899 EXTRAGONADAL ENDODERMAL SINUS TUMOR IN EARLY CHILDHOOD: TREATMENT RESPONSE AND LONGTERM EFFECT

Abstract

Endodermal sinus tumor is the most common form of germ cell malignancy found in the infantile testis. Histologically identical tumor may originate in extragonadal sites in young children, predominately in sacrococcygeal teratomas or presacral space. Extragonadal endodermal sinus tumors in young children appear to respond to cisplatinum (DDP) combinations, unlike the poor response reported in extragonadal, mainly mediastinal and retro-peritoneal, germ cell tumors in the adolescent or adult. Treatment methods and results in six children with endodermal sinus tumor occurring in sacral sites are the subject of this report. All presented to Children's Hospital of Michigan (1975-82) between ages 12-31 months(median 20 mo) with unresectable or disseminated disease. Two of six underwent resection of a benign sacrococcygeal teratoma within the first month of life and returned with metastatic disease to lungs and retroperitoneal node or liver. Three children presented with previously undetected sacral mass, one with bone and lung metastases. Approach to the two without metastatic disease included vincristine-cyclophosphamide-dactinomycin; one survives disease free at nine years. The sixth child, the only male, presented with urinary retention secondary to presacral primary and multiple lung nodules; he is disease free at 32 months following four courses of DDP-velbanbleomycin. The remaining living child has had a 36 mo course of recurring disease responsive to four different DDP combinations.