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Pregnancies following conservative treatment of malignant ovarian tumors.

Sixteen patients with malignant ovarian tumors were treated conservatively; 1 had a serous cystadenoma of low potential malignancy; 6 had a mucinous cystadenoma of low potential malignancy; 2 had a pure dysgerminoma; 2 had a mature solid teratoma; 3 had a mucinous cystadenocarcinoma; and 2 had an endodermal sinus tumor. Postoperative chemotherapy was given in 12 cases and 1 patient with a pure dysgerminoma had radiotherapy. Eighteen pregnancies occurred in 13 patients. One full-term and one 8 month premature infant died in utero, and 2 infants had malformations; the other 14 infants are all well. The patient with moderately differentiated mucinous cystadenocarcinoma who delivered a premature infant and 2 patients with endodermal sinus tumors died of recurrence.

Adolescent↗

[Malignant ovarian tumors in childhood].

Malignant ovarian tumors only account for 3 per cent of all malignancies under 15 years of age, and more than two third of them are of germ cell origin. Abdominal mass and/or pains are the usual revealing symptoms and abnormal sexual prematurity is infrequently encountered. Diagnostic ultrasonography is currently the most useful imaging investigation. Alpha-foeto-protein (alpha-FP) serum determination is mandatory before surgery to indicate the presence of endodermal sinus tumor cells. Surgical procedure is in childhood usually restricted to unilateral salpingo-oophorectomy, if allowed by tumoral spreading which is carefully searched in the abdominal cavity. Thus will be defined, along with the post-operative lymphangiography, the extension of the tumor, according to the staging system of Wollner (Memorial Hospital NY) more than the FIGO. Malignant teratomas are the most frequent malignant germ cell ovarian tumors in young females. They may realize pure or mixed pathological types of immature teratoma, embryonal carcinoma, endodermal sinus tumor and very seldom choriocarcinoma. Serial assays of serum alpha-FP are of utmost value to follow therapeutic progress or detect recurrences. Since new sequential multidrug protocol including, vincristine, actinomycin D, cyclophosphamide, adriamycin, méthotrexate and cis-platinum, protracted survivals are to be expected in a higher number of patients with localized stages and even in some with advanced disease. Value of extended pelvi-abdominal and/or lymph node radiotherapy is still under evaluation. Ovarian dysgerminomas are radiosensitive tumors in which chemotherapy is as a rule not indicated. Relapses may be most often prevented by a prophylactic irradiation of iliac ipsilateral, lumboaortic, mediastinal and supraclavicular nodes. Among functional ovarian tumors, mainly granulosa cell types with isosexual precocity, malignant tumors are so rarely encountered that no complementary treatment has to be performed after a complete resection. Cystadenocarcinoma or borderline ovarian epithelial tumors occur after puberty and require the same treatment as in adult though keeping a conservative view. Ovarian deposits of Burkitt's tumor may account in some exposed african areas for most ovarians malignancies in childhood. Gonadoblastomas with both stromal and germ cell elements are exclusively to be found in dysgenetic gonads.

Adolescent↗

Extragonadal endodermal sinus (yolk sac) tumor. Angiographic findings and literature review.

The angiographic and pathologic features of an endodermal sinus tumor (EST) arising from the retroperitoneum in a 19-month-old boy are presented. Although rare instances of extragonadal origin of EST have been reported, this is the first detailed description of the angiographic abnormalities. The angiographic appearance was that of a primarily retroperitoneal tumor with parasitized feeding arteries from the liver and right kidney, early filling venous radicles, sinusoidal pooling of contrast material, and inferior vena caval obstruction.

Aortography↗

Fertility-preserving treatment for patients with malignant germ cell tumors of the ovary.

AIM: The aim of this study was to investigate whether fertility preservation influences the clinical outcome in patients with malignant germ cell tumors of the ovary (MGCTO). METHODS: A case study analysis was performed on patients with MGCTO treated at Kurume University Hospital between 1986 and 2004. Thirty-five patients were included in the study, 14 with immature teratoma, 11 with dysgerminoma, eight with endodermal sinus tumor, and two with mixed germ cell tumor. Twenty-three patients had International Federation of Gynecology and Obstetrics stage I (Ia, 11; Ib, 2; Ic, 10), one had stage II, seven had stage III, and four had stage IV disease. RESULTS: Five patients with stage III or IV disease received radical surgery. Thirty patients underwent conservative surgery. As the adjuvant treatment, 30 patients received chemotherapy, while five patients did not receive any chemotherapy. The overall survival rate was 97.1%. One patient died of the disease. She was 13 years old with a stage IV endodermal sinus tumor. Twelve have attempted conception, and eight have achieved at least one pregnancy (66.7%). CONCLUSIONS: Irrespective of the stage of the disease, conservative surgery and adjuvant chemotherapy for MGCTO can achieve a favorable outcome in terms of survival and fertility.

Adolescent↗

Diagnosis of a contralateral second testicular carcinoma by F18-FDG PET.

BACKGROUND: In testicular carcinoma the early diagnosis is very important because with an early therapy there are good chances for long-term survival (50-90%). Metastases of a testicular carcinoma are at first lymphogenous, hematogenous only in late stages. CASE REPORT: This is a case report about a 28-year-old man, whose testicular carcinoma (left testis) had already been operated on (unripe teratoma with parts of an embryonic carcinoma, an endodermal sinus tumor and a chorion carcinoma). Because of the elevated tumor marker AFP an FDG PET (F18-fluorodeoxyglucose positron emission tomography) investigation was made. CT (covering thorax, abdomen and pelvis) and ultrasound of the testis showed no pathological results. In the FDG PET a significant pathological FDG uptake in the right testis was found. Histology showed an unripe teratoma with parts of an embryonic carcinoma, an endodermal sinus tumor, and a chorion carcinoma. It was a second carcinoma of the contralateral testis. CONCLUSION: F18-FDG PET was a sensitive and reliable modality for diagnosis in this patient.

Adult↗

Endodermal sinus (yolk sac) tumor elements in testicular germ-cell tumors in adults: comparison of prospective and retrospective studies.

The incidence of endodermal sinus tumor (EST) or yolk sac tumor (YST) elements has been studied in two series of testicular germ-cell neoplasms in adults. One series, consisting of 200 germ-cell neoplasms seen from 1053 through 1968, was studied retrospectively, and the other, consisting of 147 cases seen from May 1974 through February 1979, was studied prospectively. Excluding the cases of pure seminoma, EST(YST) elements were found in 21 (28.7%) of 73 cases in the retrospective series and in 27 (44.4%) of 61 cases in the prospective series. The EST(YST) elements were in all cases admixed with other neoplastic germ-cell elements and varied from microscopic foci to being the predominant element within a tumor. The EST(YST) elements were histologically similar to infantile EST(YST) and EST(YST) in other locations. Serum alphafetoprotein (AFP) was determined in the majority of patients in the prospective series, and there was good correlation between the presence of EST(YST) elements within the tumor and elevated levels of AFP. The results of the present study indicate that EST(YST) elements occur quite frequently in testicular germ-cell neoplasms in adults and provide an explanation for the raised levels of serum AFP found in many adults with testicular germ-cell tumors. The results emphasize the importance of a thorough and careful pathologic examination of testicular germ-cell tumors, and the value of AFP as a tumor marker in patients with EST(YST). The results also lend further support to the view that EST(YST) elements found in testicular germ-cell tumors in adults are homologous with infantile EST(YST) and that EST(YST) is a distinctive and specific type of germ-cell neoplasm and should be included as such in the classification of testicular tumors.

Adolescent↗

Embryonal carcinoma of the testis.

Long-term survival rates were correlated with selected clinical features in 479 patients with embryonal carcinoma of the testis and 33 patients with endodermal sinus tumor (infantile embryonal carcinoma, yolk sac tumor). In the period 1977 to 1982 embryonal carcinoma accounted for 26.8% of newly diagnosed germ cell tumors and 43% of nonseminomatous germ cell tumors entered in the Centralized Cancer Patient Data System. Among patients with embryonal carcinoma, over 80% were diagnosed in the 15-to-34 year age group. Seventy-four percent of the patients had metastatic disease at the time of diagnosis, and 50% of these had distant metastases, attesting to the aggressiveness of embryonal carcinoma and its tendency to early hematogenous spread. Despite the highly malignant nature of the tumor, the overall 5-year survival rate with treatments used was an excellent, 88%. Survival was correlated with the extent of disease at the time of diagnosis; the 5-year actuarial survival rates for patients with localized, regional, and distant disease were 98%, 96%, and 74%, respectively. Endodermal sinus tumor was uncommon (1.8% of all testicular germ cell tumors), occurred predominantly in the younger age group (0-24 years), and in 50% of the cases was localized to the testis. The survival rate for the 33 patients with this form of tumor was slightly worse than for the "adult form" of embryonal carcinoma. The authors conclude that survival of patients with embryonal carcinoma has greatly improved over the last decade as a result of improved methods for early detection of metastatic deposits and the effectiveness of newer chemotherapies in the treatment of disseminated disease.

Age Factors↗

Alpha1-antitrypsin and alpha-fetoprotein. Protein markers in endodermal sinus (yolk sac) tumors.

A combined immunocytochemical and quantitative serum and tissue study was performed on a group of endodermal sinus (yolk sac) tumors, localizing and measuring both alpha1-antitrypsin (AAT) and alpha-fetoprotein (AFP) in tumor tissue and patient sera. Utilizing indirect immunofluorescent and triple-sandwich immunoperoxidase methods, both proteins were demonstrated within intra- and extracellular periodic acid-Schiff-positive hyaline globules characteristic of the tumor, as well as within the cytoplasm of tumor epithelial cells lining endodermal sinuses, where AAT deposition predominated. Tumor tissue extracts confirmed the presence of significant quantities of both proteins, and pretreatment serum elevations of both showed a parallel decline during therapy. In this study, AAT is characterized as a tumor protein marker for the first time, and a parallelism between AAT and AFP is demonstrated in both serum and tumor tissue. These findings represent additional supportive evidence for the yolk sac origin of endodermal sinus tumors in man.

Child↗

Transient cortical blindness during chemotherapy (PVB) for ovarian germ cell tumor.

A 17 year-old female with stage IIIc endodermal sinus tumor of the ovary developed transient cortical blindness and severe hypertension after 5 cycles of PVB regimen consisting of cisplatin, vinblastine and bleomycin. Clinical and radiological findings were compatible with Posterior LeukoEncephalopathy (PLE). Her visual acuity and blood pressure completely recovered within a few days after supportive treatment with antihypertensive drug. This condition is unpredictable but it can be reversible without long term sequelae. Most reports suggested that this rare toxicity was from cisplatin therapy. However, the exact pathophysiogenesis of this condition was not known precisely. Prompt reduction in blood pressure and withdrawal of immunosuppressive agents might lead to rapid reversal of this syndrome and prevent permanent brain damage.

Adolescent↗

DNA copy number changes in malignant ovarian germ cell tumors.

Malignant ovarian germ cell tumors (OGCTs) include immature teratomas (ITs), dysgerminomas (DGs), endodermal sinus tumors (ESTs), choriocarcinomas, and embryonal carcinomas. Knowledge about the genetic changes associated with malignant OGCT development is sparse. We therefore analyzed 25 OGCTs (12 DGs, 4 ESTs, and 9 ITs) for gains and losses by comparative genomic hybridization. In total, more gains than losses were observed, and the number of alterations ranged from 0-20 per tumor. The average number of changes among DGs, ESTs, and ITs was 10, 6, and 1.4, respectively. The most common changes in DGs were gains from chromosome arms 1p (33%), 6p (33%), 12p (67%), 12q (75%), 15q (42%), 20q (50%), 21q (67%), and 22q (58%); gains of the whole of chromosomes 7 (42%), 8 (42%), 17 (42%), and 19 (50%); and losses from 13q (58%). Two of three DGs with a gonadoblastoma component showed gains of 3p21 and loss of 5p, whereas none of the nine pure DGs had these changes, suggesting that they might be characteristic either of gonadoblastoma or of DG developing from a gonadoblastoma. Gain of 12p and gain from 1q were seen in three of four ESTs, whereas gains from 3p, 11q, and Xp and loss from 18q were each found in two tumors. Five of the ITs revealed changes (range, 1-4 changes/tumor), with gains from 1p, 16p, 19, and 22q each being found in two tumors. We conclude that ovarian DGs and ESTs seem to develop via the same genetic pathways that are already known for testicular germ cell tumors. On the other hand, ITs do not exhibit gain of 12p and also typically show fewer changes than other malignant OGCTs, indicating that they arise via different pathogenetic mechanisms.

Adolescent↗

Pediatric ovarian tumors: a review of 67 cases.

BACKGROUND: Ovarian tumors are uncommon but important childhood neoplasms. PROCEDURE: We reviewed records of 67 pediatric patients presenting to three pediatric referral centers from 1980 to 2003. RESULTS: Thirty patients had benign tumors. Thirty-seven patients had malignant tumors: 11 immature teratomas, seven malignant mixed germ cell tumors, seven juvenile granulosa cell tumors, five dysgerminomas, two endodermal sinus tumors, two serous papillary cystadenocarcinomas, one small cell carcinoma, one anaplastic sex-cord tumor, and one undifferentiated sarcoma. More than half presented with abdominal pain. Forty-six percent had an abdominal mass at the time of presentation. Other signs and symptoms included poor appetite (15%), urinary symptoms/urinary infection (9%), menstrual changes (9%), and weight loss (6%). Precocious puberty was noted in seven patients. Torsion was seen more often in patients with benign tumors (23 vs. 8%); two patients had both torsion and acute appendicitis. The neoplasm was an incidental finding in 12 patients. CONCLUSIONS: Fifty-five percent of the 67 ovarian tumors presenting to our centers were malignant. Pain was the most common symptom, although presence of an abdominal mass was frequent, and other symptoms non-specific. Almost all neoplasms presented as unilateral masses and rarely were metastatic at diagnosis. Ovarian tumors must be considered in the differential diagnosis of young girls with abdominal pain, mass, or other non-specific symptoms.

Adolescent↗

Improved prognosis of intracranial non-germinoma germ cell tumors with multimodality therapy.

The 5 year survival for patients with malignant intracranial non-germinoma germ cell tumors (NGGCT) which include endodermal sinus tumors, embryonal carcinomas, choriocarcinomas and immature teratomas is less than 25% following a small resection and radiotherapy. In an effort to improve the survival of these patients, an approach using an attempt at radical resection where feasible followed by multi-modality 'sandwich' therapy (chemotherapy-radiation-chemotherapy) was used to treat 18 newly diagnosed patients between 1986 and 1994 in a multi-institution study. Fourteen patients had histologically proven NGGCT and four were presumed NGGCT because of markedly elevated concentrations of serum and/or CSF alpha fetoprotoin (AFP) and/or beta human chorionic gonadatrophin (b-HCG). The primary tumor was confined to the pineal region in 12 patients, the suprasellar region in five, and a cerebral hemisphere in one. None of the patients had central nervous system metastases at diagnosis by MRI imaging of the spine and CSF cytology. Radical surgical resection was performed initially in 11 patients (gross total -6, subtotal -5): four had a biopsy and three had no surgery. All patients then received 3 or 4 cycles of neoadjuvant chemotherapy with cisplatin (100 mg/m2/cycle) and VP-16 (500 mg/m2/cycle). Of the 12 patients with evaluable disease there were 9 responses to the neoadjuvant chemotherapy (5 CR, 4 PR); 2 patients had stable disease and I progressed during chemotherapy. Six patients with no evaluable disease after a gross total resection had a continuous complete response. Seventeen patients received radiation therapy (involved field -11, involved field + craniospinal -4, involved field + whole brain -2). Twelve patients received 4 cycles post-radiation chemotherapy with vinblastine (6.5 mg/m2/cycle). bleomycin (15 U/m2/cycle), VP-16 (300 mg/m2/cycle, carboplatin (450 mg/m2/cycle). A total of four patients have died (3-progressive/recurrent disease, 1-metabolic). Four year actuarial event-free and total survival rates are 67% and 74%. This multi-modality adjuvant therapy approach appears to dramatically improve the outcome of malignant intracranial NGGCT.

Adolescent↗

Testicular germ cell tumors in childhood: treatment results of 52 patients.

We analysed the treatment results of 52 children with testicular germ cell tumors. Histopathological diagnoses were endodermal sinus tumor (63.4%), embryonal carcinoma (28.8%), teratocarcinoma (5.7%), and mixed tumors (2.1%). Radical inguinal orchiectomy was performed in 42 patients and retroperitoneal lymph node dissection in 10 (3/10 positive). Overall survival rates were: whole group: 71.2%; stage I: 89.7%; II: 68.5%; III: 31.2%; IV: 30% (p =.001). Five-year overall survival rates were 85.8% and 100% for stage I patients who received chemotherapy or not (p =.27); BEP regimen: 85.7%; classical VAC: 67.9%; vinblastine + bleomycin: 63.6%. Chemotherapy is not required in stage I. BEP regimen is effective in testicular germ cell tumors.

Adolescent↗

Gonadal endodermal sinus (yolk sac) tumor with pure intestinal differentiation: a new histologic type.

We studied 3 cases of a variant of endodermal sinus tumor (EST) or yolk sac tumor (YST). Two tumors originated in the ovary and one in the testis. The patients had very high levels of serum alpha-fetoprotein (AFP). All three tumors had a characteristic histologic appearance and were composed of acinar structures lined by columnar epithelium with large, immature nuclei. Immunohistochemistry and electron microscopy, including freeze-fracture studies, confirmed that this unusual tumor is one with exclusive intestinal differentiation. We feel that this unique tumor is a pure EST (YST) with exclusive intestinal differentiation.

Adult↗

Estrogen and progesterone receptors in endodermal sinus (yolk sac) tumor. Evaluation of immunocytochemical and biochemical methods.

Frozen tissue from four ovarian and two testicular endodermal sinus (yolk sac) tumors was studied with both biochemical and immunocytochemical methods to detect the presence of estrogen receptors (ERs) and progesterone receptors (PRs). Using the biochemical assay, one of the ovarian tumors was shown to contain low levels of ERs, whereas in the remaining five tumors no ERs were detected, and PRs were undetectable in all six tumors. In the frozen sections immunostained for ERs and PRs, no specific nuclear staining was detected in any of the six tumors. Since the immunocytochemical method provides a more tissue-specific evaluation of hormone receptors, the low level of ERs detected in one tumor using the biochemical assay is attributed to nonspecific binding. It appears that endodermal sinus tumor expresses neither ERs nor PRs.

Female↗

Endodermal sinus (yolk sac) tumor of vagina and cervix in an infant.

A case of vaginal and cervical endodermal sinus (yolk sac) tumor in a 6-month-old female infant is reported. The patient presented with an intermittent bloody discharge on her diapers. Pelvic CT showed an irregular soft tissue density mass with heterogeneous enhancement within the vagina and extending to the cervix. The histopathologic features were identical to those of endodermal sinus tumors.

Female↗

Primary yolk sac tumor of the prostate in a patient with Klinefelter's syndrome.

Primary yolk sac (endodermal sinus) tumor of the prostate is extremely rare with only 2 cases reported in the literature. We describe a case of primary yolk sac tumor of the prostate in a man with Klinefelter's syndrome. Treatment included 4 courses of combination chemotherapy followed by retroperitoneal lymph node dissection, cystoprostatectomy and ileal conduit urinary diversion. We review the association of Klinefelter's syndrome with extragonadal germ cell tumor along with the management of this rare disease.

Adult↗