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Primary pineal endodermal sinus tumor with elevated serum and CSF alphafetoprotein levels. Case report.

A 12-year-old girl with an alphafetoprotein-producing brain tumor in the pineal region is presented. The marker was detected in both serum and cerebrospinal fluid. The amount of the marker decreased after irradiation of the tumor and systemic chemotherapy and increased prior to tumor recurrence. At autopsy, the tumor was histopathologically diagnosed as an endodermal sinus tumor, and alphafetoprotein was demonstrated in the tumor cells by the immunofluorescence method. Alphafetoprotein may represent a specific and sensitive marker for predicting tumor histopathology and for monitoring the therapeutic response in patients with primary intracranial germ-cell tumors.

Brain Neoplasms↗

Endodermal sinus tumor of the ovary: the Hacettepe University experience.

OBJECTIVE: The purpose of this study was to evaluate the treatment regimens used for patients with endodermal sinus tumors (EST), and also to examine the prognostic value of surgicopathological variables. STUDY DESIGN: Twenty-two patients treated for pure EST, and seven patients who had mixed germ cell tumors with EST components were included. Initial surgery consisting of surgical staging to achieve optimal debulking was the principal mode of therapy. Data were obtained from patients' files, a special gynecologic oncology database, and pathology records. RESULTS: The median age at the time of diagnosis was 18 (range 8-45). Sixteen patients had stage I (55%), and 13 (45%) had stage II-IV disease. As an adjuvant therapy 18 patients (62%) received platin-based chemotherapy, three patients (10%) had non-platin-based chemotherapy, four patients (14%) had radiotherapy, and four patients (14%) had combined radiotherapy plus non-platin-based chemotherapy. The 5-year disease-free and overall survival rates were 47 and 48%, respectively. Survival rates were dismal in patients with stage II-IV disease (P < 0.001). Platin-based chemotherapy achieved significant survival benefit (P = 0.03 and P < 0.001, respectively). Fertility-saving surgery had an overall survival no worse than those who underwent more extensive surgery. There was no significant survival difference with respect to age, histology, and tumor size. CONCLUSION: Fertility-sparing surgery with a postoperative platin-based combination chemotherapy should be the selected mode of treatment.

Adolescent↗

Primary endodermal sinus tumor of the endometrium. A clinicopathologic, immunocytochemical, and ultrastructural study.

We report a case of primary endodermal sinus tumor (EST) of the endometrium in a 42-year-old female. Although numerous extragonadal EST have been reported, primary EST of the endometrium is exceedingly rare. To our knowledge this is the fourth documented case of this nature. The tumor had the typical microscopic features of EST, with papillary, tubular, reticular, and solid growth patterns; occasional Schiller-Duval bodies and many intracellular and extracellular periodic-acid Schiff positive hyaline globules were seen. The neoplastic cells stained positively for alpha-fetoprotein (AFP), alpha-1-antitrypsin (A1AT), cytokeratin, and placental alkaline phosphatase. The globules were positive for AFP, A1AT, albumin, transferrin, and fibronectin. The tumor cells were negative for type IV collagen and the beta subunit of human chorionic gonadotropin (B hcG). Electron microscopic examination showed intracellular and extracellular basement membrane-like material, intracytoplasmic lumina with microvilli, and glycogen. The patient was treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy, followed by four cycles of adjunct chemotherapy (vinblastine, bleomycin, and cisplatinum) repeated every 3 weeks. The serum AFP level was elevated significantly before the surgery and the tumor response was monitored by serial determination of serum AFP level. There was no evidence of recurrence 24 months after surgery.

Adult↗

An endodermal sinus tumor of the head and neck region.

A 2-year-old patient with a recurrent endodermal sinus tumor of the nasopharynx is presented. After the first attempt to surgically excise the tumor it recurred, causing upper airway obstruction within 2 months. Combined treatment with surgery, radiotherapy and chemotherapy resulted in a disease-free survival of four years.

Child, Preschool↗

Recurrent endodermal sinus tumor during pregnancy.

A case is reported of a patient who was found to have an endodermal sinus tumor coincident with pregnancy. She maintained a complete clinical response for 12 months after conservative surgery, uterine aspiration, and multiple-agent chemotherapy. Tumor recurrence appeared with a second pregnancy. A healthy infant was delivered despite resumption of chemotherapy. The use of cytotoxic chemotherapy during pregnancy is reviewed.

Adolescent↗

[Endodermal sinus tumor (yolk sac tumor)--report of 52 cases and review of the literature of 1224 cases].

52 cases of endodermal sinus tumors (EST) are reported and 1224 cases from the Chinese and foreign literature are reviewed. This tumor, occurring usually in the gonads and rarely at extragonadal sites, comprised 10-17.1% of the former. It is located on or along the mid-line of the body including the orbit and other organs. In this paper, there are two cases with the tumor located at the orifice of urethra and one case between the pubic bone and the urinary bladder which has never been reported. EST often occurs in people under the age of 35. EST in the gonadal sites is divided into infant-childhood and adult groups. In the former group the prognosis is better than that of the adult group when the tumor is located in the testis as well as those in the vagina. There is sex and age difference for some EST in extragonadal sites. Those in the mediastinum almost always occur in young males. Diagnosis is easily made by pathology and by serum alpha-fetal protein (SAFP). Pathologically it is divided into two types: simple EST and EST element mixed with other germ cell tumors which give the same prognosis. The authors suggest that SAFP be done for any patients with teratomata in order to avoid misdiagnosis. SAFP is not only diagnostic but also prognostic by monitering postoperative course. EST is a highly malignant tumor, rapidly growing and poor in prognosis. Recent reports show that operated patients supplemented by chemotherapy give much better results than by radiotherapy.

Adolescent↗

Endodermal sinus tumor of the vagina: a report of nine cases and review of the literature.

Nine cases of endodermal sinus tumor of the vagina from children 6 months to 2 years of age are reported. The two patients treated before the advent of modern chemotherapy died 6 to 13 months postoperatively despite attempted complete surgical resection of the tumor in one of the cases. One patient who was treated initially by hysterectomy and vaginectomy had lung metastases 6 months postoperatively and died despite chemotherapy. Six patients who were treated by a surgical procedure and received vincristine, actinomycin D, and cyclophosphamide, with radiation therapy in two cases, were alive and free of disease from 2 to 9 years postoperatively.

Age Factors↗

Endodermal sinus tumors of the ovary in children. The role of radiation therapy in relation to the clinical course.

Endodermal sinus tumor of the ovary in children has been characterized by its general resistance to therapy and its almost uniform fatality. The multidisciplinary treatment of 5 such cases is described. The author's experience suggests that these tumors are responsive to irradiation. The rationale for a combined approach is discussed, and radiation treatment volumes and doses are suggested.

Adolescent↗

[Primary intracranial endodermal sinus tumor with a skull base extension--a case report].

The authors described a case of primary intracranial endodermal sinus tumor (EST), and presented a review of 24 reported cases. From the middle of December 1981, this 15-year-old boy experienced progressive diplopia. At the other hospital, partial removal of the intrasellar tumor was performed by a left frontotemporal craniotomy approximately 2 months after the onset of symptoms. The histological diagnosis was suspected to be a pituitary adenoma, and thereafter, 60Co irradiation was carried out for about a month. On June 5th. 1982, the patient was admitted to the Kochi Medical School Hospital, because of increasing headache with diplopia, hoarseness, dysphagia and limitation of tongue movement. On admission, he had a small stature for his age with moderate diabetes insipidus. Neurological examination showed left abducens, glossopharyngeal and vagal nerve palsies in addition to bitemporal hemianopsia. CT scans revealed a heterogenously enhanced high density mass lesion behind the dorsum sellae with an extension from the upper clivus to the sphenoid sinus. Cerebral angiograms showed posterior displacement of the basilar artery. In the endocrinological examination, panhypopituitarism was recognized with a high level of serum alfa-fetoprotein (AFP). While, serum carcinoembryonic antigen (CEA) and human chorionic gonadotropin (HCG) were in normal range. On July 1st. 1982, a left suboccipital craniectomy was performed. The tumor existed mainly in the extradural space along the clivus, and only a small intradural portion of the tumor was excited.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Fine needle aspiration biopsy findings in endodermal sinus tumors. A report of four cases with cytologic, immunocytochemical and ultrastructural findings.

We present four cases of endodermal sinus tumor affecting children (median age, 20 months). Three tumors were located in the sacrococcygeal region and one in the right testicle. All cases were diagnosed by fine needle aspiration biopsy. The most characteristic features were cells arranged in a papillary-like configuration, vacuolated cytoplasm and intracellular and extracellular deposits of pink, homogeneous material. Immunocytochemical study showed alpha-fetoprotein-positive cells. Ultrastructural study showed intracytoplasmic inclusions of electron-dense material and basement membrane-like material in the intercellular spaces.

Antineoplastic Combined Chemotherapy Protocols↗

Endodermal sinus tumor of the ovary: a clinical and pathologic analysis of 71 cases.

The clinical and pathologic features of 71 endodermal sinus tumors of the ovary were studied in an effort to delineate the histogenesis and biologic behavior of this neoplasm and to evaluate the efficacy of different forms of treatment. Alpha-fetoprotein (AFP) was identified in hyaline droplets, cell cytoplasm, and intercellular spaces of all 15 tumors examined by an immunoperoxidase technique; this supports the view that the neoplasm simulates yolk sac endoderm. There were only nine survivors among 65 patients on whom follow-up information was available; the actuarial survival was 13% at 3 years. Of the neoplasms that recurred, 93% did so within 1 year, and of those patients who died, 93% did so within 2 years. The size and stage of the tumor had prognostic significance, but the patient's age, the mitotic activity, and histologic pattern did not. Although 71% of the patients had Stage I tumors at the time of diagnosis, subclinical metastasis was present in 84% of Stage I patients. Triple chemotherapy (vincristine, actinomycin D, and cyclophosphamide (VAC)) employed after unilateral salpingo-oophorectomy in four patients with Stage I tumors resultivors among 12 Stage I patients treated with combined surgery and radiation. The finding of AFP in all tumors in which this was evaluated suggests that serum radioimmunoassay might be useful to monitor response to therapy.

Adolescent↗

Endodermal sinus tumor originating in the region of the pineal gland: ultrastructural and immunohistochemical study.

This is a case report of an endodermal sinus tumor (EST) arising in the pineal region of a 19-year-old girl (first case studied by electron microscopy). The ultrastructure of the tumor was similar to that of the previously reported cases of EST of ovaries, mediastinum, and sacrococcygeal area. A characteristic basement membrane-like material has been identified within endoplasmic reticulum of tumor cells and in intercellular spaces. Alphafetoprotein has been detected in the spinal fluid of the patient by radioimmunoassays and within tumor tissue by immunohistochemical techniques. Combination therapy consisting of radiation, surgical decompression, chemotherapy (BCNU), and prednisone has been employed in the treatment of this patient without significant benefit. The patient expired three months after the surgery, no autopsy was obtained.

Adolescent↗

Endodermal sinus tumor of the ovary during pregnancy: a case report.

Serum alpha-fetoprotein screening led to the detection of an endodermal sinus tumor of the ovary in a 24-year-old female in week 17 of pregnancy. After surgery, chemotherapy was postponed. In week 28 levels of serum alpha-fetoprotein increased, but delivery was delayed until 33 weeks' gestation. After delivery, the patient received four chemotherapy courses (cisplatin, etoposide, and bleomycin). Mother (24 months after last chemotherapy) and child are doing well.

Adult↗

Cisplatin, vinblastine, and bleomycin combination chemotherapy in endodermal sinus tumor of the ovary.

The combination of cisplatin, vinblastine, and bleomycin was administered to 13 previously untreated patients with pure endodermal sinus tumor of the ovary. Twelve had unilateral salpingo-oophorectomy as initial surgery. Among 11 women whose alpha-fetoprotein (AFP) levels returned to normal after cisplatin, vinblastine, and bleomycin therapy, nine are alive with no evidence of disease after periods of 20 months to six years and two months. In two patients, AFP levels returned to normal only after the introduction of different combination chemotherapies. Hematologic toxicity was moderate. Pulmonary toxicity due to bleomycin occurred in two cases. All menstruating patients resumed normal menstrual function after the interruption of chemotherapy. Two patients delivered normal term infants.

Adolescent↗

An endodermal sinus tumor diagnosed in pregnancy: case report and review of the literature.

OBJECTIVE: The use of chemotherapeutic drugs in pregnancy is a rare occurrence. Experience and results need to be shared. METHOD: At 23 weeks gestational age, a patient was diagnosed with an endodermal sinus tumor of the ovary. She received one course of postoperative bleomycin, cisplatin, and etoposide. One week later, the patient was investigated for increasing abdominal pain and the fetus was noted to have ventriculomegaly. RESULTS: The baby was delivered, and the patient was surgically staged and completed chemotherapy. Although the patient is alive and well 16 months after delivery, the infant has developed significant ventriculomegaly with cerebral atrophy. CONCLUSION: The etiology of this infant's significant cerebral atrophy is not clear. Tumor-specific, perioperative, or drug-related events must be considered.

Adult↗

Endodermal sinus tumor arising from a dysgenetic gonad in a 46,XX female combined with müllerian agenesis.

The occurrence of neoplastic transformation in a dysgenetic gonad of a female with 46,XX karyotype is very rare. Moreover, to our knowledge, a case of endodermal sinus tumor arising from that setting combined with Müllerian agenesis has not been reported. This case suggests the possibility of development of cancer in a dysgenetic ovary of a female even with 46,XX or Müllerian agenesis.

Adult↗

[Cisplatin, vinblastine, and bleomycin combination chemotherapy in a stage IV pure endodermal sinus tumor of the ovary--a case report of a long-term survival].

Discussed is a case of a stage IV pure endodermal sinus tumor (EST) of the ovary that was treated with a PVB protocol following cytoreductive surgery. The serum alpha-fetoprotein (AFP) dosage before treatment was 83,546 ng/ml, and a complete clinical response was achieved with the disappearance of pleural effusions, ascites, and normalization of the serum AFP. A second-look laparotomy was substituted by serial AFP determinations and the patient remained free of disease for two years. Recently, complete remissions and long term survivals of patients with a stage I-II disease have been reported, though a prolonged or a complete remission of a stage IV EST of the ovary is still a rarity.

Adolescent↗