[Endodermal sinus tumors in teratomas of the thymus gland].
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Six children with yolk sac tumor, 3 occurring in gonadal sites and 3 in extragonadal ones, were studied with respect to pathological features, laboratory findings and clinical course. Their histological appearance was similar regardless of the site of origin. The prognosis was poor, but all the cases exhibited objective response to chemotherapy. Two children are alive and off therapy from 27 and 60 months. Three of the six patients in which serum alfa-fetoprotein determinations were performed showed positive results. The demonstration of alfa-fetoprotein in the serum of these patients could prove to be of prognostic value, indicating the presence of residual or recurrent disease. It is advisable to gather cases from many Institutions in order to specify prognostic factors, to know the natural history of this tumor and to prepare an effective therapeutic program.
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Endodermalsinus tumors are rare and belong to the group of malignant gem cell tumors. Histogenetically they are differently interpreted. Their unfavorable prognosis is to be attributed to existing uncertainties regarding therapeutical procedure and to the rapid tumor expansion. Our case of an advanced endodermalsinus tumor of the right ovary was operated on palliatively and then subjected to antineoplastic chemotherapy with adriamycine, bleomycine and vincristine, subsequently with actinomycine D and vinblastine. There was a complete remission of the tumor. The patient survived for 43 months free of tumor.
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The first reported case of successful pregnancy following unilateral adnexectomy and combination chemotherapy for nedodermal sinus tumor is presented. The rationale for conservative management of this virulent neoplasm is discussed.
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