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Lectin-binding heterogeneity of alphafetoprotein (AFP). An observation in nude mouse xenografts of endodermal sinus tumors and in pediatric surgical patients.

We determined by affinity chromatography on concanavalin A-Sepharose the carbohydrate variant patterns of alphafetoprotein in the sera of 15 infants and children with endodermal sinus tumors (five cases), a neonatal mature teratoma (one case), hepatoblastomas (two cases), pancreatic carcinoma (one case), biliary atresia (four cases), neonatal hepatitis (one case) and neonatal hyperbilirubinemia (one case), in the sera from four normal neonates, and in the sera from two kinds of nude mice bearing human endodermal sinus tumors. Sera from patients with endodermal sinus tumors and pancreatic carcinoma were found to contain a relatively high proportion (48.4 +/- 4.5 and 52.6%) of alphafetoprotein which did not bind to concanavalin A. Sera from nude mice with human endodermal sinus tumors contained AFP, 96.2% of which did not bind to concanavalin A. Sera from patients with other lesions (nine cases) and from normal neonates, whose AFPs are all presumed to be of hepatic origin, contained much less (5.9 +/- 3.6%) of the concanavalin A non-binding AFP variant. These results indicate that human AFP has three distinct patterns of reactivity with concanavalin A and that studies in xenograft models may give important information relating to the glycosylation and secretion process of AFP.

Adolescent↗

Endodermal sinus tumor of the pineal region.

Two cases of endodermal sinus tumor of the pineal region are reported and compared with 16 others in the literature. The preponderance of neoplasms occurs in boys, usually in their second decade. The diagnosis is usually established within three months of the onset of non-specific symptoms of an intracranial mass. Treatment with operation or radiotherapy alone is inadequate. Most patients have died within two years, some with widespread intradural metastases. The one individual who has survived five years was treated with both operation and radiotherapy.

Adolescent↗

Extragonadal endodermal sinus tumors in children: a review of 24 cases.

Endodermal sinus tumors (EST) are highly malignant lesions with a tendency to recur locally and with a high incidence of metastatic disease at the time of diagnosis. The clinical and radiographic spectra of 24 patients with extragonadal EST are reported. There were 17 females and seven males and the mean age of presentation was 21 months. The primary lesion occurred in the sacrococcygeal region in 16 patients; in the vagina in two patients; in the anterior mediastinum in two patients; and in the bladder, liver, nasopharynx and posterior cranial fossa in one patient each. Computed tomography (CT) is the most accurate study in delineating the extent of primary lesions particularly in the pelvis and head and neck and also for demonstrating metastatic disease in the lungs. Significant improvement in survival rates is obtained with a combination of surgery, chemotherapy and radiotherapy.

Child, Preschool↗

Combination chemotherapy with cisplatin, vinblastine, and bleomycin for endodermal sinus tumor of the ovary.

Four consecutive patients with endodermal sinus tumor of the ovary were treated following surgery with intensive combination chemotherapy consisting of cisplatin, vinblastine, and bleomycin. All four of these patients remain continuously free of recurrent disease from 22 to 43 months following their original diagnosis. Two patients developed severe pulmonary toxicity related to the administration of bleomycin. Both of these patients also experienced Raynaud's phenomenon. The two patients whose initial surgery consisted of unilateral salpingo-oophorectomy have regular, spontaneous menstrual cycles, and one of them has become pregnant twice following the completion of chemotherapy.

Adolescent↗

Endodermal sinus tumor of the infant vagina.

A case of endodermal sinus tumor of the infant vagina is reported with long-term survival after successful therapy by surgery, chemotherapy, and radiation. The previous 26 reported cases are reviewed and the problems of therapy and long-term management are discussed. Therapy of these tumors should be monitored by alpha-fetoprotein radioimmunoassay.

Antineoplastic Combined Chemotherapy Protocols↗

Primary mediastinal endodermal sinus tumors: CT evaluation.

Five patients with primary mediastinal endodermal sinus tumor have been evaluated by computed tomography (CT). The tumors were all located in the anterior mediastinum. Prechemotherapy CT demonstrated large, irregularly enhancing inhomogeneous masses without fat components. Calcification was visualized in two tumors. Some parts of the pleura-lung interface were irregular in four cases. Obliteration of fat planes to pericardium and chest wall was evident in all cases, and there was infiltration of intercostal muscles in four. Postchemotherapy CT in two patients who responded to chemotherapy showed either newly developed cystic lesions or cystic change of the original mediastinal mass. The cystic masses had more regular borders, a homogeneous hypodense center, and a smooth peripheral rim of contrast-enhancing tissue.

Adolescent↗

Successful treatment of a pineal endodermal sinus tumor. Case report.

A patient with a pineal endodermal sinus tumor is presented who was successfully treated by a combination of surgery, adjuvant chemotherapy, and craniospinal irradiation. Two years after diagnosis, he is free of any disease. A review of the literature shows that such an outcome is very unusual. A multidisciplinary treatment is recommended for this rare tumor, using chemotherapy as adjuvant treatment.

Adolescent↗

[Clinical aspects of ovarian endodermal sinus tumor].

To study the clinical characteristics of ovarian endodermal sinus tumor, 22 patients with this disease who were managed at the Kurume University Hospital from 1970 to 1983 are analysed retrospectively. Before 1977 the patients received mainly radiation therapy as a postoperative treatment, and only two of 13 patients during this period survived. After 1978, multi-drug combination chemotherapy brought a remarkable improvement in the prognosis showing six survival cases out of 8 patients, and four of them are still alive with no evidence of the disease. Although the therapeutic efficacy of the combination of vincristine, actinomycin D and cyclophosphamide, or cis-diamminedichloroplatinum, vinblastine and bleomycin was evident as previously reported, half of the cases achieved a drug-resistance against these regimens during the first line therapy and this required a change in the program. Further, two drop out cases caused by the severe side effects of these aggressive treatment were noted. The best treatment for malignant germ cell tumors is not yet known, and a more effective and acceptable program is demanded to attain the goal of chemotherapy.

Adolescent↗

[Modified VAB-3 combination chemotherapy of stage IV endodermal sinus tumor of the ovary].

A case of pure endodermal sinus tumor (EST) of the ovary with huge multiple liver metastases was treated with modified VAB-3 protocol. Serum alpha-fetoprotein (AFP) before treatment was 137,775 ng/ml. A complete clinical response was achieved with rapid shrinkage of liver metastases and normalization of serum AFP. Second-look laparotomy preformed 17 months after the start of chemotherapy revealed no evidence of disease. VAB-3 combination chemotherapy, which was proved to be very effective in the treatment of metastatic testicular cancer, also seems to be effective in the treatment of advanced EST of the ovary. This is believed to be the first detailed report of the successful management of advanced EST of the ovary.

Adult↗

Second-look laparotomy in endodermal sinus tumor: a report of two patients with normal levels of alpha-fetoprotein and residual tumor at reexploration.

The role of second-look laparotomy in the management of patients with endodermal sinus tumor of the ovary is controversial. We report two women who converted to a normal alpha-fetoprotein (AFP) level during treatment with combination chemotherapy, yet were found to have residual endodermal sinus tumor at second-look laparotomy. In view of the limited experience with this rare disease, we continue to recommend second-look laparotomy for patients who have completed chemotherapy for endodermal sinus tumor of the ovary, regardless of the serum AFP level.

Adolescent↗

Teratoma associated with endodermal sinus tumor.

A 3-1/2-year-old girl was diagnosed as having a mediastinal endodermal sinus tumor with pulmonary, bony, and hilar lymph node metastases. Following 7 months of treatment with chemotherapy, thoracic CT (computerized tomogram) scan showed the presence of a residual mass. Upon further surgical exploration the residual mass, which was found to be intrapericardial and arising from the ascending aorta, was resected. This second lesion was shown to be a benign cystic teratoma. The combination of an intrapericardial teratoma and a mediastinal endodermal sinus tumor in a female child is probably unique. The possible etiology for the combination of these two pathologies is discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Endodermal sinus tumor of the ovary: a case series with flow cytometric DNA content analysis.

Endodermal sinus tumors (EST) are rare germ cell ovarian malignancies occurring primarily in young women. A retrospective review of the Magee-Womens Hospital tumor registry revealed eight cases of pure EST and two mixed tumors in which the EST component was predominant. Mean patient age was 18.2 years. Abdominal pain was the most common presenting symptom and a pelvic mass was palpable in all patients. Four patients are currently alive and well with no evidence of disease. All were treated with surgery and combination chemotherapy. Flow cytometric DNA content analysis of paraffin-embedded tumor tissue identified similar aneuploid cell populations in three of five tumors analyzed with relative peak positions of 1.72, 1.62, and 1.70. The management of women with endodermal sinus tumor remains controversial with regard to type of chemotherapy employed and the use of second-look laparotomy. The prognosis role of flow cytometric DNA content analysis is yet to be determined.

Adult↗

Endodermal sinus tumor of the mediastinum. Cytologic diagnosis on a pleural effusion.

A primary mediastinal endodermal sinus tumor in a young man was diagnosed by cytologic examination of a pleural effusion. Subsequent evaluation revealed a greatly elevated serum alphafetoprotein (AFP); computed tomographic scan of the chest showed a large anterior mediastinal mass. Routine examination of the smears and cell block preparations revealed clusters of tumor cells with a few intracytoplasmic hyaline droplets. Immunohistochemical stains for AFP, alpha-1-antitrypsin and cytokeratin were positive in the tumor cells while stains for carcinoembryonic antigen and the beta subunit of human chorionic gonadotropin were negative. This supported the diagnosis of endodermal sinus tumor, a rare primary tumor within the mediastinum.

Adult↗

Endodermal sinus tumor of the mediastinum. Ultrastructural study.

This is the eighth case report of an endodermal sinus tumor of the mediastinum (first case studied by electron microscopy). The ultrastructure of the tumor was found to mimic that of the normal yolk sac and was also similar to that of three previously reported cases of endodermal sinus tumor of the ovary, thus confirming the correctness of Teilum's original interpretation of the tumor as arising in germ cells and differentiating towards extraembryonic structures.

Adolescent↗

Treatment of endodermal sinus tumor in children using a regimen that lacks bleomycin.

BACKGROUND: The EPO-VAC protocol was initiated to study 1) the efficacy of adding a cisplatin regimen (EPO) to VAC alone (the previous standard of care) and 2) the effect of replacing bleomycin with etoposide in the treatment of pediatric endodermal sinus tumors. METHODS: The eligibility requirements for entry included age <21 years at diagnosis, diagnosis of a primary gonadal or extragonadal tumor (excluding central nervous system tumors and stage I testicular tumors), and histological confirmation of endodermal sinus tumor. Children who met the eligibility criteria were treated with four courses of EPO (etoposide, cisplatin, vincristine) alternating with three courses of VAC (vincristine, dactinomycin, and cyclophosphamide). RESULTS: Eleven children were entered on the protocol. Six patients had extragonadal disease, five patients had ovarian primaries. Seven patients had low-stage tumor (I or II) and four had advanced-stage tumor (III or IV). Three of six evaluable patients attained a complete response at 21 weeks. Three patients with a residual soft tissue mass at restaging underwent further therapy. No patient has relapsed after a median of 51 (range 14-88) months of follow-up. CONCLUSIONS: The results of this protocol suggests that a cisplatin-containing regimen that lacks bleomycin is active in childhood endodermal sinus tumors.

Adolescent↗

Endodermal sinus tumor of the ovary: a paradoxical response to chemotherapy.

A patient with ovarian endodermal sinus tumor metastatic to the liver responded unusually to chemotherapy. She experienced histologically documented regression of all intraperitoneal disease with the exception of the liver metastases when given vinblastine, bleomycin, and cisplatin. She eventually died of liver failure. A review of patients with ovarian endodermal sinus tumor who either presented with initial liver metastases or had recurrent disease in the liver leads us to conclude that the liver is a preferential site for spread, that liver metastases carry an ominous prognosis, and that failure to see prompt liver metastatic tumor response after systemic chemotherapy should signal a need for a more aggressive therapeutic approach to the liver lesions.

Adult↗

[Primary endodermal sinus tumor of the fourth ventricle (author's transl)].

A case, a 18-year-old male, of an endodermal sinus tumor (yolk sac tumor) in the fourth ventricle, was reported. The patient had a month history of headache, vomiting and gait disturbance prior to the hospitalization, when he admitted to our service he was in lethargic condition with left cerebellar ataxia and horizontal nystagmus. Lumbar tap revealed clear CSF under normal pressure of 110 mm H2O with the CSF protein of 432.5 mg/dl and cell count of 147/3. The vertebral angiography demonstrated space occupying lesion in the posterior fossa. Plain CT demonstrated only disappearance of the fourth ventricle and slightly dilated bilateral ventricles and third ventricle. However diffuse high density area around the fourth ventricle was demonstrated and the wall of bilateral anterior horn was slightly enhanced, after injection of contrast media. There was no other abnormal findings around the pineal region. Suboccipital craniectomy was performed and the tumor was totally removed macroscopically. The tumor was situated in th floor of the fourth ventricle and infiltrated into the fourth ventricular wall and th adjacent cerebellar tissue. The tumor was with soft, greyish color and extremely vascular. Histologically the tumor was diagnosed as endodermal sinus tumor according to Teilum's classification. There were stellate cells arranged in a loose with vacuolated network which formed cystic cavities and a complicated network of honeycomb appearance with a system of communicating cavities and channels. Various size of intra- and extracellular PAS-positive hyaline globules were also seen. Glomerular-like structure (Schiller-Duval body) was not observed. Immunoperoxidase study clearly demonstrated the presence of intra- and extracytoplasmic alpha-fetoprotein granules in the tumor tissue. The amount of the serum alpha-fetoprotein, measured by radioimmunoassay, showed 400 ng/ml. After irradiation in the posterior fossa (5000 rad) the patient was discharged. Three months later, follow up CT demonstrated small high density area in the anterior horn of the left lateral ventricle, so he was rehospitalised. Irradiation in the whole brain was again administered. The tumor was very radiosensitive. CT, after 800 rad, demonstrated complete disappearance of the tumor. After irradiation totally (3000 rad), he discharged with left cerebellar ataxia.

Adolescent↗

[Endodermal sinus tumor of sacrococcygeal region in the infant--clinico-pathologic analysis of 6 cases].

Endodermal sinus tumor can be found in the ovaries, testes and extragonadal sites, such as sacrococcygeal, retroperitoneal, mediastinal regions or other mid line structures. Sacrococcygeal endodermal sinus tumor and its characteristics are presented. The age of patients with lesions in this region is youngest among all. Their ages ranged 10 months to 3 years in our series. Five of them were females. Most cases had difficulty in passing stools. When first seen in the clinic, all were in the advanced stage with the tumor infiltrating into the sacrococcygeal bones, presacral soft tissues and rectum already. The residual lesions recurred rapidly after operation. All cases were not treated systematically by chemotherapy or radiotherapy, and died within 1-15 months. Pathologically, the composition of the tumor is the same in every site, assuming patterns of reticular or solid, glandular or pseudopapillary appearance, Schillar-Duval bodies, hyaline bodies and so on. Positive serum AFP test may be helpful in the differential diagnosis and prognostic evaluation.

Child, Preschool↗