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Malignant sacrococcygeal yolk sac (endodermal sinus) tumor.

We report a 2 1/2 years old female child who presented with Malignant Sacrococcygeal Yolk sac (Endodermal Sinus) tumor as presacral mass with ulceration of overlying skin. Her ultrasonography (USG)abdomen revealed a huge presecral mass with irregular margins extending in to the pelvis, pushing the rectum antero-laterally. CT scanning of abdomen confirmed USG finding with out enlargement of retroperitoneal lymph nodes and no involvement of the liver. Serum AFP was abnormally elevated. The tumor was excised along with Coccyx in-toto through posterior approach dividing the sacrum and 3-Drugs (VAC) Chemotherapy started postoperatively i.e. 2-weeks after surgery. Here we discuss our experience with this extremely rare malignant extra-gonadal germ-cell tumor and review of literature.

Child, Preschool↗

Establishment of the organizing activity of the lower endodermal half of the dorsal marginal zone is a primary and necessary event for dorsal axis formation in Cynops pyrrhogaster.

The formation of the head and trunk-tail organizers in the dorsal marginal zone (DMZ) of an amphibian embryo is thought to require spatial and temporal interactions between the Nieuwkoop center and the DMZ. Recent studies of the Xenopus embryo suggested that intra-DMZ interaction is also needed to establish the regional specificity of the DMZ. However, it is not yet clarified when and how the final pattern of the head and trunk-tail organizers is established. To analyze the intra-DMZ interactions, we injected suramin into the blastocoel of the mid-blastula of the urodele, Cynops pyrrhogaster, at 6 h prior to the onset of gastrulation. The pigmented blastopore formed normally, but the convergent extension and involution of the DMZ and dorsal axis formation of the embryo were completely inhibited. Expression of gsc, chd and Lim-1 were not maintained, but noggin was unaffected in the suramin-treated embryos. Dorsal axis formation and the expression of these genes of the suramin-treated embryos were rescued by replacing the lower endodermal half of the DMZ (LDMZ) with normal LDMZ. The present results of embryological and molecular examinations indicate that organizing activity of the early Cynops gastrula DMZ is restricted to the LDMZ, and that the organizing activity of the LDMZ is established during the late blastula stages. The results also indicate that LDMZ triggers the sequential interaction within the DMZ that establishes the final pattern of the regional specificity of the DMZ, and that the formation of the LDMZ is a primary and necessary event for dorsal axis formation.

Animals↗

Metastatic endodermal sinus tumor: CT appearances.

A one-week-old boy had undergone resection of a sacrococcygeal benign cystic teratoma. At the age of 12 months, he had a serum alpha-fetoprotein level of 139,000 IU/ml and a recurrent pelvic mass which was removed, and the microscopic examination revealed endodermal sinus tumor. Postoperatively, massively enlarged inguinal lymph nodes and abdominal distention developed. Computerized tomography displayed enlarged inguinal lymph nodes, metastatic lesions in the liver, and a pelvic recurrent mass. He received BEP (bleomycin, etoposide, cisplatin) chemotherapy regimen, and a complete remission was achieved with a normal serum alpha-fetoprotein. Close follow-up and serum alpha-fetoprotein monitoring are mandatory after the resection of a sacrococcygeal teratoma.

Antineoplastic Combined Chemotherapy Protocols↗

Endodermally-derived and neural crest-derived differentiation antigens expressed by a human lung tumor.

The plasma membrane antigens of an undifferentiated small cell (oat cell) carcinoma of the lung were studied by the indirect immunofluorescence method on frozen section substrates with a rabbit antiserum prepared to the tumor plasma membrane fraction. After appropriate absorption of the antiserum, at least two differentiation antigens present on the tumor cells but undetectable on normal lung surface or glandular epithelial cells were identified. One antigen(s) was characteristic of certain normal, endodermally-derived epithelial cells of the digestive system, including those of colonic mucosa, hepatic ducts, pancreatic ducts and acini, and islets of Langerhans. The other antigen(s) was characteristic of certain normal, neural crest-derived cells in the peripheral nervous system, including cells in peripheral nerve, dorsal root ganglion, and anterior roots of the spinal cord; parasympathetic ganglion cells in the colon; and small nerves and nerve processes in the lung, colon, and skin. It was concluded that the presence of these differentiation antigens on the tumor cells resulted from the expression of gene products repressed in the normal cell of origin of the tumor.

Absorption↗

[Tumors of the endodermal sinus (author's transl)].

Seven cases of tumors of the endodermal sinus are reviewed. This is a highly malignant germinal tumor, arisen from extraembrionary cell differentiation. It is different both biologically and pathologically from sacrocoxigeal teratoma. There are no survivors in these series. All seven patients have been surgically treated chemotherapy has been used in four, and X-ray therapy in two. It is noteworthy that alpha-phetoprotein is present in patients with this type of tumor. This fact can be used both in diagnosis of the tumor and of its recurrence.

Adenoma↗

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↗

Intercellular relationships during cavitation of aggregates of extraembryonic endoderm cells from gastrulating chick embryos.

Extraembryonic endoderm cells from gastrulating chick embryos undergo epiboly and change from a multilayered cell group to a single cell layer surrounding the yolk. Single cell suspensions from this cell layer can aggregate in vitro to form aggregates that cavitate. To study the stages of cavitation aggregates were harvested after different times in culture, and fixed and processed for light and electron microscopy. In aggregates harvested at 75 min of culture cell contact consisted of areas of parallel and close membrane apposition and interdigitation. Desmosomes were occasionally observed. Aggregates in the early stages of cavitation (24 h) contained numerous intercellular spaces bordered by irregularly shaped cells which appeared to be digesting their yolk and releasing material extracellularly. Long cytoplasmic projections were extended into these spaces. In addition to regions of parallel membrane apposition and interdigitation, desmosomes and adherens junctions were observed. Cells closer to the periphery of the aggregates displayed fewer cell projections and also showed signs of release of material extracellularly. After 48 h of culture, a single smooth-walled central cavity was present and cells still exhibited signs of extracellular release of material. These same cell shapes and intercellular junctions were also observed when area opaca tissue dissected from gastrulating embryos was examined. Aggregates of different sizes were created and cultured. The results suggest that a critical tissue mass may be important for cavitation.

Animals↗

[Role of the endoderm in the ventro-lateral mesoderm and primordial germ cells determination in Pleurodeles waltlii (author's transl)].

The ventro-lateral amphibian mesoderm is the presumptive area of blood cells, lateral plates and mesenchyme. To these mesodermal derivatives primordial germ cells (PGCs) must be added in the urodeles. Mesoderm and PGCs are formed from ectodermal cap of the gastrula under inductive influence of ventral endoderm. The present study was carried out to investigate the evolution of ventro-lateral and PGCs determination in Pleurodeles waltlii. The experiments are performed with mesodermal explant extirpated at successive stages of development, from young gastrula to mid-neurula (st. 8a, 9, 13, 15 and 18) and grafted in the tail-bud ventral or dorsal region. In the larva, the analysis of the prestations formed by the graft according to its age and its location on the host is made in serial sections. In dorsal location, the fate of the ventro-lateral gastrula mesoderm is dorsalized by contact with the host axial structures. This influence is characterized by a large amount of nephretic tubules. The potential presence of blood cells and PGCs in the young gastrula ventro-lateral mesoderm having been shown in other experiments, it seems that these cells are unable to self-differentiation before the end of gastrulation. It is proposed that their final determination is acquired only by a complementary interaction taking place during gastrulation and beginning neurulation.

Animals↗

Infantile endodermal sinus tumor presenting with vaginal bleeding: report of a case.

Endodermal sinus tumor (EST) of the vagina is extremely rare and primarily affects infants. We report on a six-month-old female infant with EST of the vagina who presented with vaginal spotting of one month's duration. Pelvic ultrasound and computerized tomography showed a 3.8 x 3.5 cm heterogeneous mass between the bladder and the rectum. The serum alpha-fetoprotein (AFP) level was high (1270 ng/mL) and the beta-human chorionic gonadotropin was undetectable. She received surgical intervention followed by chemotherapy. The patient was disease-free and serum AFP remained undetectable during the eight-month follow-up period.

Endodermal Sinus Tumor↗

[Endodermal sinus tumor of the testis in children: report of 9 cases].

Nine children with endodermal sinus tumor of testis have been treated since 1982. Their age ranged from 7 months to 5 years. Radical inguinal orchiectomy was carried out in all the patients. Four patients have received irregular chemotherapy. The follow-up showed that 4 died of the metastasis within 5 years and 5 (stage I clinically) survived without evidence of the recurrence for 1.5-7 years after operation. Serum AFP determination may give important information for the classification of the tumor and the evaluation of the follow-up results. For the cases of clinical stage I, radical orchiectomy, short course of chemotherapy, and frequent observations of AFP level are advocated.

Child, Preschool↗

Aspects of morphogenesis and cytodifferentiation of endoderm offered by organ culture techniques.

In this article, I will summarize the results obtained in my laboratory from the study of organogenesis since 1970. The results have shown region-specific directive influence of mesenchymes and reactivity of endoderms including those of the allantois, yolk sac, digestive tract and urogenital sinus. It was also shown that morphogenesis can take place without coupling cytodifferentiation in heterologous recombination experiments.

Androgens↗

Endodermal sinus tumor: report of 12 cases.

Twelve cases of endodermal sinus tumor were reviewed. There were 10 females and 2 males with a median age at presentation of 3 years. The primary site was sacrococcygeal in 4 patients, vaginal in 3, retroperitoneal in 2, and testicular, ovarian and left chest wall in one each. The diagnosis rested on histopathological examination and elevation of serum alfa feto protein levels (median 46,200 ng/ml). Two patients had Stage I disease, 9 had Stage III and one had Stage IV disease. Patients were managed by surgery and chemotherapy (BVP regime). All patients on BVP (even those lost at later stages), had achieved clinical remission with the first cycle of treatment.

Child↗

Retroperitoneal endodermal sinus tumor. Report of a case with an abnormal cervicovaginal smear.

An unusual case of an endodermal sinus tumor arising in the retroperitoneum and invading the fallopian tube is reported. The patient presented with malignant cells mimicking adenocarcinoma in the cervicovaginal smear. The differential diagnoses of such cells in cervicovaginal cytology are discussed. The cytohistologic, immunohistochemical and ultrastructural findings of the tumor are correlated.

Adult↗

[An endodermal sinus tumor of the ovary. A case report].

The authors present the clinical history of a 15-year-old young woman, student, from David, Chiriquí, who complained of menstrual irregularities associated with the sensation of a mass in the right iliac fossa. Exploratory laparotomy revealed a right ovarian tumor, which was totally resected. The histopathological examination showed an endodermal sinus tumor, stage one, in the right ovary. The patient received four cycles of cytotoxic i.v. therapy every 3 weeks, after surgery, with vinblastine 6 mg/m2 body surface (B.S.) on days 1 and 2, bleomycin 15 u daily for 5 days, cisplatin 20 mg/m2 B.S. daily for 5 days, plus hydration with normal saline (5 to 6 l/day). Follow up studies with abdominal and pelvic sonograms, chest x-ray, feto protein and physical examination have not shown evidence of tumor activity 36 months after diagnosis.

Adolescent↗

Behavior of endodermal "button cells" during metamorphosis of ascidian larvae.

Swimming larvae of Phallusia mamillata are known to have "button cells" of endodermal origin between the ventral surface of the pharynx and the epidermis, that are stainable by various techniques. By immunofluorescence with anti-tubulin antibody and confocal laser microscopy, we obtained a bright reaction at one pole of the cells, suggesting the presence of a cap of tubulin and of microtubules overlaying the nucleus. During metamorphosis the microtubule-rich pseudopods at their base reach the epidermis, especially in the area near the adhesive papillae. Then they emigrate through the epidermis and become roundish again.

Animals↗

[Organization and differentiation of the somite mesoderm cultured in the presence of adjacent tissues (notocord, spinal cord, ectoderm, endoderm) in anurans].

The explantation of pieces of somitic mesoderm alone or in association with neighbouring tissues (notochord, spinal cord, ectoderm, endoderm) shows slearly the power of self-differentiation of the somitic tissue. Only, the spinal cord does not stimulate the differentiation of the somitic mesoderm into muscular tissue. Only the notochord does not stimulate the formation of typical myotomes. It seems that ii does not possess biochemical activity, its effect if reduced to a mechanical role.

Age Factors↗