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[Endodermal cyst of the cerebellopontine angle cistern: case report].

We report a case of a 55-year-old man with endodermal cyst located in the cerebellopontine angle cistern. The patient presented with dizziness. Magnetic resonance imaging (MRI) revealed multilocular cystic lesion at the right cerebellopontine angle. T1-weighted image showed a mass with a low signal intensity, but higher intensity than CSF. Gd-DTPA T1-weighted image showed no enhancement in the mass. Diffusion-weighted image showed a mass with no signal lesion. After successful surgical removal, it was found to be an endodermal cyst. These cysts have usually been found in the spinal canal, and their intracranial occurrence is exceptional. The unusual location of the cyst and its histological features and radiological findings are discussed.

Cerebellar Diseases↗

[A case of neuroaxis endodermal cyst].

The patient was a 69-year-old male. During examination of possible deafness, a cystic mass was found in the left frontal lobe, which required surgical intervention. Though the mass adhered strongly to the dura mater macroscopically, the boundary between the mass and arachnoid was clear, having a meningioma-like appearance. The pathological diagnosis was epithelial cyst consisting of layered cubic to cylindrical epithelium. It is essential to perform an electronmicroscopic or immunohistological examination for knowing the origin of an epithelial cyst in the neuroaxis, that is, of ectodermal, endodermal, or mesenchymal origin. However, the diagnosis of heterotopic endodermal cyst was established in this patient because the continuity with ependyma cells or choroid plexus was absent without the so-called ectodermogenic tissue such as glia and nerve cells.

Aged↗

Endodermal sinus tumor: a report of two sustained remissions treated postoperatively with a combination of actinomycin D, 5-fluorouracil, and cyclophosphamide.

The literature reflects a poor prognosis for women with endodermal sinus tumor. Two women with this neoplasm, 1 having stage IA and the other stage IIB disease were treated for 1 year postoperatively with a combination of actinomycin-D, 5-fluorouracil, and cyclophosphamide. Each had therapy discontinued after a negative second laparotomy and 15 and 21 months later are without recurrence. It is suggested that this drug regimen is a benficial adjunct to surgery in the treatment of endodermal sinus tumor.

Adolescent↗

Role of the anterior visceral endoderm in restricting posterior signals in the mouse embryo.

Recent genetic and embryological experiments have demonstrated that head formation in the mouse embryo is dependent on signals provided by two organising centers during gastrulation, the anterior visceral endoderm (AVE) and the anterior primitive streak (also called the Early Gastrula Organiser, EGO). However the molecular nature of the signals triggering anterior neural formation from the epiblast is not clearly understood. The analysis of mouse mutants has allowed the identification of some of the molecular players involved in the process of head formation. In this review, we describe different mutant embryos in which impairment of visceral endoderm function leads to similar defects in antero-posterior axis specification. These phenotypes are consistent with a role of the AVE in protecting anterior embryonic regions from signals that promote posterior development. We propose that a genetic cascade in the AVE, involving HNF3beta, Lim1, Otx2, Smad2 and ActRIB, leads to the production of secreted TGFbeta antagonists that protect the anterior epiblast region from Nodal signalling.

Activin Receptors, Type I↗

Effusion cytology of endodermal sinus tumor of the colon. Report of a case.

A three-year-old boy presented with a peritoneal effusion due to an occult endodermal sinus tumor in the ascending colon. Cytologic examination of the ascitic fluid revealed clusters of round plump cells that had large hyperchromatic nuclei, finely stippled chromatin, multiple prominent nucleoli and vacuolated or mucin-containing cytoplasm, features suggesting an adenocarcinoma. The colonic primary was discovered during emergency laparotomy performed due to suspected acute hemoperitoneum. Endodermal sinus tumor should be included in the differential diagnosis when cytologic features reminiscent of adenocarcinoma are encountered in a fluid specimen from a child, especially if there is a history of a gonadal or extragonadal childhood neoplasm.

Antineoplastic Combined Chemotherapy Protocols↗

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 infant vagina treated exclusively by chemotherapy.

A 5-month-old Hispanic female, diagnosed as having endodermal sinus tumor of the vagina, was treated with combination chemotherapy in an attempt to preserve pelvic function. An incisional biopsy was performed before starting chemotherapy. After completion of chemotherapy, no residual disease was found at exploratory laparotomy. The patient received no further therapy and is disease-free 45 months later. This case represents successful treatment of a vaginal endodermal sinus tumor without any operative excision.

Antineoplastic Combined Chemotherapy Protocols↗

Estrogen and progesterone receptors in endodermal sinus (yolk sac) tumor. Evaluation of immunocytochemical and biochemical methods.

Frozen tissue from four ovarian and two testicular endodermal sinus (yolk sac) tumors was studied with both biochemical and immunocytochemical methods to detect the presence of estrogen receptors (ERs) and progesterone receptors (PRs). Using the biochemical assay, one of the ovarian tumors was shown to contain low levels of ERs, whereas in the remaining five tumors no ERs were detected, and PRs were undetectable in all six tumors. In the frozen sections immunostained for ERs and PRs, no specific nuclear staining was detected in any of the six tumors. Since the immunocytochemical method provides a more tissue-specific evaluation of hormone receptors, the low level of ERs detected in one tumor using the biochemical assay is attributed to nonspecific binding. It appears that endodermal sinus tumor expresses neither ERs nor PRs.

Female↗

[Light and electron microscopic observations on endodermal sinus tumor of the ovary].

The results of light and electron microscopic studies of the endodermal sinus tumor of ovary are presented. Although histologic features of the tumors appeared to be much varied, ultrastructurally all tumor cell seemed to show changes essentially of the same nature. The histogenesis of endodermal sinus tumor production of AFP by the hyaline globule and the basement membrane-like substance present only in this type of tumor, are discussed. The results of the present study support the view that the tumor originate from the embryonal yolk sac cells.

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↗

[Terminology and chemotherapy of the so-called endodermal sinus tumor].

In addition to a discussion on the histogenetic, terminological and clinical problems concerning the so called endodermal sinus tumor a typical case in a far advanced stage is reported. Chemotherapy with vincristine and adriblastine was not able to influence the deleterious course of desease. Anyhow, since little is known about the effect of chemotherapy on the so called endodermal sinus tumor, this report might be of advantage.

Adult↗

alpha-Fetoprotein and other serum proteins synthesized by endodermal sinus tumor transplanted into nude mice.

The human yolk sac is said to synthesize not only alpha-fetoprotein but also other serum proteins, i.e., albumin, prealbumin, alpha 1-antitrypsin, and transferrin. Two endodermal sinus tumors (yolk sac tumors) were successfully transplanted into athymic nude mice. Concentration of these human serum proteins in the sera of the nude mice and tumor extracts was determined quantitatively by electro-immunodiffusion and radioimmunoassay. alpha-Fetoprotein and albumin were detected in one case, and alpha 1-antitrypsin and alpha-fetoprotein in the other. In the former case, the amount of alpha-fetoprotein synthesized was larger than that of albumin, whereas the amount of alpha-fetoprotein was smaller than that of alpha 1-antitrypsin in the latter. It is tempting to consider that human endodermal sinus tumors simulate the human yolk sac functionally, but the pattern of protein synthesis may differ from case to case.

Animals↗

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↗

[Effect of anti-human alpha-fetoprotein serum on human endodermal sinus tumor transplanted in nude mice (author's transl)].

The effects of horse anti-human alpha-fetoprotein (AFP) immunoglobulin (IgG) on growth, serum AFP concentration and histopathology of human endodermal sinus tumor serially xeno-transplanted in nude mice were examined. Anti-AFP IgG given in amounts sufficient to neutralize the serum AFP secreted by the tumor moderately reduced tumor size and significantly decreased the serum AFP levels of treated nude mice. On histopathologic examination, spindle shaped tumor cells of reticular pattern often fell into the state of necrosis. But little change was observed on the cells consisting of both solid patterns and Schiller-Duval bodies. With a growth rate of re-transplanted tissues respectively, which were obtained from both antibody administrated and control tumors, no disparity was found between two groups. Indeed this result is preliminary report, but inhibitory effects of anti-AFP IgG against tumor growth and AFP producing ability of endodermal sinus tumor xeno-transplanted in nude mouse is hopeful to apply these data on clinical cancer immunochemotherapy.

Animals↗

[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↗

[A child with successfully treated posterior mediastinal endodermal sinus tumor].

A 15-month-old boy with posterior mediastinal endodermal sinus tumor is reported. The patient was admitted with recurrent hemothorax, and his serum alpha-fetoprotein level was 29,520 ng/ml. After removal of the posterior mediastinal tumor, VAC therapy (vincristine, actinomycin D, cyclophosphamide) was started. The chemotherapy was continued 18 months. The patient continues to do well with no sign of tumor and a normal alpha-fetoprotein level 53 months after the operation and 35 months after the completion of therapy. He is the youngest patient documented in the literature and successfully treated for mediastinal endodermal sinus tumor.

Antineoplastic Combined Chemotherapy Protocols↗

[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 of the vulva in an infant.

An endodermal sinus tumor arising in the vulva of a 22-month-old infant is reported and the features of 3 previously recorded endodermal sinus tumors of the vulva are reviewed. Despite radical surgery, radiation therapy, and chemotherapy the tumor was fatal within 6 months. Only one of the 3 previously described patients with this type of vulvar tumor has survived more than 2 years. That tumor was the smallest reported, being the only one under 2 cm in diameter.

Biopsy↗