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The incidence of yolk sac tumor (endodermal sinus tumor) elements in germ cell tumors of the testis in adults.

The incidence of yolk sac tumor (endodermal sinus tumor) elements was studied in 147 germ cell neoplasms of the testis in adults observed over a 4 1/2-year period. Excluding 79 cases of pure seminoma, yolk sac tumor elements were found in 26 (38%) of 68 tumors; in 8 tumors the yolk sac tumor was the predominant element. Yolk sac tumor elements were found admixed with all other germ cell tumor elements. Tumors composed entirely of yolk sac tumor were not encountered. Fifteen (57%) of the 26 patients with tumors containing yolk sac tumor elements have died of their disease during the period under study, compared with 13 (31%) of the remaining 42 patients, suggesting that the prognosis of adults with testicular tumors containing yolk sac tumor elements is unfavorable. This is in contrast to the relatively good prognosis of infants and young children with testicular yolk sac tumor, and similar to the prognosis of female patients with ovarian yolk sac tumor. The results of the present study give further support to the view that yolk sac tumor (endodermal sinus tumor) is not adistinctive neoplasm of the infant's testis, but a germ cell neoplasm differentiating in the direction of yolk sac or vitelline structures, and occurring in the testis, ovary and extragonadal sites in common with other germ cell neoplasms.

Adolescent

Yolk sac tumors (endodermal sinus tumors) of the extracranial head and neck regions.

Yolk sac tumors (or endodermal sinus tumors) are rare neoplasms of germ cell origin that have been reported in gonadal sites (testis and ovary). Extragonadal yolk sac tumors are uncommonly seen, and in particular, they are rare in the extracranial head and neck regions; we found 27 well-documented cases in the English-language literature. We discuss their clinical and pathologic features.

Adolescent

Bleomycin, vinblastine, and cis-platinum in the treatment of advanced endodermal sinus tumor.

The endodermal sinus tumor has traditionally been associated with an exceedingly poor prognosis. Three women with advanced pure endodermal sinus tumors were treated with a combination of bleomycin, vinblastine, and cis-platinum (VBC). Serum alpha-fetoprotein levels were monitored for all patients during and after therapy, and in each case the alpha-fetoprotein returned to normal range, correlating with complete clinical remission. Second-look laparotomy was negative for tumor in each case. Two patients have had no overt sign of recurrence 12 months after cessation of therapy. The third patient had post-treatment elevation of alpha-fetoprotein levels, and eventually was found to have recurrent tumor 6 months after chemotherapy was stopped. The VBC combination, previously found effective in testicular germ cell tumors, is also effective in ovarian germ cell tumors.

Adolescent

Endodermal sinus tumor of the pineal region: case report.

A 13-year-old boy presented with symptoms and signs of a posterior 3rd ventricle tumor associated with raised levels of serum and cerebrospinal alpha-fetoprotein. The patient underwent subtotal resection of the tumor followed by craniospinal radiation. Histopathological examination revealed a pure endodermal sinus tumor. Endodermal sinus tumors represent a rare type of germ cell tumor, only 13 intracranial cases having been reported in the literature.

Adolescent

Ultrastructure and immunohistochemical staining of a transplanted endodermal sinus tumor.

An endodermal sinus (yolk sac) tumor was successfully transplanted into athymic nude mice. Histologic and ultrastructural investigations revealed that the transplanted tumor had a characteristic appearance with numerous Shiller-Duval bodies, endodermal sinus structures and ultrastructural profiles as previously described in human material. The endodermal sinus tumor and normal human yolk sac have been found to synthesize not only alpha-fetoprotein (AFP), but also other serum proteins, namely, albumin, prealbumin, alpha 1-antitrypsin, and transferrin. Serological study by radioimmunoassay demonstrated AFP, carcinoembryonic antigen (CEA) and human chorionic gonadotropin (HCG) in the sera of the tumor-bearing nude mice and in cyst fluid from the transplanted tumor. Immunohistochemical investigation using the unlabeled antibody peroxidase-antiperoxidase method showed using the unlabeled antibody peroxidase-antiperoxidase method showed that the tumor cells produced CEA, alpha 1-antitrypsin, transferrin, HCG as well as AFP. These immunohistochemical staining properties were correlated with the findings on radioimmunoassay.

Adult

Conservative management of testicular endodermal sinus tumor in childhood.

Endodermal sinus tumor is the most common testicular neoplasm in childhood. The management of children with this neoplasm remains controversial. We have treated prospectively 5 children with stage I endodermal sinus tumor with limited surgery and no adjuvant therapy. The median patient age at diagnosis was 21 months (range 5 to 24 months). All children underwent an inguinal orchiectomy with high ligation of the spermatic cord. Retroperitoneal node dissection was not performed in any case and no child received adjuvant chemotherapy or radiation therapy. All patients were well without evidence of recurrent disease at a median followup of 46 months (range 19 to 72 months). Because these tumors usually are localized at the time of diagnosis, rarely spread to the retroperitoneal nodes and have a biological marker in most cases, and because good salvage chemotherapy is available for patients with relapse, we believe that nonmetastatic testicular endodermal sinus tumors in children can be managed with radical orchiectomy alone. Retroperitoneal node dissection is not necessary and adjuvant therapy is not indicated if markers return to normal. Further treatment should be reserved for the rare child with relapse.

Castration

Endodermal sinus tumors in the head and neck region.

Extragonadal germ cell tumors of the head and neck region account for only 5% of all benign and malignant germ cell tumors. Endodermal sinus tumors (EST) of the head and neck region are rare. We report three patients with EST of head and neck region over a period of 10 years; the primary sites of tumor were orbit, maxillofacial region and retroauricular region. Histopathological examination revealed malignant teratoma with predominant endodermal sinus pattern in two, and pure EST in one patient. Serum alpha fetoprotein (AFP) was elevated in all three patients. Two patients had initial surgery but did not receive adjuvant chemotherapy, as the parents refused it. Partial remission was achieved in the other patient who received chemotherapy (cisplatin, bleomycin and vinblastin) and the patient died of infection after four courses of chemotherapy.

Endodermal Sinus Tumor

[Evaluation of the results of treatment of endodermal sinus tumor in children].

Endodermal sinus tumor is a rare kind of cancer in children. In Clinical Department of Pediatric Oncology there were treated 8 children with endodermal sinus tumor (EST). 4 of them were localised in sacrococcygeal region, 3 in genital system and 1 in testis. In all these cases high level of AFP was observed. Complex therapy was done including surgery, chemotherapy and rtg-therapy, giving as result 3 years survival in 1 only case. Another case is still in course of the treatment. Unified protocol of therapy is not founded till now and prognosis in majority of children is not satisfied.

Antineoplastic Combined Chemotherapy Protocols

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

The endodermal sinus tumor is a malignant germ cell neoplasm that usually arises in gonads, but on rare occasion occurs in extragonadal locations. Five tumors of the orbit, which by light microscopy displayed features characteristic of gonadal endodermal sinus tumor, were tested for the presence of alpha fetoprotein using paraffin-embedded tissue and an immunoperoxidase technique. Each tumor contained intracytoplasmic alpha fetoprotein. Review of the clinical histories revealed that orbital endodermal sinus tumors differ from other extragonadal endodermal sinus tumors in that they occur at a younger age and when treated aggressively can result in long-term survival. In children with rapidly progressive proptosis the distinction between endodermal sinus tumor and rhabdomyosarcoma poses diagnostic difficulties for both the clinician and the pathologist. When considering the diagnosis of endodermal sinus tumor, the use of immunohistochemical testing for alpha fetoprotein can be helpful.

Child, Preschool

Primary intracranial endodermal sinus tumor. Case report.

Endodermal sinus tumor (yolk sac carcinoma), a germinal neoplasm, is rarely primary in the cranial cavity. The authors add a third case with the tumor located in the anterior third ventricular (suprasellar) region to the literature. Seventeen previously reported intracranial cases, mostly in the pineal region, are briefly reviewed.

Adolescent

Hepatoid yolk sac tumor of the ovary (endodermal sinus tumor with hepatoid differentiation): a light microscopic, ultrastructural and immunohistochemical study of seven cases.

Seven cases of ovarian yolk sac tumor (endodermal sinus tumor) with patterns resembling those of hepatocellular carcinoma were encountered in patients 7-43 years of age. Two of the patients had gonadal dysgenesis with a 46XY karyotype. At operation three tumors were confined to the ovary and four were associated with intra-abdominal metastases. Two of the Stage I tumors recurred within one year. The hepatoid pattern was a prominent feature of all the tumors and was exclusive in four of them. In one specimen it merged almost imperceptibly with a polyvesicular vitelline pattern. The hepatoid component of the tumors was characterized by discrete masses, nests and/or broad bands of large polyhedral cells with central nuclei and prominent nucleoli; gland-like spaces, some of which contained mucin, were occasionally evident. Each tumor contained numerous PAS-positive, diastase-resistant intracytoplasmic and extracytoplasmic hyaline bodies. Alpha-fetoprotein and alpha-1-antitrypsin were identified by immunoperoxidase and immunofluorescence techniques in four tumors and albumin in two. Immunoperoxidase stains for chorionic gonadotropin were negative in four cases. Ultrastructural analysis of two specimens disclosed features similar to those of hepatocellular carcinoma.

Adolescent

Endodermal sinus tumor of the ovary. The orphan tumor.

The endodermal sinus tumor of the ovary is a rare and highly lethal germ cell tumor. At least 10 different names have been applied to it, and so long as its name remains unsettled the collection of information will continue to be greatly impeded. One of the patients who came under our observation died after 21 months despite surgical removal of all evident disease followed by radiation and chemotherapy; the other patient, whose tumor could be removed only partially, and who received intensive chemotherapy for 4 months but refused all treatment thereafter, is well without evidence of disease 3 years after the initial diagnosis was made. Despite the dismal outlook for patients with this tumor, a few encouraging features emphasize the need to report new cases as they appear, and to continue the search for agents that will control it.

Adolescent

Clear cell (mesonephroid) tumors of the ovary with characteristics resembling endodermal sinus tumor.

Twenty-five ovarian clear cell tumors and six endodermal sinus tumors were studied by histochemical, immunohistochemical, and ultrastructural methods. The results of the study indicate that some clear cell tumors contain periodic acid Schiff (PAS) positive and diastase resistant hyaline globules, and also basement membrane-like material, which have been previously considered as being observed only in endodermal sinus tumor. In view of this, we would like to emphasize the importance of the presence of the various histological patterns observed in endodermal sinus tumor, in addition to the classical perivascular formations (Schiller-Duval bodies) and the presence of alpha-fetoprotein (AFP), as diagnostic features of endodermal sinus tumor. On the other hand, the presence of glycogen, abundant rough endoplasmic reticulum stacked in parallel rows, and a more uniform histological pattern are indicative of clear cell (mesonephroid) tumor of the ovary.

Adult

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