatic and psychic development. The characteristic histological patterns and clinical course of this strongly malignant tumor are demonstrated, based on 25 published case reports of endodermal sinus tumors in the vagina of little girls (aged 5-26 months). This neoplasm in early infancy has to be separated from the clear-cell adenocarcinoma of the vagina which occurs after puberty in adolescent girls and young women, and is induced by stilbestrol therapy to the mother during early pregnancy.

Adenocarcinoma↗

Endodermal sinus tumor of the ovary: imaging evaluation.

OBJECTIVE: Endodermal sinus tumor of the ovary is a rare complex malignant tumor that occurs in young women. We performed this study to determine the imaging characteristics of this tumor. CONCLUSION: Endodermal sinus tumor of the ovary presents as a large complex abdominal mass that can be predominantly cystic and should be considered in the differential diagnosis of complex abdominal masses in girls and young women. In addition, upon encountering a mass with these imaging characteristics in the proper clinical setting, serum alpha-fetoprotein levels should be measured to help make a presurgical diagnosis and to obtain a baseline value.

Adolescent↗

Endodermal sinus tumor of the mediastinum.

Endodermal sinus tumor (EST) of the mediastinum is a rare germ-cell neoplasm affecting mainly young adult males. Ten patients with EST were treated with a multimodality approach that included surgery, chemotherapy, and radiotherapy. All patients had relapses after achieving a transient response except one who is still in complete remission more than five years following the diagnosis of the disease. Optimal therapy for this neoplasm has yet to be discovered. The disease is a subset of extragonadal germ cell tumors which appears to be lethal in most cases, particularly when the primary tumor is unresectable. The first case of five-year disease-free survival is described.

Adolescent↗

Successful treatment of primary endodermal sinus tumor of the endometrium.

Endodermal sinus tumor (EST) arising in the endometrium is an extremely rare malignant neoplasm. To the authors' knowledge, this is only the second report of an EST arising in this location. The tumor was exophytic, 2.4 cm in diameter, and was located in the fundus uteri. It had infiltrated the circumambient endometrium slightly. Preoperative serum alpha-fetoprotein (AFP) levels were markedly elevated, 1580 ng/ml. The patient received a simple abdominal hysterectomy and bilateral salpingo-oophorectomy followed by combination chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide. To date, there has been no evidence of tumor recurrence, and serum AFP titers, which had returned to normal 7 weeks after surgery, continue to remain within normal limits. We believe this is the first successful treatment of a documented case of primary EST of the endometrium.

Adult↗

Endodermal sinus tumor.

Three cases of Endodermal Sinus Tumor are presented and its difference from tumors of the mesonephroma type and associations with the embryoma are pointed out. The three cases were "pure" types and the patients died within 5 months in spite of surgical treatment and chemotherapy. Autopsy showed extensive involvement of peritoneum, diaphragm and pleura.

Adolescent↗

Endodermal sinus tumor of the ovary.

Endodermal sinus tumor (EST) of the ovary is a germ cell tumor that is now recognized more frequently than in the past. Since problems concerning natural history, prognosis and therapy of these neoplasms are not entirely resolved, it should be underlined the need to report new cases. The authors describe four cases of EST with reference to serial alphafetoprotein determination by radioimmunoassay; they also emphasize the importance of this technique as a very sensitive method in monitoring therapy and in detecting recurrences of disease even before any clinical evidence.

Adolescent↗

A 62-year-old woman with endodermal sinus tumor of the ovary.

An endodermal sinus tumor of the ovary in a 62-year-old woman is described. This is the first case reported of which I am aware of endodermal sinus tumor of the ovary in a woman after the age of 60 years.

Antineoplastic Combined Chemotherapy Protocols↗

The histogenetic-embryologic basis for reappearance of alpha-fetoprotein in endodermal sinus tumors (yolk sac tumors) and teratomas.

The mechanism of neosynthesis of the human tumor-associated fetal antigen alpha-fetoprotein (AFP) in a variable percentage of patients with testicular, ovarian and extragonadal germ cell tumors has generally been considered unknown or beyond any simple explanation. Of decisive importance is the cellular basis for AFP production 1. in ontogenesis and 2. in malignancy as dependent on an exact tumor histogenesis. Based on (1) the histogenetic-embryologic classification of germ cell tumors and the concept of yolk sac tumor (or endodermal sinus tumor), (2) the available clinical and experimental observations, and (3) the immunofluorescent localization of AFP in the endodermal sinus tumor of the human testis, it is concluded that AFP synthesis in these neoplasms is explained by the fact that they contain yolk sac endoderm, which produce AFP analogous with the physiological AFP synthesis by the fetal yolk sac in early embryogenesis.

Adult↗

Ovarian mixed germ cell tumor composed of dysgerminoma, endodermal sinus tumor, choriocarcinoma and mature teratoma in a 44-year-old woman: case report and literature review.

A case of ovarian mixed germ cell tumor in a 44-year-old woman was examined. The tumor was well circumscribed, measured 15 x 11 x 10 cm and appeared solid and partly cystic on the cut surface. Light microscopic examinations revealed that the tumor was composed of four different neoplastic germ cell elements, intermingled with each other. They are: (i) choriocarcinoma, immunohistochemically positive for human placental lactogen (hPL) and human chorionic gonadotropin (hCG); (ii) dysgerminoma, positive for placental alkaline phosphatase; (iii) endodermal sinus tumor positive for alpha-fetoprotein (AFP); and (iv) mature teratoma. Among these histological types, dysgerminoma occupied more than 50% of the neoplasm. The patient was diagnosed as a stage la ovarian mixed germ cell tumor and was subsequently treated with chemotherapy. A second-look laparotomy after completion of chemotherapy revealed no residual tumors in the abdomen and the patient is alive and well 15 months after operation. This is the fourth reported case of ovarian mixed germ cell tumor arising in patients over 40 years old.

Adult↗

Endodermal sinus tumor: a clinical and pathological correlation.

Endodermal sinus tumors occur in the gonads and in a variety of extragonadal locations, particularly as the malignant component of sacral teratomas. Light and electron microscopic studies as well as the presence of alpha fetoprotein support the concept that these lesions arise from germ cells that migrate from the extraembryonic yolk sac. These tumors in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. Peritoneal implants are seen from tumors in the ovary, testis, and following ventriculoperitoneal shunts for yolk sac tumors of the pineal gland. There is no satisfactory therapy for the endodermal sinus tumor, regardless of location, when there are distant metastases at the time of diagnosis; however, we have documented complete regression of metastases in one child following therapy with vinblastine, bleomycin, and cis-platinum. Nine of 13 boys with endodermal sinus tumors of the testis survived in this series of patients. All survivors were treated with orchiectomy and lymph node excision. Surgical excision combined with chemotherapy and radiation led to survivors in children with large extragonadal pelvic and retroperitoneal tumors. We recommend vigorous multimodal therapy with surgery and chemotherapy. Radiation is given to the primary lesion when it is too extensive for initial resection or to eradicate residual disease following chemotherapy. Alpha fetoprotein is a valuable tumor marker.

Adolescent↗

Endodermal sinus tumor of the maxillary sinus: a case report.

Endodermal sinus tumor is an uncommon malignant germ cell tumor that occurs in both gonadal and extragonadal tissues. Endodermal sinus tumors of the head and neck, exclusive of the central nervous system, are rare. We report a case of endodermal sinus tumor arising from the maxillary sinus in a 4-year-old boy. Combined treatment with radiation therapy and chemotherapy resulted in complete tumor regression.

Child, Preschool↗

Sudden death due to pulmonary tumor embolism in a patient with endodermal sinus tumor of the ovary.

Only 2 cases of fatal pulmonary tumor embolism have been described in patients having malignant tumors of the ovary. Both were diffuse microtumor emboli in the pulmonary arteries of elderly individuals. We present a teen-aged patient who died suddenly due to a massive tumor embolism of the right main pulmonary artery after a two-year remission of an endodermal sinus tumor of the ovary. Upon her first admission, she was treated with radical operation and insufficient chemotherapy. Two years later, she was readmitted to our hospital because of the sudden onset of dyspnea and shock; and she died 5 hours after readmission. A case of endodermal sinus tumor of the ovary with fatal pulmonary tumor embolism is described.

Adolescent↗

Ovarian endodermal sinus tumor in a postmenopausal woman.

BACKGROUND: Ovarian endodermal sinus tumor is rare in postmenopausal women. CASE: We report the case of a 75-year-old woman with a pure endodermal sinus tumor of the ovary. CONCLUSION: We believe this to be the oldest patient reported with an ovarian endodermal sinus tumor. The histogenesis of this entity in older patients may be different than when it occurs in young women.

Age Factors↗

Clinical experience with conservative surgery for vaginal endodermal sinus tumor.

Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric malignancy, were managed with conservative surgery followed by adjuvant chemotherapy. The first case involved a 9-month-old girl with EST of the vagina, who was treated with a partial vaginectomy and VAC regimen (vincristine, actinomycin D, cyclophosphamide) during a 2-year period. The serum alpha-fetoprotein (AFP) level returned to normal after excision of the tumor, and it remained normal throughout the treatment period. There was no evidence of disease 30 months after diagnosis. The second case involved an 8-month-old girl with EST of the vagina, who was treated with local excision of the mass through a vaginotomy. The VAC regimen was administered, but the serum AFP level remained elevated. A follow-up abdominopelvic computed tomography scan, taken 4 months after the operation, showed local recurrence of the tumor. The VAC regimen was then changed to a BEP regimen (bleomycin, etoposide, cisplatin). The serum AFP level returned to normal after 2 courses of the new regimen, and no tumor was visible on the follow-up magnetic resonance imaging study. For vaginal EST, primary conservative surgery and adjuvant chemotherapy are attractive measures to preserve both reproductive and sexual function. The extent of conservative surgery requires at least a partial vaginectomy. Simple tumor excision may not be adequate to achieve cure or to prevent local recurrence, even with adjuvant chemotherapy. The serum AFP level is useful for diagnosing and monitoring vaginal EST in the infant.

Antineoplastic Combined Chemotherapy Protocols↗