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PubMed1998

Endodermal sinus (yolk sac) tumor of oral cavity originating from gingiva.

Endodermal sinus tumor is a malignant germ cell tumor which usually arises in gonads. Extragonadal endodermal sinus tumors in the head and neck are very rare. Here a gingival endodermal sinus tumor is reported. The lesion demonstrated typical microscopic features of the endodermal sinus tumors of gonads. The tumor cells showed alpha-fetoprotein immunoreactivity in immunohistochemical evaluation. The serum alpha-fetoprotein level was high. This is the first gingival case in the related literature.

Biopsy↗

PubMed1993

[Immunocytohistochemical studies of AFP producing gastric cancer--cytomorphology and characteristics of AFP positive cells].

In 24 cases (5.5%) out of 438 cases of gastric cancer showing serum AFP over 20 ng/ml, 14 cases (3.2%) showed AFP positive reaction in cancer cells by immunostaining. AFP localization in cancer cells classified into marginal type, granular type, diffuse type and inclusion type. Mixed combination of these types increased in high serum AFP level. Cytomorphology of AFP positive cells was classified to clear cell type, hepatoid cell type and round cell type. Clear cell type showed endodermal sinus like pattern in histomorphology. Clear cell type and hepatoid cell type were mixed, and transient. These types showed eosinophilic hyaline like globules, poor mucin production, and rich glycogen in cytoplasm. By immunohistochemistry showed positive rate in HCG:21.4%, PLAL:50. 0%, SP1:50.0%, CK:14.3% and alpha 1-AT:92.9%, and recognized reaction of germ cell tumor (endodermal sinus tumor) marker. We thought AFP producing gastric cancer suggested differentiation to germ cell tumor (endodermal sinus tumor). The AFP-positively stained cases were found in a high age group with advanced cancer located pylorus to corpus, medullary growth pattern, and high rate in liver metastases.

Adenocarcinoma, Clear Cell↗

PubMed1994

DNA ploidy analysis of pediatric germ cell tumors.

To determine the usefulness of DNA ploidy analysis in the biological assessment of pediatric germ cell tumors (GCTs), paraffin-embedded tissues from primary, recurrent, and metastatic neoplasms in 32 patients under age 18 were analyzed using static image cytometry. Primary sites included testis (eight), ovary (nine), sacrococcygeal region (10), central nervous system (three), and other extragonadal sites (two). The majority of the tumors were endodermal sinus tumors (ESTs) and teratomas. Of 13 teratomas, eight were diploid and five aneuploid. Immature ovarian teratomas of up to Grade II were diploid, whereas all of three Grade III immature teratomas were aneuploid. All sacrococcygeal teratomas, regardless of grade, were diploid; the development of EST in two sacrococcygeal teratomas was associated with the development of aneuploidy. Of 17 endodermal sinus tumors, five were diploid, five aneuploid, and seven tetraploid. All diploid ESTs occurred in the testis or extragonadally in patients 24 mo of age or younger. Infantile testicular ESTs were either tetraploid (one) or diploid (two), and ovarian ESTs were either aneuploid (two) or tetraploid (three). Comparing our data with that available for adult GCTs, we suggest that some pediatric GCTs may be pathogenetically distinct from adult GCTs. Adult testicular GCTs have been shown to be aneuploid, whereas infantile testicular ESTs are often tetraploid or diploid. This suggests a different pathogenesis for these groups of tumors and may explain their biological differences as well. The transformation of a sacrococcygeal teratoma into a malignant EST is poorly understood. Ploidy analysis presented suggests genetic alterations may accompany this transformation.

Adolescent↗

PubMed1995

Current management of malignant germ cell tumor of the ovary.

Malignant germ cell tumors are an uncommon type of ovarian cancer which account for fewer than 5% of the total in Western countries and 20% in Japan. In females younger than 20, they represent approximately two-thirds of malignant ovarian tumors. Immature teratoma, endodermal sinus tumor, dysgerminoma and mixed type account for the majority (more than 80%), while embryonal carcinoma and polyembryoma are very few. The age of the patients ranges from 6 to 69 years with a median of 16-20 years. Clinically, these tumors are characterized by rapid growth and extensive intraabdominal spread. The symptoms and signs range from 1 day to 6 months with a median of 4 weeks, and the patients usually present with abdominal pain, palpable mass, abdominal distention and vaginal bleeding, and a very few with amenorrhea and precocious puberty. The size of tumors varies from 7 cm to 40 cm with a median of 15-16 cm. The tumor is rarely bilateral (12-19%) and never so in cases of endodermal sinus tumor. Diagnosis depends mainly on age, abdominal symptoms, size and consistency of the tumor, and tumor markers AFP and hCG. Surgery is the first step of management followed by adjuvant therapy, which depends on the histologic type. Dysgerminoma is very sensitive to radiation while other germ cell tumors are not. A combination chemotherapy currently used is VAC or VBP. Both are highly effective. The VBP regimen seems to have a stronger cancerocidal effect, while the VAC regimen is less toxic. VAC produces excellent results in stage I, while VBP is more effective for advanced disease. Conservative surgery and a combination chemotherapy (VAC, VBP) are appropriate for young patients who desire to retain their fertility. Second-look laparotomy is still controversial. As long as AFP or hCG or both can be used to monitor the disease in patients positive for these sensitive and reliable markers, or in an early stage with complete resection, second-look laparotomy is not useful. Survival is associated with prognostic factors, i.e., histologic type, clinical staging operation, lymph node and residual tumor. Patients with endodermal sinus tumor or mixed type tumor had a poorer outcome. The survival rate was higher in patients with earlier disease (stage I or II) and those who underwent primary surgery. Metastasis to the lymph node is not related to prognosis. The presence and size of residual tumors after surgery were closely related to the prognosis.

Adolescent↗

PubMed1979

Endodermal sinus (yolk sac) tumors in infants and children.

Endodermal sinus tumors, as a specific entity, were first proposed and described by Teilum on the basis of morphological and histogenetic features. Recent work utilizing tumor markers (alpha-fetoprotein), immunofluorescent and electron microscopic studies have supported Teilum's original concept as to the origin of these tumors. Five typical presentations of endodermal sinus tumors seen in the pediatric age group were reviewed with reference to the original presentation, the utilization of tumor markers (alpha-fetoprotein) and the prognostic implications of this neoplasm.

Child↗

PubMed1978

Yolk sac tumors with pure and mixed polyvesicular vitelline patterns.

Four cases of polyvesicular vitelline tumor are presented; two were of a previously unreported pure type, and the other two were mixed with endodermal sinus tumor. The morphologic features of the vesicles favor an endodermal origin, as originally proposed by Teilum. Marked specialization of the vesicular lining cells, seen ultrastructurally, suggests a differentiation toward gut structures and mature yolk sac. One case of pure polyvesicular vitelline tumor showed massive erythropoiesis. We propose that the pure tumor reflects an intermediate degree of differentiation within the selectively endodermal yolk sac tumor group, that is, a further stage of organization than the endodermal sinus tumor. In our cases of pure polyvesicular vitelline tumor, the marked degree of differentiaiton was correlated with an improved prognosis, as in the case of the possible homologue of this tumor, the yolk sac tumor of the infant testis. In contrast, the two cases of the tumor admixed with endodermal sinus tumor illustrated the low survival rate expected in the pure endodermal sinus tumor; in these cases the metastases had no polyvesicular component. Because of the significance of such a difference in prognosis we emphasize the importance of an accurate diagnosis, suggesting that a large number of sections be taken in order to demonstrate any endodermal sinus tumor component that may be present, and that the possibility of pure polyvesicular vitelline tumor always be considered in the differential diagnosis of multicystic ovarian tumors.

Adult↗

PubMed1994

Posterior mediastinal endodermal sinus (yolk sac) tumor in a female patient.

Primary endodermal sinus tumor (yolk sac tumor) of the mediastinum is uncommon. Most patients are young and male, and the great majority of tumors are found in the anterior mediastinum. We report a case of primary posterior mediastinal endodermal sinus tumor occurring in a female patient. Surgical excision was performed and three courses of combination chemotherapy were subsequently given. The serum alpha-fetoprotein level returned to normal.

Adolescent↗

PubMed1985

Retroperitoneal germ cell tumors in childhood. A clinical and pathologic study of 11 cases.

During the last 54 years at the Children's Hospital 11 children (10 female, 1 male) had been treated for an extragonadal germ cell tumor arising in the retroperitoneum. There were eight teratomas (five mature, three immature), two endodermal sinus tumors and one tumor with a mixture of both components. Abdominal pain or discomfort was the usual presenting complaint, with the average age at diagnosis being 18 months. On physical examination, each child had a palpable abdominal mass usually localized in the upper quadrants. The finding of calcification, bone or teeth, on radiologic study was most helpful in establishing a preoperative diagnosis of teratoma. The preferred treatment for children with mature and immature teratomas is complete surgical resection; decisions regarding adjuvant therapy for patients with immature tumors must be evaluated on an individual basis. The prognosis remains guarded for children with unresectable primaries or those with endodermal sinus tumor. Of three tumor-related deaths, two were due to metastatic endodermal sinus tumor and one resulted from extensive local growth by immature teratoma. Successful management of children with endodermal sinus tumor requires surgery aimed at debulking or complete resection in combination with aggressive adjuvant chemotherapy.

Child, Preschool↗

PubMedDate not supplied

Testicular germ cell tumors in prepubertal children.

Pediatric testicular germ cell tumors are rare. Fifteen children, all less than 5 years of age, were evaluated and treated during February 1987 to July 1996. The median age was 18 months (range, 4-60 months). All were staged according to the Pediatric Oncology Group/Children's Cancer Study Group staging system. Seven patients had stage III disease. Histologically, 9 patients had pure endodermal sinus tumor, 1 had endodermal sinus tumor with embryonal carcinoma, 1 had embryonal carcinoma alone, 2 had immature teratoma, and 2 had mature teratoma. Six children were kept on surveillance. All others received chemotherapy with cisplatin, bleomycin, and vinblastine. The 10-year actuarial overall survival rate was 86.7%.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1987

Malignant ovarian tumors in children and adolescents.

Eighteen patients aged 4 to 18 years (median, 10 years) were treated at the Children's Cancer Research Center of The Children's Hospital of Philadelphia (CHP) from September 1973 to September 1983 for malignant ovarian tumors. The pathologic categories were endodermal sinus tumor (eight patients), embryonal carcinoma (three patients), pure dysgerminoma (three patients), adenocarcinoma (three patients), and malignant granulosa-cell tumor (one patient). In 1973 and 1975, respectively, two patients with endodermal sinus tumors were treated with surgical removal with or without radiation therapy (RT) and chemotherapy with vincristine (Vc) and actinomycin D+ cyclophosphamide + Adriamycin (Adria Laboratories, Columbus, OH) (ACAdr); both developed local recurrence within 12 months and died of tumor. Since 1977, four of six patients with endodermal sinus tumors have been managed successfully with surgery followed by chemotherapy with cisplatin (P), bleomycin (B), vinblastine (Vb), and ACAdr; none received RT. One died of recurrent tumor and the other died of congestive heart failure attributed to Adriamycin (Adr). All three patients with embryonal carcinoma were well after surgical excision alone (one patient) or surgery and chemotherapy with cisplatin + bleomycin + vinblastine (PBVb) + ACAdr (two patients). Treatment of the remaining seven patients included excision alone (two patients) or excision and chemotherapy (five patients) with various combinations of Vc, ACAdr, and P. One patient with granulosa-theca cell tumor also received whole abdominal RT (3000 rad by external beam), and died of congestive heart failure attributed to Adr. The other six children were free of detectable tumor 16 months to 8 years from diagnosis. Chemotherapy with PBVb and actinomycin D + cyclophosphamide (AC) should be used in the postoperative management of young persons with malignant ovarian tumors. Adr is of doubtful value and may be considerably toxic. RT is of limited use and usually is not indicated.

Adenocarcinoma↗

PubMed1981

Successful treatment of metastatic extragonadal endodermal sinus (yolk sac) tumor in childhood.

Two children with metastatic extragonadal endodermal sinus tumor who had failed VAC chemotherapy were treated with combination chemotherapy consisting of cis-platinum, vinblastine, doxorubicin HCl (Adriamycin) and bleomycin (cis-VAB). There was a complete response in both patients with one child remaining tumor-free 3 1/2 years after metastasis and 2 1/2 years after stopping therapy. The main toxic reactions encountered were associated with the use of cis-platinum, but these were manageable and resolved upon completion of therapy. Cis-VAB appears to be a safe and effective regimen for the treatment of metastatic endodermal sinus tumor. A study of the efficacy of cis-VAB in a larger group of children with endodermal sinus tumor of poor prognosis seems indicated.

Adolescent↗

PubMed1998

Genetic analysis of ovarian germ cell tumors by comparative genomic hybridization.

Ovarian germ cell tumors (OGCTs) show a heterogeneity that is not seen in their testicular counterparts and include benign mature cystic teratomas, intermediate immature teratomas, malignant germ cell tumors [GCTs (dysgerminomas, endodermal sinus tumors, and mixed GCTs)], and GCTs arising in dysgenetic gonads of 46,XY individuals. Comparative genomic hybridization was used to analyze 27 OGCTs for regions of relative gain or loss. The analysis of 21 malignant OGCTs (12 dysgerminomas, 6 endodermal sinus tumors, and 3 mixed GCTs) demonstrated genetic alterations similar to those reported in adult testicular GCTs. The most common regions gained include chromosomes 12p (16 of 21 tumors), 21 (10 of 21 tumors), 8 (8 of 21 tumors), and 1q (6 of 21 tumors). The most common region lost was chromosome 13. Regions of high-level gain were identified at 12p11-12 and 4q11. The profile of gains and losses was similar in the different histological subtypes within this category. One tumor presented in a 46,XY patient; this tumor was diploid and showed a gain of 12p. Immature teratomas (six cases) showed only one case with an abnormality, which was a gain of chromosome 14. We conclude that malignant OGCTs are genetically similar to those found in the adult testis; however, immature teratomas show no consistent gains or losses and are therefore different from those presenting in the adult testis. A review of the literature suggests that genetic abnormalities in this group may herald a worse prognosis. Lastly, OGCTs in dysgenetic gonads arise in a diploid rather than a tetraploid cell line, yet they also show a gain of 12p.

Adolescent↗

PubMed1995

Malignant germ cell tumors of the ovary: Hacettepe hospital experience.

BACKGROUND: To determine the most important prognostic factors in patients with malignant germ cell tumors of the ovary. METHODS: Age, histologic type, tumor size, stage, treatment mode, type of surgery of 67 patients with malignant germ cell tumors managed consecutively were evaluated retrospectively using multivariate analysis. RESULTS: The overall survival rate was 60.6%. This figure was 78.5% for dysgerminoma, 53.3% for immature teratoma, 12.0% for endodermal sinus tumor, 33.3% for mixed germ cell tumor and 100.0% for others. The patients with endodermal sinus tumor had significantly poorer survival than other groups. Among the factors analyzed for survival histologic type was found to be the most significant predictor for survival. CONCLUSIONS: Finding of histologic type as the strongest independent risk factor may be evaluated in correlation with the literature that these tumors have historically been investigated as dysgerminomatous and nondysgerminomatous tumors. Furthermore, endodermal sinus tumors had long been known for poor survival. Based on this data, despite advent of staging and effective chemotherapy, prognosis still is basically related to histologic type and histology of tumor should be taken into account while comparing results.

Adult↗

PubMed1990

Nuclear deoxyribonucleic acid content (ploidy) of endodermal sinus (yolk sac) tumor.

Paraffin sections from 30 endodermal sinus (yolk sac) tumors were Feulgen-stained, and nuclear DNA content (ploidy) was assessed through DNA cytophotometry using the microTICAS image analysis system. The series consisted of 20 ovarian, 8 testicular, 1 sacrococcygeal, and 1 mediastinal tumors. Of the 30 endodermal sinus (yolk sac) tumors 29 were shown to be aneuploid, whereas the remaining tumor which exhibited the recently described primitive intestinal or enteric pattern was diploid and may thus be considered a neoplasm showing a somewhat higher degree of differentiation as compared to tumors showing the other histologic patterns of endodermal sinus (yolk sac) tumors.

Aneuploidy↗

PubMed1998

Yolk sac tumor of the ear in a child.

OBJECTIVE: This study aimed to describe the clinical, histopathologic, and immunohistochemical findings of the second case of an endodermal sinus tumor of the ear. STUDY DESIGN: This clinical study is a retrospective case report combined with a literature review. SETTING: The patient was treated at the University Erasmus Hospital. INTERVENTION: Diagnosis was confirmed by computed tomographic scan and histologic analysis. The treatment was surgical. RESULTS: The patient underwent surgical treatment, and a locoregional relapse developed. The clinical evolution was in accordance with previous reports of extragonadal endodermal sinus tumor sites. The family refused chemotherapy, and the patient died 3 months after admission. CONCLUSIONS: An endodermal sinus tumor is a malignant germ cell tumor that usually involves the gonadal tissue. Extragonadal sites are rare, and different hypotheses have been evoked to explain these locations. Treatment options include surgical removal and chemotherapy. This is the second case of an endodermal sinus tumor of the ear.

Child, Preschool↗

PubMed2004

Orbital, middle cranial fossa, and pterygopalatine fossa yolk sac tumor in an infant.

A yolk sac tumor, also known as an endodermal sinus tumor, was diagnosed in a 15-month-old infant who presented with rapidly progressive right eye proptosis. Imaging of the orbits and brain revealed a mass in the right orbit, middle cranial fossa, and pterygopalatine fossa. A lateral orbitotomy was performed to take a biopsy specimen and to partially debulk the tumor secondary to signs of optic nerve compromise. The biopsy specimen revealed a yolk sac tumor, and the patient underwent systemic chemotherapeutic treatment. Because orbital endodermal sinus tumors have been infrequently reported, there are no firm prognostic or treatment guidelines. Our case demonstrates that early recognition, limited orbital debulking, and chemotherapy can have an excellent short-term outcome.

Antineoplastic Agents↗

PubMed1986

Transitional (cylindric) cell carcinoma with endodermal sinus tumor-like features of the nasopharynx and paranasal sinuses. Clinicopathologic and immunohistochemical study of two cases.

We report two cases of adult patients with primary tumors in the nasopharynx and paranasal sinuses, which were characterized histologically by areas of transitional cell (cylindric or "schneiderian") carcinoma and other foci virtually indistinguishable from endodermal sinus tumor (yolk sac carcinoma). Immunohistochemical studies supported the impression of yolk sac differentiation. Both patients were treated with irradiation and multidrug chemotherapy. One patient died of uncontrollable recurrence of tumor, whereas the other developed locally recurrent, transitional cell carcinoma in the absence of yolk sac tumor. The concurrence of these patterns of carcinoma, which has been undescribed previously to the best of our knowledge, seems to be associated with more aggressive biologic behavior than pure transitional cell carcinomas of this region.

Adult↗

PubMed1985

Immunocytochemical evidence of heterogeneous origin of alpha-fetoprotein in immature teratoma of the ovary.

alpha-Fetoprotein (AFP) has been found to be clinically important in the management of patients with gynecologic malignancies. Serum AFP levels are used as tumor markers for ovarian tumors, namely endodermal sinus tumors (EST) and embryonal tumors. In two cases of immature teratoma of the ovary without a yolk sac component, evidence for AFP production was found in a wide variety of cells of both endodermal and ectodermal origin suggesting a heterogeneous origin of AFP. To complement this finding, a summary of the ontology of AFP along with current and potential uses of an antigenically active tumor marker are presented.

Adult↗