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[Endodermal sinus tumor of ovary--analysis of 35 cases].

Thirty-five patients with pure endodermal sinus tumor of the ovary treated in our hospital from 1962 to 1984 are reported. There were 8 Stage I, 13 Stage III lesions and 14 with recurrent tumor from other hospitals. All the patients were treated by surgery followed by chemotherapy including single drug or combined chemotherapy. Five of 7 patients on high-dose DDP (120 mg/M2) responded. Two of the Stage I patients survived for 33 and 39 months. One of 2 patients by AcFuCy regime was free of tumor for 82 months. Four of 6 patients with advanced tumor treated by VAC regime responded, but only one was alive without evidence of tumor for 30 months. In 21 patients treated with other combination or single drug chemotherapy before 1980, only one (Stage Ia) is alive. It is indicated that surgery combined with multi-drug chemotherapy is the best management and high-dose DDP, VAC and AcFuCy are effective in treating pure endodermal sinus tumor of the ovary. Monitoring of serum AFP levels of the patients with endodermal sinus tumor can predict the course of the disease.

Adolescent↗

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↗

[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↗

Establishment and characterization of a human ovarian endodermal sinus tumor cell line--producing specific type of alpha-fetoprotein subfraction.

A cell line was established from an endodermal sinus tumor of the ovary from a 57-year-old Japanese woman, obtained on July 22, 1982. Histological study of the resected tumor revealed a mixture of reticular, solid and cystic patterns, being consistent with the characteristic feature. The cell line, designated as human Akimoto endodermal sinus tumor (HAEST), was subcultivated over 50 times during 29 months. The cells were spindle or columnar in shape and showed pleomorphic and neoplastic features. The modal chromosome number was stable at diploid range and the marker, large acrocentric, chromosome was identified. The cells were transplanted into the cheek pouch of hamsters and produced an endodermal sinus tumor. alpha-Fetoprotein subfractions in the conditioned media were studied by a modified method of lectin affinity crossed-line immunoelectrophoresis, and we found that the HAEST cells produced concanavalin A nonreactive subfraction, lentil lectin weakly reactive subfraction, phytohemagglutinin-E reactive subfraction and phytohemagglutinin-E nonreactive subfraction, which were predominantly synthesized by the fetal yolk sac, at an early stage of gestation. From these findings, we concluded that the HAEST was indeed an endodermal sinus tumor cell line. This line is expected to have a wide application for various laboratory studies.

Animals↗

Experience with the management of primary endodermal sinus tumor of the mediastinum.

Ten male patients with extensive primary endodermal sinus tumor of the mediastinum were treated with chemotherapy (with or without surgical excision and radiation therapy) between 1977 and 1985. Three patients, treated with cyclophosphamide-vincristine-based chemotherapy, died 1.5, 2.5, and 6.0 months, respectively, after initial diagnosis. Of the seven patients treated with cisplatin-based chemotherapy, one patient died of septicemia at 2.5 months after diagnosis and was disease-free at autopsy examination. Three other patients died of progressive disease at 7.0, 13.0, and 14.0 months, respectively. The three survivors remain alive at 17.0, 31.0, and 40.0 months from diagnosis; all are without evidence of disease. Of the three patients who underwent excision of the residual mediastinal tumor after cisplatin-based chemotherapy, persistent local disease was found in two patients. One of these two patients died of recurrent disease. All surviving patients had surgical resection of the tumor either before or after cisplatin-based chemotherapy, with or without radiotherapy and the timing of therapeutic interventions was guided by changes in the serum alpha-fetoprotein concentrations after initial therapy and during follow-up. Our experience suggests that the optimal management of patients with primary mediastinal endodermal sinus tumor requires an aggressive multidisciplinary approach guided by the extent of the tumor and the serum tumor marker levels.

Adolescent↗

Alpha-fetoprotein, prealbumin, albumin, alpha-1-antitrypsin and transferrin as diagnostic and therapeutic markers for endodermal sinus tumors.

According to Gitlin, alpha-fetoprotein (AFP), albumin, prealbumin, alpha-1-antitrypsin and transferrin are normal products of the human yolk sac. They are expected to reappear in human endodermal sinus tumor (yolk sac tumor). The synthesis of alpha-fetoprotein and other serum proteins by human endodermal sinus tumor was studied in the culture cells and in the tumor tissue transplanted into nude mice. The results gave evidences of synthesis of some of these proteins including alpha-fetoprotein and alpha-1-antitrypsin. Serum concentrations of these proteins were studied in eight children having endodermal sinus tumors. Serum AFP levels were abnormally high in all cases, whereas concentrations of other serum proteins were almost within normal ranges. This might be simply reflected by the fact that pre-albumin, albumin, alpha-1-antitrypsin, and transferrin are already present in large quantities in sera of normal subjects while alpha-fetoprotein is present only in a negligible quantity. Alpha-fetoprotein, as a diagnostic and therapeutic marker of endodermal sinus tumor, showed good correlation to the tumor growth. Serum AFP concentrations declined almost to 0 ng/ml with a half-life of 4 days when surgical removal was complete, whereas serum AFP decreased only to 100-200 ng/ml with radiation and chemotherapy alone.

Adolescent↗

Management of ovarian endodermal sinus tumor.

OBJECTIVE: To evaluate the role of combination chemotherapy and the optimal cycles of treatment in improving the prognosis of ovarian endodermal sinus tumor, and to study the relationship between the type of surgical management and the outcome of the disease. PATIENTS AND METHOD: Sixty-three patients with ovarian endodermal sinus tumor were divided into 3 groups according to the postoperative chemotherapy they had received. Group 1 (37 patients) patients were treated with at least 6 cycles of VAC (vincristine, actinomycin-D and cytoxan) or 4 cycles of PVB (cysplatin, vincristine and bleomycin). Group 2 (17 patients) patients were treated with VAC in less than 6 cycles or PVB in less than 4 cycles. Group 3 (9 patients) patients received no VAC or PVB but some other drugs like TSPA, 5FU, MTX and cytoxan in various combinations. The sustained remission rates and survivals were compared among these 3 groups. For patients with full courses of treatment with VAC or PVB (Group 1), different types of surgical management were studied about their relationship with the outcome of the disease. RESULTS: The persistent remission rates are 81.8%, 23.5% and 11.8% for group 1, group 2 and group 3 patients respectively (P < 0.001). The survival curve of group 1 is very much different from that of group 2 and group 3 patients. With full courses of chemotherapy with VAC or PVB, it appears that the different types of surgical managements (unilateral vs bilateral adenectomy; with vs without systemic lymphadenectomy; residual tumor < 2 cm vs > 2 cm) did not show definite relationship with the outcome of the disease. CONCLUSION: Combination chemotherapy with VAC or PVB dramatically improved the prognosis of ovarian endodermal sinus tumor but it should be emphasized that the favorable results could be obtained only when the treatment is given on time and in optimal cycles. Although ovarian endodermal sinus tumor is chemosensitive, appropriate surgical treatment is still important, however, the surgical techniques need some further studies.

Antineoplastic Combined Chemotherapy Protocols↗

Endodermal sinus tumor of the nasopharynx and previous mature congenital teratoma.

Congenital teratoma of the nasopharynx is a rare tumor that has shown consistently benign behavior. In contrast, endodermal sinus tumor, even more unusual nasopharyngeal germ cell tumor, displays consistently aggressive behavior with most reports documenting early death of the patient. We report the clinicopathological features of a case of endodermal sinus tumor of the nasopharynx that developed in a 3-year-old girl who had a mature teratoma excised from the same site in the neonatal period. Local recurrence of the endodermal sinus tumor was followed by disseminated disease and death within 18 months.

Child, Preschool↗

Primary endodermal sinus tumor of the cerebellar hemisphere: a case report with review of the literature.

Primary intracranial endodermal sinus tumors, which have been regarded as a rare histologic subtype, usually arise in the pineal and suprasellar regions and are often associated with components of other germ cell tumors. We report an extremely rare case of pure primary endodermal sinus tumor found in the cerebellar hemisphere. A 3-year-old boy was admitted to our institution because of gait disturbance, vomiting and deteriorated mental state. MR imaging revealed the presence of a round mass with heterogeneous enhancement in the left cerebellar hemisphere. Radical surgical removal of the tumor was performed, followed by adjuvant chemotherapy, consisting of etoposide, carboplatin and bleomycin. The patient has since attended regular follow-ups, without any neurological deficit or signs of recurrence in the 4 years since diagnosis.

Antineoplastic Combined Chemotherapy Protocols↗

Emergence of mature teratoma following treatment of sacrococcygeal endodermal sinus tumor: CT and MR imaging with pathological correlation.

We report a case of pseudoretroconversion in which, clinically and pathologically, it appeared that an endodermal sinus tumor retroconverted to mature teratoma. An 8-month-old white female infant with a sacrococcygeal tumor, diagnosed by biopsy as endodermal sinus tumor (yolk sac tumor), was treated with chemotherapy. The residual tumor after treatment was mature teratoma confirmed by imaging and by pathological examination. We present the computerized tomography (CT) and magnetic resonance imaging (MRI) findings with pathological correlation.

Endodermal Sinus Tumor↗

Endodermal sinus tumor of the infant vagina treated exclusively with chemotherapy.

Endodermal sinus tumor (EST) of the vagina is a rare malignancy usually diagnosed before 3 years of age. Historically, the approach to therapy has included radical surgical resection, with adjuvant irradiation, and chemotherapy. An infant presented with vaginal bleeding, imaging evidence of a vaginal mass and an elevated alpha-fetoprotein level. Examination under anesthesia with vaginal biopsies confirmed the diagnosis of an EST (yolk sac) tumor of the vagina. After 5 cycles of chemotherapy, the alpha-fetoprotein had normalized and repeat vaginal biopsies for suspected residual disease was negative for malignancy. To allow preservation of sexual and reproductive function, chemotherapy as a sole modality of treatment for EST should be considered.

Antineoplastic Combined Chemotherapy Protocols↗

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 in pregnancy: report of a case and review of the literature.

The endodermal sinus tumor is a rare germ cell tumor characterized by extremely rapid growth and grave prognosis. The case of a 17-year-old black primigravida with a 19-week intrauterine gestation and concurrent endodermal sinus tumor of the ovary is presented. This case is the eighth reported case of this tumor during pregnancy. alpha-Fetoprotein and other oncofetal antigens are postulated as agents of rapid growth of this tumor. It is suggested that immunotherapy with antibodies against alpha-fetoprotein and other oncofetal antigens might change the unfavorable outcome of this tumor.

Adolescent↗

[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 the pineal region: case report.

This is a case report of an endodermal sinus tumor occurring in the pineal region of a 12-year-old Caucasian boy. Germ cell tumors of this type demonstrate a selective overgrowth of yolk sac endoderm associated with extraembryonic mesoblast and arise perferentially in the gonads of young children. This is the ninth known report of this tumor in the world literature. Its occurrence in the posterior third ventricle again confirms that pineal region germ cell tumors are histologically analogous to germ cell tumors originating in the gonads. A disturbance in the region of the primitive streak is thought to account for the exclusive midline location of this tumor in extragonadal sites. This patient was treated with a combination of vincristine, actinomycin D, and cyclophosphamide, and concomitant cobalt irradiation. For endodermal sinus tumors in the pineal region, this combination appears more effective than previously reported treatment by irradiation alone.

Adolescent↗

Endodermal sinus tumor of the mediastinum. A report of seven cases and review of the literature.

Primary mediastinal endodermal sinus tumor is rare, and to date only 49 cases have been described in the English-language literature. Seven new cases are reported. Light microscopic examination showed characteristic features including papillary, reticular, tubular and solid growth patterns, complete or incomplete Schiller-Duval bodies and intracellular or extracellular periodic acid-Schiff-positive material. Immunohistochemical studies showed alpha-1-antitrypsin in seven, alpha-fetoprotein in seven, keratin in six, and carcinoembryonic antigen in four cases. The beta subunit of human chorionic gonadotropin, albumin, fibronectin, and transferrin were not found in any case. Electron microscopic studies performed in four cases showed intracellular and extracellular basement membrane-like material, multiple large multivesicular bodies, desmosomes and microvilli. Mediastinal endodermal sinus tumor can and must be differentiated from poorly differentiated adenocarcinoma metastatic or extending to the mediastinum because of the distinctly different prognoses and therapies. In spite of modern chemotherapy, the prognosis of mediastinal endodermal sinus tumor remains poor. The single most important prognostic indicator is whether the tumor mass can be completely excised before or after chemotherapy.

Adolescent↗