Aims: Yolk sac tumours of the ovary (YST), also called endodermal sinus tumours (EST) are rare and highly malignant tumours of utmost importance occurring in children and young adults. In the past, outcome was very poor and the disease was almost always fatal. With the refinement of chemotherapeutic regimens in the last several decades, survival rates have improved dramatically and fertility preserving surgery has become possible. The aim of this review is to provide the reader with an analysis of the available literature and a rational approach to patient management.
Methods: We performed a literature search in the PubMed database and the reference lists of relevant articles concerning yolk sac tumours of the ovary.
Findings and conclusion: There are no randomised studies relating to the management of YST of the ovary. The available literature is composed of retrospective reviews and case reports that span several decades. Prognosis nowadays is good in stage I and II but still comparable to that of ovarian epithelial cancer in stage III and IV. The overall good prognosis is due to the fact that most of ovarian YST are diagnosed at an early stage where 5years survival reaches 95%. Appropriate surgical treatment for patients where fertility needs to be preserved consists in laparotomy with unilateral salpingo-oophorectomy, peritoneal cytologic studies, omentectomy, multiple peritoneal and abdominal biopsies and resection of all visible disease. Three courses of BEP (bleomycin, etoposide, cisplatin) is the current standard therapy and four courses is recommended in case of bulky residual disease after surgery. Serum alpha-feto-protein (AFP) is a useful marker for the diagnosis and management of YST.