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Results for “Endodermal Sinus Tumor”

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PubMed1988

Genetic abnormalities in men with germ cell tumors.

We retrospectively reviewed the genetic abnormalities detected clinically in 455 men with advanced germ cell tumors referred for chemotherapy. Of the patients 49 had extragonadal and 406 had testicular germ cell tumors. Of 19 patients with mediastinal germ cell tumors 4 (21 per cent, 3 with teratocarcinoma and 1 with endodermal sinus tumor) had Klinefelter's syndrome. Three of these patients had a 47XXY and 1 had a 48XXYY karyotype. No Klinefelter's syndrome was observed among 30 consecutive patients with retroperitoneal germ cell tumors or among the 406 with testicular tumors. Karyotypes of 35 consecutive patients with testis cancer without evident congenital abnormalities showed normal chromosomal patterns. We found 2 patients with Down's syndrome and testicular tumor, for an incidence of 0.5 per cent (probably significant). We also describe 2 cases of nonseminomatous testicular cancer and Marfan's syndrome (0.5 per cent incidence versus a 5 of 100,000 incidence of Marfan's syndrome in the general population). Apparently, genetic abnormalities are increased in men with germ cell tumors and we discuss the significance of this association.

Adolescent↗

PubMed1990

The role of tumor markers in gynecologic oncology.

Human chorionic gonadotropin is a highly sensitive and specific tumor marker for gestational trophoblastic neoplasia that accurately reflects tumor volume and the clinical course of disease. In women with endodermal sinus tumors and embryonal carcinomas, alpha-fetoprotein usually is a reliable marker, and it accurately predicts the presence of yolk sac elements in mixed germ cell tumors. CA 125 is the most widely utilized tumor marker presently available for use in patients with epithelial ovarian cancer. It has utility in monitoring therapy and differentiating benign from malignant pelvic masses. Measurement of serial CA 125 levels in postmenopausal women may facilitate screening for occult early stage disease. In the future, monoclonal antibodies that recognize tumor-associated antigens may prove useful for radionuclide imaging.

Antibodies, Monoclonal↗

PubMed1989

[Treatment of primary mediastinal germ cell tumors].

Primary mediastinal germ cell tumors are clinically classified into mature (benign) teratomas and malignant germ cell tumors. Mature teratomas should be surgically excised after diagnosis, because unnecessary delay may result in the rupture of tumors or in malignant degeneration. The prognosis after surgical removal of tumor is good and there is no recurrence of tumor after complete excision. Malignant germ cell tumors are therapeutically classified into seminomatous and nonseminomatous germ cell tumors. Nonseminomatous germ cell tumors comprise immature teratoma, teratocarcinoma (malignant teratoma), embryonal carcinoma, yolk sac (endodermal sinus) tumor and choriocarcinoma. Treatments for patients with them require an multidisciplinary therapeutic approach with radiotherapy or chemotherapy combined with surgical intervention. The prognosis of patients with these tumors are poor, however seminomatous germ cell tumors have a better prognosis than that of nonseminomatous tumors, because they are responsive to radiotherapy or chemotherapy and long survivals over five years after treatment are not rare among them. An aggressive cisplatin-based combination chemotherapy is performed for patients with nonseminomatous tumors who have mostly the elevated serum levels of AFP or beta-HCG as tumor marker. The serum levels of tumor markers reflect precisely the biological behavior of nonseminomatous tumors. Patients with the normalised serum levels of tumor markers after an medical intervention may have a good long prognosis following radical resection of tumors, although the sustained high serum levels of tumor markers after treatment indicate a poor prognosis. Long survivals over five years after therapy are extremely rare among them.

Combined Modality Therapy↗

PubMed1993

Maxillary extragonadal germ cell tumor: a case report.

A single case report of a patient with primary extragonadal endodermal sinus tumor affecting the maxillary region is reported here. The patient was treated with combination chemotherapy consisting of bleomycin, cisplatin and etoposide. The child achieved complete remission and is now disease free for three years.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1985

Primary and secondary tumors of childhood involving the heart, pericardium, and great vessels. A report of 75 cases and review of the literature.

Between 1919 and 1981, 16 children with primary cardiac tumors (8 rhabdomyomas, 5 fibromas, 2 myxomas, and 1 rhabdomyosarcoma) and 59 children with secondary tumors of the cardiovascular system were seen at The Hospital for Sick Children in Toronto. Distant metastases in 45 children of the latter group, in descending order of frequency, were from non-Hodgkin's lymphoma, neuroblastoma, soft tissue and bone sarcoma, Wilms' tumor, and hepatoma, and involved the myocardium and pericardium. In the remaining 14 children, tumor thrombi from Wilms' tumor (9 cases), adrenal (2 cases) and hepatocellular carcinoma (2 cases), and endodermal sinus tumor (1 case) extended directly into the great veins and/or cardiac chambers. Children with primary and secondary tumors often present with nonspecific clinical, plain radiographic, electrocardiographic, and M-mode echocardiographic findings. Early recognition, utilizing special diagnostic procedures such as two-dimensional echocardiography, computerized axial tomography, angiocardiography, and inferior venocavography, followed by elective surgical resection of tumor under cardiopulmonary bypass and/or radiation and chemotherapy, offers patients with cardiovascular tumors the best chance of cure.

Angiocardiography↗

PubMed1987

Production of alpha-fetoprotein by human germ cell tumors in vivo and in vitro.

The production of alpha-fetoprotein (AFP) by human germ cell tumors was studied in surgical specimens, cultured cells and transplanted tumors in nude mice. AFP was detected most frequently in yolk sac (endodermal sinus) tumors, and it was also detected, though only occasionally, in embryonal carcinomas and in teratomas. AFP-positive cells in teratomas presented a glandular or hepatoid appearance. Our experimental data suggest that AFP production in embryonal carcinomas is due to the functional expression of yolk sac differentiation preceding its morphologic expression. Although the transplanted tumors maintained the productivity of AFP, the cultured cells were not successful in maintaining a continuous AFP production.

Adult↗

PubMed1989

An unusual embryonal tumor of the nasal cavity in a neonate--a nasal blastoma? A clinical, histologic, and ultrastructural study.

We report the clinicopathological and ultrastructural features of a primitive embryonal tumor which occurred as a polypoid mass in the nasal cavity of a newborn infant. Its primitive-appearing, glycogen-rich, small round cells exhibited epithelial characteristics histologically, immunohistochemically, and ultrastructurally. The tumor was initially diagnosed as embryonal carcinoma because of evidence for endodermal differentiation and lack of specific features of other types of small round cell tumors of childhood. However, it did not have the characteristic features of endodermal sinus tumor nor the anaplasia of the adult type of embryonal carcinoma. It had some resemblance to embryonic somatic tissues and the blastemal component of some of the primitive tumors of childhood. Because of its unique morphological appearance, which has never been reported, and its relatively indolent behavior after chemotherapy and radiotherapy, we believe that this is a distinctive primitive teratoid tumor which may be classified as nasal blastoma.

Humans↗

PubMed1985

Malignant sacrococcygeal teratoma--endodermal sinus, yolk sac tumor--in infants and children: a 32-year review.

Fifteen cases of malignant sacrococcygeal teratoma were treated at our hospital over the last 32 years. These included five cases of endodermal sinus (yolk sac) tumor that arose in otherwise benign or immature teratoma, and ten cases of pure endodermal sinus (yolk sac) tumor. Most were females, and only one was a neonate. The presence of symptoms almost always indicated local extension or distant metastases. According to Altman's classification, all but two of these malignant tumors were Types III and IV. The resectability was low, but with aggressive radiotherapy and chemotherapy, second look operations in five have been possible with increasing optimism for survival. Two of five patients with secondary resections are alive and tumor-free almost two years after therapy was discontinued. So far, only the newborn is a long-term (14 years) cure.

Child, Preschool↗

PubMedDate not supplied

Ovarian germ cell malignancy and human immunodeficiency virus (HIV) infection: a case report.

The occurrence of germ cell tumors in patients with human immunodeficiency virus (HIV) infection has not been previously reported. However, with the HIV pandemic in certain parts of the world, this association has become a reality. The approach to management as well as the role of adjuvant radiotherapy and chemotherapy is problematic as many of these therapies are toxic. The appropriate management of such tumors will remain elusive for sometime. The presentation, management, and outcome of ovarian endodermal sinus tumor in a HIV-infected patient is reported.

Adolescent↗

PubMed1987

[Preserving ovarian function in the treatment of epithelial and special (other) malignant ovarian tumors].

About 90% of malignant tumors of the ovary in Scandinavia develop from the germinal epithelium. There are great differences in the incidence rates between countries in the Western world and in Africa and Asia. The WHO classification of ovarian malignancies is generally used. The epithelial tumors comprise the serous, mucinous, endometrioid, clear cell, undifferentiated and mixed true carcinomas. In addition, borderline lesions of especially the serous and mucinous types are of interest when the question of preservation of ovarian function comes into notice. Conservative surgery, which means removal of only the afflicted ovary should be restricted to young women of the childbearing age who want to preserve the possibility of becoming pregnant. However, certain prerequisites must be fulfilled. The tumor must be located to one ovary only (Stage Ia) and must be either a borderline lesion or a Grade 1 true carcinoma of either the serous, mucinous or endometrioid type. There must be no ascites and peritoneal washings must be negative for cancer cells. Germ cell tumors are usually found in young women. Only the dysgerminomas are regularly bilateral in 10-15% of the cases. All other germ cell tumors are rarely bilateral. But both in borderline lesions, Grade 1 true carcinomas, and in germ cell tumors, a biopsy of the normal looking contralateral ovary should always be performed. Endodermal sinus tumors and immature teratomas may well be treated conservatively by surgery, but modern triple chemotherapy (VAC, PVB) must be added. Granulosa theca cell tumors are bilateral in only about 5% of the cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1990

[Treatment of malignant ovarian germ cell tumors with preservation of fertility].

Preservation of fertility was practiced in 28 young patients with malignant germ cell tumors from 1962 through 1987. The pathologic diagnosis was immature teratoma in 16, endodermal sinus tumor in 7, dysgerminoma in 2 and germ cell tumor of mixed type in 3. At laparotomy, 16 patients proved to have stage I disease, one stage II, 9 stage III and 2 stage IV disease. The tumor was confined to one ovary in all the 22 cases operated on for the first time and the preserved ovary and uterus were normal in all 6 referred cases for recurrent diseases. Postoperative chemotherapy was given to all patients except two with stage I immature teratoma. A persistent remission was achieved in 22 patients, while 5 patients died and one was out of contact. The duration of survival was more than one year for all patients, more than 3 years in 16 cases (72.7%) and more than 5 years in 13 cases (59.1%). The menstrual periods were normal in all cases except 3, of which 2 were below the age of 12 and one failed to menstruate at the age of 19 with a hypoplastic uterus and underdeveloped secondary sex characteristics. Among 12 married patients, 7 of 10 desirous of childbirth became pregnant during follow-ups. Of these, 6 had normal term-deliveries and one is currently pregnant. The preliminary conclusion is that preservation of fertility for young patients with malignant ovarian germ cell tumor is a safe and practicable procedure in the absence of involvement of the contralateral ovary and uterus.

Adolescent↗

PubMed1989

Malignant mediastinal germ-cell tumors in childhood: a report of two cases achieving long-term disease-free survival.

Two cases of malignant mediastinal germ-cell tumor are reported. Case 1 is a 20-month-old girl with endodermal sinus tumor (EST) and is, to our best knowledge, the third female pediatric case of mediastinal EST in the literature. Case 2 is a 15-year-old boy with malignant teratoma (mature teratoma with poorly differentiated squamous cell carcinoma). Both cases were treated by primary combination chemotherapy of vincristine, actinomycin D, and cyclophosphamide or vinblastine, cisplatin, and bleomycin, secondary operation (complete resection), radiation to the tumor bed, and some courses of consolidation chemotherapy. Malignant mediastinal germ-cell tumors have been considered to have a poor prognosis, being different from gonadal tumors; however, long-term survivals with no evidence of recurrence were achieved both in case 1 (for 55 months) and in case 2 (for 56 months).

Adolescent↗

PubMed1996

[Ovarian tumors in childhood].

Over 16 years 60 girls underwent surgery for ovarian disease. A total of 23 girls suffered from ovarian tumors, as was confirmed by surgery and histological examination in 18 cases. In five girls ultrasound, CT, and MRT examinations showed tumors, which later turned out to be ovarian cysts; in two cases a tumor was found despite the preoperative diagnosis of an ovarian cyst. A total of 15 patients underwent total resection of the ovary, two partial tumor resections and one exploratory laparotomy. Histological findings: eight teratomas, three dysgerminomas, two endodermal sinus tumors, one cystadenoma, one thecalutein cell tumor, one Sertoli cell tumor, one germinal cell tumor, and one angiofibroma. Five tumors were malignant. Surgery and histological examinations are necessary to exclude ovarian tumors.

Adolescent↗

PubMed1985

Malignant ovarian germ cell tumors: a review of thirty-six cases.

Thirty-six patients with malignant ovarian germ cell tumors were treated between 1972 and 1983, including 16 with immature teratoma, five with endodermal sinus tumor, seven with dysgerminoma, and eight with mixed germ cell tumors. The median age at presentation was 18 years and mean primary tumor diameter was 18 cm. Twenty-five of the 27 patients who were treated with multiple-agent chemotherapy underwent second-look procedures, only two of which revealed persistent malignancy. No patients have developed recurrence after a negative second-look operation. Two of the three patients with failure of initial chemotherapeutic regimens had complete remissions with second regimens. Two patients have died of malignancy, one who presented with a Stage IA mixed germ cell tumor and one noncompliant patient with a Stage IA, grade 2 immature teratoma. The other 34 patients are alive without evidence of disease from 21 to 141 months, with a median follow-up of 68 months. These data confirm that multiple-agent chemotherapy has dramatically improved the prognosis for patients with malignant nondysgerminomatous ovarian germ cell tumors.

Adolescent↗

PubMed1988

Embryology of the gonad with reference to special tumors of the ovary and testis.

The ovaries and testes have very similar development patterns until about the fourth month of embryonic life. A review of the similar development (until the fourth month) provides an explanation for the origin of tumors commonly associated with ovarian tissue appearing in the testis and vice versa. Embryonic surface epithelium gives rise to mesothelial tissue from which the common epithelial tumors arise in the ovary. Since these cells disappear from the testis when the tunica albuginea develops, this offers an explanation for the lack of common epithelial tumors in the testis. The embryonic gonadal tissue or sex cord epithelium and the primitive germ cells give rise to similar tumors in both the ovary and the testis. The extraembryonal tumors arise from the extraembryonal cells and trophoblastic cells, which give rise to the endodermal sinus tumors and choriocarcinomas, respectively. Since there is such a similarity in the development of the ovary and testis with the potential for residual cells to remain, an explanation is offered for the development of similar tumors in the ovary and the testis.

Female↗

PubMedDate not supplied

The significance of alpha-fetoprotein in the serum of patients with malignant teratomas and related gonadal neoplasms.

Sera from eleven patients with gonadal germ cell tumors were tested for alpha-fetoprotein (AFP) by counterelectrophoresis. Ovarian neoplasms containing either the endodermal sinus tumor (EST) or dysgerminoma and half of the testicular embryonal carcinomas elaborated the protein into the serum. The presence of AFP in the serum seemed to correlate with early metastasis or a fatal outcome. That the highest levels of AFP were found in cases of EST was in keeping with the theory that the tumor originates in cells of the yolk sac, a principal embryonic site of AFP synthesis.

Adenocarcinoma↗

PubMed1987

Intrapericardial yolk sac tumor in an infant girl.

This article is a report on the first known case of an intrapericardial yolk sac (endodermal sinus) tumor discovered in a 14-month-old girl. The patient presented with a systolic murmur, cardiomegaly, and manifestations of cardiac failure. At surgery a large, friable, intrapericardial mass with no evidence of extrapericardial spread was found. Histologic and immunohistochemical findings were diagnostic of yolk sac tumor. Following initial resection, chemotherapy, and a second resection 13 months after the first, the patient died 24 months after diagnosis. Tumor enlargement was associated with elevated serum alpha-fetoprotein levels.

Antineoplastic Agents↗

PubMed1991

Treatment of malignant ovarian germ cell tumors with preservation of fertility: a report of 28 cases.

Preservation of fertility was carried out in 28 young patients with malignant germ cell tumors of the ovary from 1962 through 1987. The pathologic diagnosis was immature teratoma in 16, endodermal sinus tumor in 7, dysgerminoma in 2, and germ cell tumor of mixed type in 3. At laparotomy, 16 patients were proved to have stage I disease, 1 had stage II disease, 9 had stage III disease, and 2 had stage IV disease. The tumor was confined to one ovary in all 22 cases treated for the first time, and the preserved ovary and uterus were normal in all 6 referred cases with recurrent diseases. Postoperative chemotherapy was given to all patients except two with stage I immature teratoma. Persistent remission was achieved in 22 patients; 5 patients died and 1 was lost to follow-up. The duration of follow-up was more than 1 year for all surviving patients, more than 3 years in 16 cases (72.7%), and more than 5 years in 13 cases (59.1%). The menstrual periods were normal in all except 3 cases, of whom 2 were below the age of 12 and one failed to menstruate at the age of 21 with a hypoplastic uterus and underdeveloped secondary sex characters. Among 12 married patients, 7 of 10 desirous of child-birth became pregnant during follow-up. Of these, 6 had a normal term delivery and 1 is currently pregnant. The preliminary conclusion is that preservation of fertility for young patients with malignant germ cell tumors of the ovary, regardless of the stage of the disease, is a safe and practicable procedure in the absence of involvement of the contralateral ovary and uterus.

Adolescent↗