SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Glucagon-producing cells differentiate in allantoic endoderm cultured on the chorio-allantois in association with intestinal or pancreatic mesoderm. In addition, goblet cells can be recognised among the epithelial cells.
The case presented is a 17-year-old male patient with a pineal region endodermal sinus tumor manifested by obstructive hydrocephalus and upward gaze limitation. The highly aggressive nature of this rare malignancy was evidenced by spontaneous tumor bleeding. The patient was treated with a cerebrospinal fluid (CSF) shunt and microsurgical tumor excision followed by irradiation and combination chemotherapy consisting of cisplatin, vincristine, methotrexate and bleomycin. The response to therapy was monitored by serial measurements of serum and CSF alpha-fetoprotein level. The patient remains free of any clinical or radiographic evidence of tumor recurrence 38 months after the initial diagnosis. This experience supports the rationale for an aggressive multidisciplinary approach toward this rare and highly malignant neoplasm.
Endodermal sinus tumor of Cervix is an extremely rare cause of vaginal bleeding. Because of its rarity and interesting features, it is reported with a brief review of literature.
Primary extragonadal mediastinal endodermal sinus tumour is rare, and to date very few cases have been reported in the literature. We present here a case of rare extragonadal highly malignant commonest germ cell tumour in an infant who presented with a rapidly progressive mediastinal mass with dry non-productive cough, tachypnea without significant respiratory distress or toxicity.
We report two cases of vaginal bleeding in infants caused by endodermal sinus tumours of the vagina. The clinical features, diagnostic laboratory investigations and prognosis of this rare paediatric malignancy are discussed.
Serum AFP was determined serially by radioimmunoassay in 13 patients with ovarian germ cell tumors and in one patient with bilateral pure gonadoblastoma. There were 4 patients with pure dysgerminoma, one with pure endodermal sinus tumor (EST) and 8 with mixed germ cell tumors, all containing EST. The patients with dysgerminoma and gonadoblastoma had normal serum AFP at all times. All patients with tumors containing EST had raised serum AFP, although in most cases it was first determined between 1 and 3 weeks after operation and there was no evidence of metastases. Serum AFP became normal 5 to 7 weeks after operation and began to rise when disease recurred. Serum AFP determinations detected presence of recurrent disease long before it became detectable by other methods. Serum CEA was determined serially by radioimmunoassay in 8 of these patients, including 2 who dies with metastases, and was normal on all occasions.
A primary yolk-sac (endodermal sinus) tumor of the prostate occurring in a 51-year-old man is described, and the difficulties associated with classification of this tumor are discussed. Yolk-sac tumors have been reported to arise in several extragonadal sites, but to our knowledge this is the first documented case of a germinal cell tumor occurring the the prostate. This tumor in extragonadal sites carries a grave prognosis. Despite radical surgery, radiotherapy, and chemotherapy, this patient died 10 months after the tumor was diagnosed.
A review of the histology, clinical findings and results of therapy in 9 females with endodermal sinus tumor (EST) is presented. Five patients had histologically pure EST; 4 had EST mixed with other germ cell components. The site of primary tumor was the ovary in 8 of the 9 females; the remaining patient with an extraovarian primary represents the first reported case of EST arising in the vulva. The addition of combination chemotherapy has prolonged survival over historical controls treated with surgery or surgery plus irradiation. Adjuvant chemotherapy appears warranted as treatment for occult metastatic disease; postoperative radiation therapy appears useful in providing local control of primary disease. There is a suggestion of increased sensitivity of EST to combination chemotherapy as compared to other germ cell histologies with which it is commonly admixed.
This is a case report of an endodermal sinus tumor (EST) arising in the pineal region of a 19-year-old girl (first case studied by electron microscopy). The ultrastructure of the tumor was similar to that of the previously reported cases of EST of ovaries, mediastinum, and sacrococcygeal area. A characteristic basement membrane-like material has been identified within endoplasmic reticulum of tumor cells and in intercellular spaces. Alphafetoprotein has been detected in the spinal fluid of the patient by radioimmunoassays and within tumor tissue by immunohistochemical techniques. Combination therapy consisting of radiation, surgical decompression, chemotherapy (BCNU), and prednisone has been employed in the treatment of this patient without significant benefit. The patient expired three months after the surgery, no autopsy was obtained.
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.
The incidence of endodermal sinus tumor (EST) or yolk sac tumor (YST) elements has been studied in two series of testicular germ-cell neoplasms in adults. One series, consisting of 200 germ-cell neoplasms seen from 1053 through 1968, was studied retrospectively, and the other, consisting of 147 cases seen from May 1974 through February 1979, was studied prospectively. Excluding the cases of pure seminoma, EST(YST) elements were found in 21 (28.7%) of 73 cases in the retrospective series and in 27 (44.4%) of 61 cases in the prospective series. The EST(YST) elements were in all cases admixed with other neoplastic germ-cell elements and varied from microscopic foci to being the predominant element within a tumor. The EST(YST) elements were histologically similar to infantile EST(YST) and EST(YST) in other locations. Serum alphafetoprotein (AFP) was determined in the majority of patients in the prospective series, and there was good correlation between the presence of EST(YST) elements within the tumor and elevated levels of AFP. The results of the present study indicate that EST(YST) elements occur quite frequently in testicular germ-cell neoplasms in adults and provide an explanation for the raised levels of serum AFP found in many adults with testicular germ-cell tumors. The results emphasize the importance of a thorough and careful pathologic examination of testicular germ-cell tumors, and the value of AFP as a tumor marker in patients with EST(YST). The results also lend further support to the view that EST(YST) elements found in testicular germ-cell tumors in adults are homologous with infantile EST(YST) and that EST(YST) is a distinctive and specific type of germ-cell neoplasm and should be included as such in the classification of testicular tumors.
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.
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.
This case report concerns an endodermal sinus tumor (EST) arising in the pineal region of a 16-year-old boy who died 3 months after radiation. He developed extensive abdominal metastases through a ventriculoperitoneal shunt, whereas the primary tumor and a suprasellar metastasis could be controlled by radiotherapy, demonstrated by autopsy. The histologic diagnosis was supported by an elevated level of alpha-fetoprotein in serum and the demonstration of this marker in the tumor tissue by immunoperoxidase method. The poor diagnosis of all previously reported cases with pineal EST requires a combined modality of surgical approach, radiotherapy, and concomitant chemotherapy.
This report describes six patients with endodermal sinus tumor of the vagina and cervix, a polypoid friable tumor whose clinical presentation in girls younger than age 3 years simulates the presentation of sarcoma botryoides. In four of the six patients, the referring diagnosis was sarcoma botryoides. Five patients were treated with excisional surgery, and all six with chemotherapy. One patient with pulmonary metastases maintained a complete clinical response to vincristine, actinomycin-D, and cyclophosphamide (VAC) for 11 months. This is the first report of such a response. One patient with a vaginal lesion remains clinically free of disease 2 years after local excision and 18 months of VAC chemotherapy. This is the first report of apparently successful therapy that allowed retention of childbearing potential. Four of the six patients have been disease-free from 2 to 23 years. After examining the world literature, it is concluded that a combination of chemotherapy and surgery offers a reasonable prospect of cure with a minimum of serious side effects.
Endodermal sinus tumor of the vagina, a rare pediatric malignancy, was managed with primary chemotherapy (vincristine, dactinomycin, cyclophosphamide [VAC]) followed by local excision without radical surgery or pelvic irradiation. Treatment-related complications were acceptable, and there is no evidence of disease 30 months after discontinuing all therapy (50 months after diagnosis). As a treatment option, primary chemotherapy is viewed as a highly attractive alternative modality, preserving potential reproductive and sexual function in long-term survivors, and appears to be the treatment of choice.
The first known case of primary anterior mediastinal endodermal sinus (yolk sac) tumor in a female patient, occurring in a 20-month-old infant, is reported. The child presented with cough, fever, and listlessness. Chest x-ray revealed a right anterior mediastinal mass. At thoracotomy a large anterior mediastinal tumor extending from the neck to the diaphragm was found, and was almost totally resected. Microscopically, the tumor displayed many of the histologic patterns observed in EST. Other neoplastic germ cell elements were not identified. The ultrastructural and immunohistochemical findings further confirmed the diagnosis. Serum alpha-fetoprotein (AFP) level, determined during surgery, was elevated to 65,200 ng/ml, whereas serum beta-human chorionic gonadotropin level was normal. Postoperatively, combination chemotherapy consisting of vinblastine, bleomycin, cisplatin, dactinomycin, cyclophosphamide, and doxorubicin was administered with a maintenance program. After 18 weeks on this regimen all the findings were normal, including serum AFP level. The child is well and disease-free 25 months after diagnosis.
A 12-year-old girl was admitted to the hospital for evaluation of an abdominal mass. A preoperative computed tomography scan showed a large tumor in the pelvis. The serum alpha-fetoprotein level was 2,170,000 ng/ml. A 3000-g left ovarian neoplasm was resected. It was encapsulated and showed focal microcystic degeneration, necrosis, and hemorrhage. Microscopically, it was composed of gland-like spaces containing mucin-positive material and surrounded by scant fibrovascular tissue. The epithelial cells were low columnar with immature oval, basophilic nuclei. Immunoperoxidase staining for alpha-fetoprotein and alpha1-antitrypsin were positive. Enzyme histochemistry was negative for alkaline phosphatase and positive for alpha-naphthyl acetate esterase. Electron microscopy, including freeze-fracture analysis, showed desmosomes and tight junctions. No gap junctions were identified. Actin filaments, glycogen, and microvilli were abundant. This is the first case of an ovarian endodermal sinus tumor with exclusive enteric differentiation.