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Pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung).

A tumor of the lung in a 35-year-old woman contained numerous complex, branching tubules resembling the developing fetal lung in its canalicular state. Some of the epithelial cells within the tubules were argyrophilic, and electron microscopy demonstrated the dense-core neurosecretory-type cytoplasmic granules. These characteristics support an endodermal origin for this neoplasm. Morphologically the tubules are similar to the epithelial component seen in pulmonary blastoma, but the tumor lacks the sarcomatous areas integral to the concept of blastoma. The designation pulmonary endodermal tumor resembling fetal lung may better describe this unusual neoplasm.

Adult↗

Endodermal sinus tumor of the ovary: review of 10 cases.

Endodermal sinus tumors are rare but highly malignant neoplasms of children and young adults. In all, only about 200 cases have been reported in the literature. Ten patients with endodermal sinus tumor of the ovary who were treated at Jackson Memorial Hospital from 1977 to 1989 were retrospectively reviewed. The median age of these patients was 24.7 years. The most common presenting symptom was abdominal pain, occurring in all our patients. Nine of the 10 patients received combination chemotherapy; all but one survived and are currently well. The patient who received no chemotherapy died. Eight of the ten patients are alive and well. The roles of alpha-fetoprotein monitoring, second-look laparotomy, and conservative surgery are discussed. The need for aggressive combination chemotherapy for this tumor is confirmed by this study.

Adolescent↗

Endodermal cyst of the cervical spine treated by an anterior approach for resection and shunting.

In most reported cases, a posterior approach to the cervical spine was used to remove an endodermal cyst or to place a shunt for cyst drainage. The authors performed partial excision, shunt insertion, and anterior spinal fusion in a patient with endodermal cyst of the cervical spine. Symptoms resolved after surgery and magnetic resonance images indicated morphologic restoration of the spinal cord.

Adult↗

Endodermal sinus (yolk sac) tumor in infants and children. A clinical and pathologic study: an 11 year review.

We reviewed the clinical features, treatment, and results of children with gonadal and extragonadal yolk sac (endodermal sinus) tumors seen in the King Faisal Specialist Hospital and Research Centre between 1976 and 1987. There were nine children (seven girls and two boys) with ages ranging from 7 months to 12 years (median of 3.5 years). Sites of origin included the vagina (two cases), face (two cases), sacrum (two cases), mediastinum (one case), ovary (one case), and testicle (1 case). All children had elevated alpha-fetoprotein (AFP) at diagnosis. One girl had complete surgical excision of an ovarian tumor at the time of diagnosis, and one boy had surgical excision of the testis. In the remaining seven children, the tumor was unresectable. Surgery was limited to a biopsy in six children. All patients received different combinations of chemotherapy, including vincristine (VCR), actinomycin D (Act-D), cyclophosphamide (Cyclo), adriamycin (Adria), bleomycin (Bleo), cis-platinum (CDDP), vinblastine (VBL), and VP-16. Of the nine patients, one was lost to follow-up while in remission, five died, one was lost to follow-up, and three are alive and disease-free at 15, 55, and 67 months from diagnosis. This review demonstrates an unusual preponderance of the extragonadal form of endodermal sinus tumor among our patients.

Antineoplastic Combined Chemotherapy Protocols↗

Endodermal cyst of the cervical spinal cord with associated partial fusion of the vertebrae.

A case of a 43-year-old woman with an intradural endodermal cyst at the C4 spinal cord level is presented. The lesion manifested with progressive neck pain and weakness of lower extremities. The presumptive diagnosis of extramedullary benign cystic lesion was established after MRI examination. Coexisting partial fusion of the C5-C6 vertebral bodies was found on X-ray films. The patient was cured by resection of the cyst wall, performed by laminectomy. Pathological examination revealed a cyst wall lined with ciliated cuboidal cells, resembling the respiratory epithelium. Immunohistochemistry demonstrated cytokeratin and epithelial membrane antigen expression in the lining of the cyst. Endodermal cyst should be considered in the differential diagnosis of intraspinal cystic lesions, especially those associated with vertebral anomalies.

Adult↗

Endodermal sinus tumor of the stomach.

This is a case report of an endodermal sinus tumor arising in the stomach of a 72-year-old Japanese woman. The tumor showed Borrmann 2 type at the mucosal surface, and the main mass occupied the subserosal layer with outgoing extension. Microscopically, the tumor showed typical characteristics of the endodermal sinus tumor, associated with embryonal carcinoma, poorly differentiated adenocarcinoma and well differentiated tubular adenocarcinoma. Alpha-fetoprotein was detected in the serum of the patient by radioimmunoassay and within tumor tissue by immunohistochemical techniques. Ultrastructurally, a characteristic basement membrane-like material has been identified in intercellular spaces.

Adenocarcinoma↗

Histological evidence for cell proliferation activity in cystic tumor (endodermal heterotopia) of the atrioventricular node.

Cystic tumor (endodermal heterotopia) of the atrioventricular (AV) node in a 95-year-old female is described. Electrocardiograms showed complete AV blockage with a narrow QRS morphology resulting from the long-standing first degree of AV blockage since age 61. A cardiac pacemaker was implanted at age 83. The patient died of pneumonia at age 95. Autopsy demonstrated a normal heart weight (320 g), normal coronary artery and normal myocardium. Histological examination of the conduction system revealed a marked fibrotic sinoatrial (SA) node and a cystic lesion in the lower right atrium, including the approaches to the AV node. The bundle of His and its branches were completely intact. Cellular nests and cystically dilated tubules were scattered within a loose tissue stroma, which measured 18 x 12 x 5 mm. There were several mitotic figures among the tubules, as well as nuclear fragments similar to apoptotic bodies and exfoliation of the tumor cells. Immunohistochemical studies demonstrated a positive reaction for epithelial membrane antigen, carcinoembryonic antigen, CA19-9, cytokeratin and secretory component in the tubular cells, and a negative reaction for thrombomodulin. Endocrine cells among the tubules were positive only for calcitonin and serotonin. Ultrastructurally, sparse microvilli and desmosome-like structures between the tumor cells were observed. These findings support that this lesion originates from the endoderm. Moreover, it is likely that progression in the degree of AV block may be explained by the cell proliferation activity of the tumor.

Aged↗

Serum alpha-foetoprotein as a marker for endodermal sinus tumour (yolk sac tumour) or a vitelline component of "teratocarcinoma".

The correlation between serum alpha-foetoprotein (AFP) and the clinical pathological finding of 24 germ cell tumours arising from the testes (14 cases), the ovaries (3 cases), the mediastinum (3 cases), the retroperitoneal region (2 cases), and the sacrococcygeal region (2 cases) are presented. Irrespective of marked differences in age and sex of the patients, primary site of the tumours and clinical outcome, the 24 cases constituted a homogeneous group in fundamental histological patterns and in AFP synthesis. In all cases of endodermal sinus tumour or teratocarcinomas with a distinct vitelline component an increased serum AFP concentrations was found in the pre-operative serum samples. AFP was also demonstrated in the tumour tissue by quantitative determination of AFP in tumour homogenate (5 cases) and, by immunofluorescence technique, positive staining of the cells lining the endodermal sinuses and of the hyaline globules was found (3 cases). In 12 germ cell tumours without vitelline components in the tumour tissue sections, a normal AFP concentration below 20 mug/1 was found in preoperative serum samples.

Adolescent↗

Regulation of the differentiation of teratocarcinoma cells into primitive endoderm by G alpha i2.

The amount of the heterotrimeric G protein subunit G alpha i2 decreases after the induction of F9 teratocarcinoma cells to become primitive endoderm in the presence of retinoic acid (RA). The reduction of the G alpha i2 protein in F9 cells by antisense RNA expression was associated with (i) loss of receptor-mediated inhibition of adenylyl cyclase; (ii) decreased cell doubling time; (iii) induction of a primitive, endoderm-like phenotype in the absence of RA; and (iv) production of the differentiation marker tissue-type plasminogen activator. Expression of a constitutively active, mutant G alpha i2 blocked RA-induced differentiation. These data suggest the involvement of G alpha i2 in the control of stem cell differentiation and provide insight into the involvement of G proteins in growth regulation.

Adenylyl Cyclases↗

Ultrastructural analysis of chick embryo blastoderms explanted in vitro in absence of endoderm. Differentiation capacity of mesoderm.

A study of the structural characteristics of stage 5 chick embryo blastoderms, cultured in vitro, was carried out after previous elimination of endoderm to analyse the differentiation capacity of mesoderm. Our results show that only the precardiac mesoderm is determined at this early stage and that it is able to differentiate cardiac tissue in the absence of the endoderm. The ultrastructural characteristics of this tissue are described. However, the degree of differentiation of cardiomyoblasts does not reach the expected level of organization and myofibrillar complexity.

Animals↗

Immunohistochemical localisation of alpha-fetoprotein (AFP) in germ cell tumours: evidence for AFP production by tissues different from endodermal sinus tumour.

20 germ cell tumours have been studied with respect to the presence of alpha-fetoprotein (AFP), using the peroxidase-antiperoxidase (PAP) technique. 6 out of 20 tumours contained elements of endodermal sinus tumour (EST) and were AFP positive. 16 tumours were diagnosed either as pure embryonal carcinomas (6) or as mixed germ cell tumours, containing elements of embryonal carcinoma (10). In 3 of these 16 tumours AFP was localised definitely in the embryonal carcinoma cells; in an additional 6, AFP was also detected but it could not be decided whether AFP was present in embryonal carcinoma cells or in EST cells during early differentiation. In 2 of 7 immature teratomas, AFP was shown to be present in cylindric epithelia. All seminomas (4) studied were AFP-negative. These results show that AFP, which occurs regularly in EST, may also be present in embryonal carcinomas as well as in immature teratomas. Thus, it seems that the immunohistochemical demonstration of AFP by the PAP technique is a suitable method of identifying late stages of embryonal carcinoma or early stages of endodermal sinus tumour during the process of differentiation.

Dysgerminoma↗

45,XO/46,X,dic(Yq) mosaicism in Turner's phenotype with endodermal sinus tumor of the ovary.

A case of a endodermal sinus tumor of the ovary in a patient with 45,XO/46,X,dic(Y) mosaicism is reported because of the rarity of the karyotype and condition. A 15-year-old girl was admitted to Yamagata University Hospital because of constipation for several days. Physical examination showed webbing of the neck, cubitus valgus and short stature. Her abdomen was bulging. Chromosomal analysis showed 45,XO/46,X,dic(Yq) mosaicism in karyotype. alpha-Fetoprotein and CA-125 in the serum were high. A left ovarian tumor was found by laparotomy; however, the right ovary was a streak gonad and the uterus was hypoplastic. An endodermal sinus tumor was diagnosed by a pathologist. After operation, cisplatin-vinblastin-bleomycin chemotherapy was instituted and the tumor marker went down. This patient is still healthy and under observation at the outpatient clinic.

Adolescent↗

XIHbox 8, an endoderm-specific Xenopus homeodomain protein, is closely related to a mammalian insulin gene transcription factor.

The cis-acting sequences that mediate insulin gene expression exclusively in pancreatic islet beta-cells are localized within the 5'-flanking region between nucleotides -340 and -91. We have identified an evolutionarily conserved, A+T-rich element at -201/-196 basepairs in the rat insulin II gene that is essential for efficient expression in beta-cells. Affinity-purified antibody to the XIHbox 8 protein super-shifted the major beta-cell-activator factor complex binding to the -201/-196 element. XIHbox 8 is a Xenopus endoderm-specific homeodomain protein whose expression is restricted to the nucleus of endodermal cells of the duodenum and developing pancreas. Antibody to XIHbox 8 specifically interacts with a 47-kilodalton protein present in this DNA complex. Immunohistochemical studies revealed XIHbox 8-like proteins within the nucleus of almost all mouse islet beta-cells and a subset of islet alpha- and beta-cells. These results are consistent with the proposal that an XIHbox 8-related homeoprotein of 47 kilodalton is required for expression of the mammalian insulin gene in beta-cells. Experiments conducted with antiserum raised to somatostatin transcription factor-1 (STF-1), a recently isolated mammalian XIHbox 8-related homeoprotein, indicate that the STF-1 protein is the mammalian homolog of Xenopus XIHbox 8.

Amino Acid Sequence↗

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↗

Endocytosis of the symbiotic dinoflagellate Symbiodinium microadriaticum Freudenthal by endodermal cells of the scyphistomae of Cassiopeia xamachana and resistance of the algae to host digestion.

The ingestion and fate of four types of particles by endodermal cells of the scyphistomae of Cassiopeia xamachana were investigated by scanning and transmission electron microscopy. Ferritin was endocytosed pinocytotically by invagination of the plasmalemma. These small pinocytotic vesicles fuse with other similar vesicles to form larger ferritin-containing vacuoles, which eventually fuse with lysosomes. Such secondary lysosomes exhibit acid phosphatase activity. The co-occurrence of acid phosphatase activity and ferritin in secondary lysosomes achieved maximum frequency within 2 h of uptake of ferritin and was evident for at least 4 h following uptake. Artemia particles, live freshly isolated symbiotic algae (Symbiodinium microadriaticum), and heat-killed S. microadriaticum are phagocytosed by endodermal cells. Ferritin-labelled lysosomes fused with food vacuoles containing particles of Artemia. Vacuoles containing heat-killed S. microadriaticum also showed evidence of phago-lysosome fusion. S. microadriaticum in situ (i.e. in host cells) after 3 days exposure to the photosynthetic inhibitor, 3-(3-4-dichlorophenyl)-1,1-dimethylurea, appeared degenerate, and were found in loose-fitting host vacuoles, many in mid and apical portions of the host cell. More than 70% of these vacuoles with moribund algae contained the ferritin label, indicating that lysosome fusion had occurred. In contrast, live S. microadriaticum in control animals were almost always found at the base of the host cell in individual tight-fitting vacuoles with no evidence of lysosome fusion. Live S. microadriaticum apparently escape host digestion by prohibiting the fusion of lysosomes with the vacuole in which they reside. Vacuoles containing defunct algal symbionts, in contrast, were subject to lysosomal attack.

Acid Phosphatase↗

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↗

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 (yolk-sac carcinoma) of the orbit.

A 15-month-old boy with rapidly progressive proptosis had an unusual malignant germ-cell tumor of the orbit and nasopharynx identified as an endodermal sinus tumor (yolk-sac carcinoma). Endodermal sinus tumors arising in extra gonadal locations are highly malignant and generally lethal. The boy was treated with radiation and three-drug chemotherapy and has survived more than eight years.

Exophthalmos↗