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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↗

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↗

Endodermal sinus tumor of the omentum: case report.

We report a case of primary endodermal sinus tumor of the omentum in a 46-year-old woman. The patient had been referred to our hospital for pain in the right abdomen. CT scan revealed a right-sided abdominal mass. At laparotomy, a large omental tumor extending to the bowel, uterus, ovaries and peritoneum was found, and it was totally resected. The tumor displayed the typical histological patterns observed in endodermal sinus tumor, and the immunohistochemical findings confirmed the diagnosis. Serum AFP level was 21.550 ng/ml prior to laparotomy, and decreased to 13.845 ng/mL after surgery, whereas the level of serum beta human chorionic gonadotropin was normal. Post-surgery, the patient received combination chemotherapy consisting of cisplatin, etoposide and bleomycin every 3 weeks for a total of 6 cycles. At the end of the treatment, all the findings, including AFP level, were normal.

Biomarkers, Tumor↗

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↗

Comparative morphology of endodermal sinus tumor (Teilum) to human yolk sac and a proposal of endodermal cell tumor.

Histological criteria of 8 pure and typical endodermal sinus tumor (EST) were compared with the morphological features of 8 human yolk sacs from 5 to 13 weeks of pregnancy. No common features were found with regard to endodermal sinus structure (ESS) and clear-cell entoblastic pattern (CCEP), but magma reticulare (MR) and eosinophilic hyaline globules (EHG) were detected as common findings. As to the morphology and synthesis of alpha-fetoprotein (AFP), some tumor cells with eosinophilic-granular cytoplasm lining ESS had similarity to the endodermal cell of human yolk sac. Histological features of EST mimicked the morphological structure of endodermal sinus in rodent yolk sac. Pathological study on an ovarian and a gastric adenocarcinoma with high level of AFP concentration demonstrated two cell patterns with eosinophilic-granular and clear cytoplasm showing analogousness to the endodermal cell of human yolk sac. It was suggested that these tumors showed a selective differentiation to the endodermal cell of human yolk sac and might be called ECT. Further study clarified that ovarian embryonal carcinoma or teratocarcinoma with higher AFP concentration had vitelline component of EST or ECT or both.

Adenocarcinoma↗

Congenital endodermal sinus tumor of the penis.

Congenitally occurring endodermal sinus tumors are very rare and frequently occur in association with teratomatous growths. The most common location for these tumors in infancy is the gonads. Tumors of the penis are rare in any age group. As far as we know, this tumor has never been reported in the penis. A 17-month-old baby boy was noted to have a pimple-like lesion at the corona of the glans penis. This was stationary and ignored for a year. At this point, the lump began increasing in size. A further delay of 6 months elapsed before a definitive diagnosis was made. This case emphasizes that any nodule in this area in the neonate is ignored with jeopardy.

Combined Modality Therapy↗

Endodermal sinus tumor of the anterior mediastinum.

An endodermal sinus tumor presenting as a lobulated anterior mediastinal mass in a 20-year-old white man is reported. The clinical implications, histology, and origin of this rare germ cell tumor are discussed. The invasive nature of this highly malignant neoplasm prevented complete surgical resection.

Adult↗

Transhepatic artery chemoembolization for liver metastases of primary retroperitoneal endodermal sinus tumor. A case report.

BACKGROUND: Primary retroperitoneal endodermal sinus tumors (EST) are extremely rare and highly malignant. Hepatic metastases from EST are often very difficult to manage and carry a dismal prognosis. Transhepatic artery chemoembolization has been widely applied in primary unresectable hepatoma and had been reported to have antitumor activity for some metastatic tumors. We report a case in which transhepatic artery chemo-embolization was employed to control hepatic metastases from EST. CASE: A 35-year-old woman presented to our service with primary retroperitoneal EST associated with multiple hepatic metastases. The patient underwent aggressive debulking surgery followed by a combination chemotherapy regimen of cisplatin, vinblastine and bleomycin. Transhepatic artery chemoembolization with adriamycin, lipoidol and gelfoam was subsequently employed to treat hepatic metastases. CONCLUSION: The combination of transhepatic artery chemoembolization and systemic chemotherapy after surgical resection of primary retroperitoneal EST was effective in controlling the hepatic disease in this case.

Adult↗

The role of second look laparotomy and tumor markers in the follow-up of endodermal sinus tumor of the ovary. A case report and review of the literature.

Endodermal sinus tumor (EST) of the ovary is extremely rare and little information exists about therapy and the role of second-look laparotomy in the management of this entity. A case of EST of the ovary in a 21 year old woman is reported. She received conservative surgery and six courses of combination therapy consisting of Vincristine, Actinomycin D and Cyclophosphamide before second-look laparotomy. Due to progression of the disease second-line polychemotherapy with Vinblastine, Bleomycin and Cisplatin was administered. This new regimen reduced the alpha-fetoprotein to normal levels although the patient was not free of disease on second-look laparotomy. Precise guidelines for the management of this disease, especially in advanced stages, are still lacking.

Adult↗

Endodermal sinus tumor of the vagina: radiation therapy and progeny.

BACKGROUND: Extraovarian endodermal sinus tumor generally originates in the vagina or cervix of young girls. Some 50 cases are known, all younger than 3 years of age. Many were treated with outdated regimens, and there are 5-year survival data on only nine of them. We describe a curative outcome of primary radiation only. CASE: A 40-year-old woman had a history of localized endodermal sinus tumor of the vagina, stage I, treated by brachytherapy at the age of 6 months. Radiation had a severe impact upon the later functioning of her pelvic organs, with marked deformity of the pelvic floor, vagina, urethra, and bones of the pelvic outlet. She conceived by homologous insemination at the age of 34. Delivery was by cesarean at 31.5 weeks' gestation. CONCLUSION: Current forms of management give preference to limited excisional surgery and multi-agent chemotherapy, analogous to that used for the treatment of endodermal sinus tumor of the ovary. This is the first report of a patient treated by radium needles and the only known patient who had a subsequent pregnancy.

Adult↗

[Ultrastructure of endodermal sinus tumor of the ovary (author's transl)].

The ovarian endodermal sinus tumor is a relatively rare type of malignant neoplasm. Ultrastructurally, the tumor cells proved to have numbers of ribosomes, glycogen granules and well-developed rough endoplasmic reticulum surrounding the mitochondria. The photomicroscopically visible hyaline globules were observed as round homogeneous non-structural substances both in and outside the cytoplasm. It was surmised that it is synthesized in the rough endoplasmic reticulum. The tumor cells could be classified into three types on the basis of the forms of cytoplasmic organella and intracellular homogeneous spheres. The first type of cell proved to be well-developed cytoplasmic organella, with a few small spheres and a large aggregate of glycogen granules and ribosomes. The second type of cell proved to have extensive non-structural cytoplasm and a number of largesized spheres. The third type was deemed to be of the intermediate type. In other words, it was assumed that morphological changes were responsible for the tumor cells changing from the first type to the second type. A similarity was noted between these ultrastructures and the yolk sac endodermal cells of the very initial stage, therefore the name endodermal sinus tumor was assumed to be adequate.

Adult↗

Endodermal sinus tumor of the vagina in an infant: magnetic resonance imaging evaluation.

Endodermal sinus tumor is a rare and highly malignant lesion. This report documents the clinical findings and magnetic resonance imaging (MRI) of an uncommon case of the endodermal sinus tumor arising in the vagina of a 6-month-old infant. MRI was extremely accurate in delineating the possible extent and location of the vaginal lesion. This is the first report to demonstrate MRI of vaginal endodermal sinus tumor.

Antineoplastic Combined Chemotherapy Protocols↗

[Endodermal sinus tumor--a contribution to the clinical aspects of ovarian tumors].

Two cases of endodermal sinus tumor (EST) are described in a 19-year-old girl and a 50-year-old menopausal women, respectively. Exstirpation uteri totalis cum adnexis was carried out in both cases. According to the FIGO the tumors were classified as Ic. The histologic diagnosis of EST was based on the presence of elements such as extraembryonic mesoderm, yolk sac mesoderm, vascular structures with Schiller-Duval bodies, PAS-positive globules in Duval sinus spaces. In the first case, where the proposed chemotherapy was refused, the patient died 4 months later with symptoms of abdominal metastases. In the other case, chemotherapy was applied according to the VAC program. At 13 months of the treatment a relaparotomy was made and the lymphatic nodes taken showed no metastases. Three months later, the tumor relapsed and the patient died at 22 months of the therapy. In the light of their observations and literature data the authors recommended a radical surgical intervention combined with intense polychemotherapy as the only effective approach to the problem.

Adult↗

Endodermal sinus tumor: immunophenotypic expression of a carcinoma.

A series of five endodermal sinus tumors was studied for their cytoskeletal and other phenotypic markers. They included 2 ovarian, 2 testicular, and 1 inguinal tumors. The cytoskeletal expression was also studied by gel electrophoresis and immunoblotting. Every tumor was diffusely and strongly immunostained for cytokeratin. By SDS-PAGE and immunoblotting, cytokeratins 8 & 18 were detected. Vimentin was focally coexpressed in 4 cases. The stroma was diffusely immunostained for vimentin. None of them expressed desmin, neurofilament, or glial filament protein. Desmoplakin was expressed only in one ovarian tumor. Alpha-fetoprotein and S-100 protein were also diffusely positive among the neoplastic cells; intracytoplasmic globules were especially strongly immunostained. These findings suggest that endodermal sinus tumors represent a group of pure malignant epithelial neoplasms, and may be regarded as primitive carcinomas.

Adult↗

Germ cell neoplasms of head and neck soft tissues: a pathologic spectrum of teratomatous and endodermal sinus tumors.

Germ-cell neoplasms, in particular teratomas with immature and mature somatic type tissues, are some of the most commonly found tumors in children. Approximately 5% of these neoplasms appear in one of several extracranial sites in the head and neck region. This study reports the clinical, pathologic and immunohistochemical findings in six germ-cell neoplasms occurring in the neck and facial areas. A mass was recognized at birth in five children, and the sixth patient was 2 1/2 years old at diagnosis. Four of the six neoplasms contained one or another element of endodermal sinus tumor; two of these had a mixed pattern of endodermal sinus tumor and teratoma. The other two cases were purely teratomas. The serum alpha-fetoprotein was known to be elevated in three children whose tumors had endodermal sinus elements; it returned to normal level in two of the children, but remained high in the one fatal case. Placental alkaline phosphatase and alpha-fetoprotein were demonstrated immunohistochemically in two of the three cases, with available tissue containing endodermal sinus tumor. Teratomatous metastases in ipsilateral cervical lymph nodes were found in one patient with a pure teratoma; that patient is disease-free one year after surgery. Only nine previous examples of endodermal sinus tumor have been reported in the head and neck region, exclusive of the central nervous system. There is one other case in the literature of a congenital cervicothyroidal teratoma with metastatic disease. These six neoplasms illustrate the clinical and pathologic spectrum in this nosologically homogeneous, but morphologically diverse, category of tumors.

Alkaline Phosphatase↗

[Diagnosis and treatment of endodermal sinus tumor of the vagina].

OBJECTIVES: To describe the clinical characteristics and the role of chemotherapy in endodermal sinus tumor of the vagina. METHOD: Two patients with endodermal sinus tumor of the vagina were presented focusing on the clinical manifestations and outcome of the chemotherapy. RESULTS: Patient's age was quite young, 2 and 3 years old respectively. Vaginal bleeding and a polypoid and fragile tumor of the vagina were main clinical features. Elevated serum alpha-FP was found before chemotherapy and dropped dramatically to normal if the tumor was sensitive to chemotherapy. Diagnosis was made by pathology and alpha-FP immunohistochemical staining. Both two patient was well responded to cisplatin vincristine bleomycin (PVB) and cisplatin etoposide bleomycin (PEB) chemotherapy. Clinical and pathological complete remission was obtained after 2-3 courses of chemotherapy without radical surgery and radiotherapy. CONCLUSIONS: Endodermal sinus tumor of vagina in infant was very sensitive to the chemotherapy. Serum alpha-FP was very useful in diagnosis and monitoring of the disease.

Antineoplastic Combined Chemotherapy Protocols↗

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↗

Primary endodermal sinus tumor of the endometrium presenting as "recurrent" endometrial adenocarcinoma.

BACKGROUND: Primary endodermal sinus tumor of the endometrium is an extremely rare malignancy with few reports in the world literature. CASE: A case of primary endodermal sinus tumor of the endometrium is presented. The case is unusual in several aspects: it occurred in a patient with a history of breast cancer and long-standing tamoxifen use, and was diagnosed only after presenting as an apparent unexpected recurrence of endometrial adenocarcinoma. The tumor recurred despite initial cytoreductive surgery and combination chemotherapy. CONCLUSION: Rare types of endometrial cancers may present as unexpected recurrences of previously resected endometrial adenocarcinomas. Appropriate therapy depends on obtaining sufficient tissue to establish an accurate diagnosis to ensure selection of proper chemotherapeutic agents.

Adenocarcinoma↗