Ovarian Yolk Sac Tumor in a Postmenopausal Patient with Malignant Müllerian Mixed Tumor: A Case Report and Systematic Review of the Literature

Abstract Background Yolk sac tumor (YST) is a most common form of malignant ovarian germ cell tumors and typically occur in young women and adolescent. Ovarian YST occurred in postmenopausal women is an extremely rare entity which has an aggressive behavior and worse outcome. The aim of this article was to provide better understanding of clinicopathological characteristics, management, and prognosis of postmenopausal YSTs.Case presentation A 64-year-old woman was admitted to our hepatobiliary surgery department due to a cystic and solid round mass in the lower right abdomen in December 2013. laboratory tests showed a-fetoprotein (AFP), carcinoembryonic antigen (CEA), and cancer antigen 19-9 (CA19-9) to be within normal limits, but serum cancer antigen (CA-125) was elevated. Exploratory laparotomy was performed including tumorectomy, right oophorectomy, partial omentectomy. Postoperatively, pathologic findings showed that the morphologic features and immunohistochemical results were consistent with ovarian YST with malignant Müllerian mixed tumor (MMMT). The patient was admitted again for recurrence of the tumor and metastasis to bilateral supraclavicular lymph nodes in August 2018. The patient underwent total abdominal hysterectomy (TAH), left salpingo-oophorectomy, right salpingectomy, pelvic lymphadenectomy, and partial peritonectomy. The patient received 6 cycles of adjuvant chemotherapy with BEP (bleomycin, etoposide, and cisplatin) postoperatively, and died of disease in August 2019. Conclusion Here, we presented a case of postmenopausal YST with MMMT. Meanwhile, a systematic literature review was conducted in the PubMed database, including case reports and case series. We summarized the clinical characteristics, treatments, and prognosis of all cases. Overall survival was calculated with Kaplan-Meier curves. Univariate analysis was performed with the log-rank test. A Cox model was constructed to determine independent predictors of mortality. As the morphological and immunophenotypical features of postmenopausal YSTs were always overlapped with somatic epithelial carcinomas, pathologists should have a high index of suspicion to diagnose ovarian YSTs in older women. Optimum chemotherapy is essential in improving prognosis of postmenopausal YSTs.