PRIMARY ENDODERMAL SINUS TUMOR OF THE VULVA - A CASE-REPORT AND REVIEW OF THE LITERATURE

Background. Extragonadal endodermal sinus tumors arising in the extern al genitalia represent an exceedingly rare malignancy in women. Six ca ses of endodermal sinus tumors of the vulva have been reported to date , with three cases failing to respond to conservative surgery and vinc ristine-based chemotherapy. We report a seventh case of vulvar endoder mal sinus tumor that was treated with radical surgery and platinum-bas ed chemotherapy. Case. RT is an 18-year-old female who presented with a vulvar mass that was diagnosed as endodermal sinus tumor at the time of biopsy. She was subsequently treated with modified radical vulvect omy and ipsilateral groin lymphadenectomy, followed by bleomycin, etop oside, and cisplatin chemotherapeutic regimen. She has since remained free of disease for 18 months as evidenced by serum alpha-fetoprotein and physical exam at 18 months. Conclusions. Vulvar endodermal sinus t umors represent a very small number of germ cell tumors in women. Base d on the previous accounts, this disease appears to be more fatal than endodermal sinus tumor arising at other sites. These tumors also have a predilection for local metastasis. Due to the previous accounts, we chose to treat this patient with radical surgery and platinum-based c hemotherapy. This treatment regimen has resulted in a disease-free sta te for 18 months. (C) 1997 Academic Press.