Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review of the histology, clinical findings and results of therapy in 9 females with endodermal sinus tumor (EST) is presented. Five patients had histologically pure EST; 4 had EST mixed with other germ cell components. The site of primary tumor was the ovary in 8 of the 9 females; the remaining patient with an extraovarian primary represents the first reported case of EST arising in the vulva. The addition of combination chemotherapy has prolonged survival over historical controls treated with surgery or surgery plus irradiation. Adjuvant chemotherapy appears warranted as treatment for occult metastatic disease; postoperative radiation therapy appears useful in providing local control of primary disease. There is a suggestion of increased sensitivity of EST to combination chemotherapy as compared to other germ cell histologies with which it is commonly admixed.
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PMID:Endodermal sinus tumor: the Stanford experience and the first reported case arising in the vulva. 63 17

This case report concerns an endodermal sinus tumor (EST) arising in the pineal region of a 16-year-old boy who died 3 months after radiation. He developed extensive abdominal metastases through a ventriculoperitoneal shunt, whereas the primary tumor and a suprasellar metastasis could be controlled by radiotherapy, demonstrated by autopsy. The histologic diagnosis was supported by an elevated level of alpha-fetoprotein in serum and the demonstration of this marker in the tumor tissue by immunoperoxidase method. The poor diagnosis of all previously reported cases with pineal EST requires a combined modality of surgical approach, radiotherapy, and concomitant chemotherapy.
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PMID:Endodermal sinus tumor of the pineal region. Metastases through a ventriculoperitoneal shunt. 674 18

Endodermal sinus tumor (EST) of the mediastinum is a rare germ-cell neoplasm affecting mainly young adult males. Ten patients with EST were treated with a multimodality approach that included surgery, chemotherapy, and radiotherapy. All patients had relapses after achieving a transient response except one who is still in complete remission more than five years following the diagnosis of the disease. Optimal therapy for this neoplasm has yet to be discovered. The disease is a subset of extragonadal germ cell tumors which appears to be lethal in most cases, particularly when the primary tumor is unresectable. The first case of five-year disease-free survival is described.
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PMID:Endodermal sinus tumor of the mediastinum. 709 10

Malignant germ cell tumors account for about 3% of neoplasms in children, and endodermal sinus tumor (EST) is the most common histological subtype. The authors reviewed 22 years' experience (at their institution) in the management of 37 patients with this tumor. Fifteen of them (41%) had a sacrococcygeal primary, 10 had a testicular tumor (27%), 6 had an ovarian tumor (16%), 3 had a vaginal tumor (8%) and 3 had tumors at other sites (8%). Seven (19%) patients presented with metastatic disease, primarily pulmonary. The serum alpha-fetoprotein (AFP) level was elevated in all cases tested. The initial chemotherapy regimen included vincristine, actinomycin, cyclophosphamide (VAC), and Adriamycin (6 patients), but since 1985 the regimen has been changed to include cisplatin or Carboplatin, etoposide, and bleomycin (21 patients). Eight patients with testicular tumors initially were treated with surgical excision alone. Computed tomography results were a poor predictor of recurrence, but AFP surveillance was extremely sensitive. No second-look operation detected residual tumor in the absence of AFP elevation. Initial relapse for all patients tended to occur early (within 2 years), locally, and often with pulmonary metastases. Although historically the prognosis for patients with EST has been poor, the overall 2-year survival rate in this series was 70%. The best prognosis was among the children who had a testicular primary tumor (survival rate, 100%). The 2-year survival rate for patients with ovarian tumors was 67%; for those with sacrococcygeal primaries it was 60%. These results suggest that the prognosis for children with EST has improved significantly over the past decade. Contributing factors include therapy based on cisplatin, etoposide, and bleomycin, and relapse surveillance with serial AFP determinations. Second-look procedures should be reserved for patients who have an increasing level of serum AFP, suspicious computed tomography findings, and no obvious evidence of metastatic disease.
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PMID:Endodermal sinus tumor in children. 886 37