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PubMed1991

Prognostic factors in yolk sac tumors of the ovary. A clinicopathologic analysis of 29 cases.

Twenty-nine ovarian cancer patients with yolk sac tumors and germ cell tumors with yolk sac tissue as a component of their disease (16 endodermal sinus tumor, 11 mixed germ cell tumors, one embryonal carcinoma, and one polyembryoma) were treated with cytoreductive surgery and combination chemotherapy. Prognostic factors were investigated in this group. Patients with Stage I disease had a more favorable prognosis (P less than 0.003) than those with Stages II and IV disease. The difference in prognosis was significant in cases where residual tumor was absent (P less than 0.003) and in cases where ascites was either absent or less than 100 ml in volume (P less than 0.05). Endodermal sinus tumor with either an intestinal (P less than 0.05) or microcystic pattern (P less than 0.01) was more common in survivors than in those who died. The age, preoperative serum alpha-fetoprotein level, maximum tumor size, and tumor weight had no significant correlation with prognosis. In advanced cases, chemotherapy regimens including cisplatin gave better results than those containing vincristine, dactinomycin, and cyclophosphamide (P less than 0.05). The optimal treatment of yolk sac tumors or tumors with yolk sac tissue as a component of the ovary is discussed in light of these results.

Adolescent↗
PubMed1976

Malignant mixed germ cell tumors of the ovary. A clinical and pathologic analysis of 30 cases.

The clinical and pathologic features of 30 ovarian mixed germ cell tumors (neoplasms containing combinations of malignant germ cell elements) were studied to determine their behavior and to compare them with pure forms of malignant germ cell tumors. Dysgerminoma was the most common constituent, found in 24 (80%), followed by endodermal sinus tumor in 21 (70%), teratoma in 16 (53%), choriocarcinoma in 6 (20%), and embryonal carcinoma in 5 (16%). The actuarial survival for the entire group was 46%, and for patients with Stage I tumors it was 50%. The most important factors in predicting the prognosis for patients with Stage I disease was the size and the histologic composition of the neoplasm. If more than one-third of a Stage I neoplasm was composed of endodermal sinus tumor, choriocarcinoma, or Grade 3 teratoma, the prognosis was poor, whereas if the tumor contained less than one-third of these components or contained combinations of dysgerminoma, embryonal carcinoma, or Grade 1 or 2 teratoma, the prognosis was excellent. All patients whose neoplasm was less than 10 cm in maximum diameter survived, regardless of the composition of the tumor. Positive pregnancy tests in nonpregnant patients reflected the presence of either frank choriocarcinoma or scattered syncytiotrophoblastic giant cells. The latter did not appear to alter the prognosis. The finding of elevated serum levels of human chorionic gonadotropin (hCG) in 38% of the nonpregnant patients suggested that serial serum assays for hCG might be useful in staging and monitoring the response to treatment in these patients.

Adolescent↗
PubMed1975

Primary endodermal sinus (yolk sac) tumor of the liver. First reported case.

A primary endodermal sinus (yolk sac) tumor of the liver occurring in an 18-month-old boy is described. Although several examples of extragonadal endodermal sinus tumors have occurred in other sites, this is believed to be the first reported instance of origin in the liver. An additional important feature was the detection of alpha-fetoprotein in a preoperative serum sample. Although no metastases were identified at the time of celiotomy, widespread metastases developed, and he died 6 1/2 months after an extended right hepatectomy was performed. Neither triple chemotherapy nor radiation therapy appeared to deter progressive spread of the neoplasm, although the metastases exhibited some radiosensitivity.

Alpha-Globulins↗
PubMed2001

Pelvic neoplasms in children.

The pelvis of the infant and child has different anatomic relationships than the adolescent or adult pelvis, and the knowledge of congenital anomalies of the rectum and bladder can assist in treating the primary neoplasms of the pelvis. The most common neoplasms are rhabdomyosarcomas of the bladder, prostate, and vagina; sacrococcygeal teratoma; and the germ cell tumors, including teratomas, endodermal sinus tumors, and the choriocarcinomas. Rapidly improving chemotherapy for all of these lesions has resulted in a changed role for the surgeon. Less radical resection of these tumors is being performed whereas it is necessary to have more precise histologic and genetic identification of the tumor and the specific anatomic location and extent of the tumor in the pelvis. Survival for all types of childhood pelvic neoplasms has improved dramatically under the influence of the combined children's cancer study groups, which have been functioning for the last 20 years. Because of the complexities of diagnosis and treatment of these pelvic neoplasms in childhood and because the survival rates have dramatically improved with the most current therapy, these children should be cared for in a children's center that is part of the major children's cancer study groups.

Child↗
PubMed1983

Steroid-receptor proteins in nonepithelial malignancies of the ovary.

Tissue samples from 20 patients with nonepithelial ovarian malignancies were assayed for cytosol estrogen (ERc) and in selected samples progestin-receptor (PRc) proteins. The nine germ cell malignancies assayed were low in ERc. One recurrent granulosa cell tumor had an ERc concentration of 36 fmole/mg cytosol protein (fmole/mg c.p.) while three other stromal tumors were low in ERc. Three of seven mixed mesodermal tumors had a high ERc concentration (34-91 fmole/c.p.). Two primary and one recurrent granulosa cell tumors and one endodermal sinus tumor had borderline to elevated PRc levels (18-254 fmole/c.p.). Four of seven mixed mesodermal tumors had borderline to slightly elevated PRc concentrations (13-22 fmole/c.p.). The low ERc in germ cell malignancies of the ovary is consistent with previous clinical observations that the growth of these tumors is not influenced by removal of the contralateral ovary. The high ERc levels detected in three of seven mixed mesodermal ovarian tumors and one of three granulosa cell tumors suggest that hormone-related therapy should be investigated in the management of patients with these malignancies.

Adolescent↗
PubMed1988

Alpha-fetoprotein producing endometrial adenocarcinoma: report of a case.

A case of endometrial adenocarcinoma which is producing alpha-fetoprotein (AFP) is described. AFP has been reported to be a tumor marker of gynecologic tumors, such as endodermal sinus tumor and embryonal carcinoma of the ovary. However, to our knowledge, there has been no report of AFP producing endometrial adenocarcinoma. The histologic study showed poorly differentiated adenocarcinoma of the endometrium and AFP was detected in the tumor cells by an immunoperoxidase-peroxidase-antiperoxidase technique.

Adenocarcinoma↗
PubMed2001

[Treatment of childhood genital malignancies].

OBJECTIVE: To present the clinical profile and the result of treatment in childhood genital malignancies. METHODS: Twenty seven childhood patients (< 12 years) with genital malignancies diagnosed and treated in Peking Union Medical College hospital were analyzed retrospectively focusing on the clinical characteristics and outcome of the treatment. RESULTS: Patients ranged in age from 2 to 12 years with mean 6.5 years at diagnosis. Of the 27 patients, 23 had ovarian malignancies including 21 (77.8%) germ cell tumor. Four of the patients had extragonadal tumors with 2 endodermal sinus tumor of the vagina, and 2 embryonic rhabdomysarcoma. In ovarian malignancies, abdominal mass and abdominal pain were main clinical features accounting for 77.8% and 74.1% respectively. In vaginal malignancies, vaginal bleeding and polypoid tumor of the vagina were important clinical manifestations. 91.3% (21/23) of the patients underwent only unilateral salpingo-oophorectomy with preservation of contralateral ovary and the uterus. Childhood genital malignancies were very sensitive to the chemotherapy. Postoperative vincristine, actinomycine, cyclophosphamide (VAC) or cisplatine, vincristine, blyomycine (PVB) chemotherapy was individualized according to the tumor site, International Federation of Gynecology and obstetrics stage and histopathology. Survival rate in our series was 71.4% during the follow-up time (mean 47.4 months). CONCLUSIONS: Malignant germ cell tumor of the ovary is the most common tumor in the childhood genital malignancies. Principle of primary surgery is preservation of fertility and chemotherapy is also considered as a very important treatment modality.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1976

[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↗
PubMedDate not supplied

The ultrastructure and histogenesis of male germ neoplasia with emphasis on seminoma with early carcinomatous features.

The range of ultrastructural morphology was studied in 107 male germ cell neoplasm to assess relationships among tumor subtypes and to consolidate diagnostic criteria. Eighty-three pure-pattern neoplasms including 47 seminomas, 26 embryonal carcinomas, 10 endodermal sinus tumors, and 24 mixed germ cell tumors were analyzed. In the seminoma category, 4 cases showing cell surface specialization in keeping with early carcinomatous transformation were noted. The finding suggested a closer link between seminoma and nonseminomatous germ cell tumors than had been traditionally recognized and was in keeping with other clinical, histologic, biochemical, and xenograft observations. Subtypes of nonseminomatous germ cell tumors also exhibited a continuum of ultrastructural morphology with some types such as embryonal carcinoma and endodermal sinus tumor often blending together. At a practical level, electron microscopy has been of value in selected differential diagnoses such as seminoma versus lymphoma.

Choriocarcinoma↗
PubMed1999

[Extraneural metastasis of pineal germinoma through a ventriculoperitoneal shunt, following histological change].

Primary germinoma of the central nervous system carries a good prognosis because of their radiosensitivity. Recurrence is rare and extraneural metastases are even more unusual. We report a unique case of a primary intracranial germinoma exhibiting complete response to radiotherapy, but recurring as an intra-abdominal yolk sac tumor. The presence of a VP shunt is thought to have facilitated metastatic spread of the intracranial neoplasm. An 21-year-old (corrected) male was admitted with headache and consciousness disturbance. Computed tomography (CT) revealed an enhanced tumor of the pineal region and hydrocephalus. A ventriculo-peritoneal shunt was emplaced immediately. No serum tumor markers such as alpha-fetoprotein or human chorionic gonadotropin were detectable. A test dose of radiotherapy (whole brain 20Gy) was given. The tumor size was remarkably decreased. Clinical diagnosis was germinoma. Additional whole brain radiation (total 45Gy) and whole spine radiation (20Gy) were given. The tumor and the hydrocephalus regressed completely and the patient returned to school. Three years later, he experienced a feeling of abdominal fullness. CT and echotomography of the abdomen showed a large peritoneal and intra-hepatic tumor. But MRI revealed no recurrent tumor of the pineal lesion or of the other areas in the central nervous system. Radiological and clinical findings showed no tumors in the testis, the retroperitoneal cavity, or the thymus. Laboratory investigation demonstrated elevated serum AFP (26,550 ng/ml). AFP level regressed after combined chemotherapy. However, the patient died due to pneumonia and multiple organ failure. Only needle necropsy was performed. The microscopic appearance of the peritoneal tumor was confirmed to be an endodermal sinus tumor. It was suspected to be a metastasis of the pineal tumor through the V-P shunt system.

Adult↗
PubMed1995

Ovarian malignancies in childhood and adolescence.

We retrospectively reviewed all cases of ovarian malignancies during a 10-year period at the Department of Obstetrics and Gynecology, University Medical School of Debrecen, Hungary. The experience with 16 cases: three epithelial tumors, one granulosa cell tumor, 11 germ cell tumors (six dysgerminoma, four teratoma, one endodermal sinus tumor), and one metastatic ovarian cancer is discussed. Malignant ovarian tumors can best be treated with conservative surgery, followed by adjuvant chemotherapy. Survival mainly depends on tumor type and stage at the time of diagnosis.

Adolescent↗
PubMed1994

Endodermal sinus (yolk sac) tumor of the urachus.

A rare case of endodermal sinus (yolk sac) tumor of urachal remnants in a two-year-old infant is described. The tumor was located in the upper part of the bladder and appeared to arise from urachal remnants. The tumor could be entirely removed. The patient is free of tumor after three years and eight months follow-up.

Chemotherapy, Adjuvant↗
PubMed1990

Surgical treatment for recurrent ovarian malignancies.

Eighty-nine cases of recurrent ovarian malignancies which had undergone surgical treatment were studied. Debulking surgery is of great value in the treatment of primary ovarian carcinoma, but for recurrent carcinomas success depends upon the pathological type. The 5-year survival rate was 75% for patients with immature teratomas, but only 6.1% for those with epithelial ovarian carcinomas and 3.6% with endodermal sinus tumors. Two cases of recurrent dysgerminoma were both alive and well more than 10 years after treatment. So repeated debulking is worthwhile for recurrent immature teratoma and dysgerminoma, while in cases of epithelial type or endodermal sinus tumors successful debulking surgery should be followed by some new kinds of adjuvant therapy, otherwise only failure will result.

Carcinoma↗
PubMed1986

Second-look laparotomy in the management of malignant germ cell tumors of the ovary.

From 1970 to 1985, 53 patients with malignant nondysgerminomatous germ cell tumors of the ovary underwent second-look laparotomy after initial surgery and combination chemotherapy. Twenty-two patients had immature teratoma, 15 had endodermal sinus tumor, 15 had mixed germ cell tumor, and one patient had embryonal carcinoma. Thirty-one of the neoplasms were stage I, four were stage II, 17 were stage III, and one was stage IV. Two patients received a combination of actinomycin-D, 5-fluorouracil, and cyclophosphamide; four patients received vinblastine, bleomycin, and cisplatin; 44 patients received vincristine, actinomycin-D, and cyclophosphamide; and three patients received a combination of the last two regimens. Second-look findings were negative in 52 patients and positive in one patient who was subsequently salvaged with further chemotherapy. One patient with stage I endodermal sinus tumor relapsed nine months after a negative second-look laparotomy and died. Two patients with negative findings subsequently died of leukemia. Of 53 patients undergoing second-look laparotomy, three are dead (one of cancer and two of leukemia), and 50 patients are surviving without disease. Although the precise role of second-look laparotomy in patients with malignant germ cell tumors is yet to be established, possible indications are discussed.

Adolescent↗
PubMed1987

Placental alkaline phosphatase immunoreactivity in testicular germ-cell neoplasms.

We analyzed the sensitivity of a polyclonal antibody to placental alkaline phosphatase (PLAP) in labelling testicular germ-cell neoplasms, by utilizing the peroxidase-antiperoxidase technique. The immunoreactivity of 89 germ-cell tumors for PLAP was as follows: 98% of cases with seminomatous elements were PLAP-positive; 97% of embryonal carcinomas and 85% of endodermal sinus tumors also showed reactivity. Cytotrophoblastic cells were focally immunoreactive in one of two cases with choriocarcinomatous elements. Staining for PLAP was strongest and most diffuse in seminomas. Intratubular germ-cell neoplasia (ITGCN) or carcinoma in situ was present in 53 (84%) of 63 specimens that had adjacent seminiferous tubules available for evaluation; PLAP was demonstrated in 98% of these. In addition, the germ-cell elements in 11 gonadoblastomas were immunoreactive for PLAP. One of 17 cases of undescended testes had ITGCN that was also strongly immunoreactive, but the remaining 16 cases were negative. Five dysgenetic gonads without ITGCN were studied, and one was immunoreactive for PLAP. Three testicular biopsy specimens from infertile men without ITGCN were PLAP-negative. Our findings indicate that PLAP is a highly sensitive marker for seminomas, for the majority of embryonal carcinomas and endodermal sinus tumors of the testis, and for ITGCN.

Alkaline Phosphatase↗
PubMed2006

Activation of Wnt/beta-catenin signaling in distinct histologic subtypes of human germ cell tumors.

The molecular signaling pathways mediating human germ cell tumor (GCT) formation and progression are poorly understood despite a large number of studies detailing recurrent cytogenetic abnormalities. Germ cell tumors consist of multiple histologic subtypes and can also be divided into infantile/childhood or adolescent/adult tumors as well as gonadal or nongonadal sites of origin. All of these parameters are important in defining clinical outcome and in understanding the pathogenesis of these tumors. We utilized complementary DNA (cDNA) microarray analysis to identify differences in signal transduction pathways between 2 histologic subtypes of malignant ovarian GCTs (dysgerminomas versus ovarian endodermal sinus tumors). Hierarchical cluster analysis using only the genes involved in Wnt/beta-catenin signaling was able to distinguish these 2 tumor subtypes from each other. Wnt13 and beta-catenin showed significant differential expression patterns between the 2 tumor subtypes, and the results were confirmed by semiquantitative reverse transcriptase-polymerase chain reaction. Additional GCTs were studied for the expression of other members of Wnt/beta-catenin signaling, including Wnt13, frizzled, disheveled, low-density lipoprotein receptor-related protein 6, and beta-catenin. Differential expression levels were identified for several histologic subtypes of human GCTs. Finally, we prepared tissue microarrays containing GCTs from 83 different patients and demonstrated high levels of beta-catenin protein expression in 100% and nuclear accumulation in approximately 50% to 70% of all endodermal sinus tumors and immature teratomas (ITs). This pattern was independent of the patient's age. No nuclear accumulation of beta-catenin was observed in germinomas, embryonal carcinomas, or choriocarcinomas. These results indicate that activation of Wnt/beta-catenin signaling plays an important role in the pathogenesis of 2 histologic subtypes of human GCTs.

Adolescent↗
PubMed1989

Endodermal sinus (yolk sac) tumor in infants and children. A clinical and pathologic study: an 11 year review.

We reviewed the clinical features, treatment, and results of children with gonadal and extragonadal yolk sac (endodermal sinus) tumors seen in the King Faisal Specialist Hospital and Research Centre between 1976 and 1987. There were nine children (seven girls and two boys) with ages ranging from 7 months to 12 years (median of 3.5 years). Sites of origin included the vagina (two cases), face (two cases), sacrum (two cases), mediastinum (one case), ovary (one case), and testicle (1 case). All children had elevated alpha-fetoprotein (AFP) at diagnosis. One girl had complete surgical excision of an ovarian tumor at the time of diagnosis, and one boy had surgical excision of the testis. In the remaining seven children, the tumor was unresectable. Surgery was limited to a biopsy in six children. All patients received different combinations of chemotherapy, including vincristine (VCR), actinomycin D (Act-D), cyclophosphamide (Cyclo), adriamycin (Adria), bleomycin (Bleo), cis-platinum (CDDP), vinblastine (VBL), and VP-16. Of the nine patients, one was lost to follow-up while in remission, five died, one was lost to follow-up, and three are alive and disease-free at 15, 55, and 67 months from diagnosis. This review demonstrates an unusual preponderance of the extragonadal form of endodermal sinus tumor among our patients.

Antineoplastic Combined Chemotherapy Protocols↗