Abstract

Primary mucinous ovarian cancer is a rare type of epithelial ovarian cancer. In this comprehensive review we discuss management recommendations for the treatment of mucinous ovarian cancer. Although most tumors are stage I at diagnosis, 15–20% are advanced stage at diagnosis. Traditionally, patients with primary mucinous ovarian cancer have been treated similarly to those with the more common serous ovarian cancer. However, recent studies have shown that mucinous ovarian cancer is very different from other types of epithelial ovarian cancer. Primary mucinous ovarian cancer is less likely to spread to lymph nodes or the upper abdomen and more likely to affect younger women, who may desire fertility-sparing therapies. Surgical management of mucinous ovarian cancer mirrors surgical management of other types of epithelial ovarian cancer and includes a bilateral salpingo-oophorectomy and total hysterectomy. When staging is indicated, it should include pelvic washing, omentectomy, and peritoneal biopsies; lymph node evaluation should be considered in patients with infiltrative tumors. The appendix should be routinely evaluated intra-operatively, but an appendectomy may be omitted if the appendix appears grossly normal. Fertility preservation can be considered in patients with gross disease confined to one ovary and a normal-appearing contralateral ovary. Patients with recurrent platinum-sensitive disease whose disease distribution suggests a high likelihood of complete gross resection may be candidates for secondary debulking. Primary mucinous ovarian cancer seems to be resistant to standard platinum-and-taxane regimens used frequently for other types of ovarian cancer. Gastrointestinal cancer regimens are another option; these include 5-fluorouracil and oxaliplatin, or capecitabine and oxaliplatin. Data on heated intra-peritoneal chemotherapy (HIPEC) for mucinous ovarian cancer are scarce, but HIPEC may be worth considering. For patients with recurrence or progression on first-line chemotherapy, we advocate enrollment in a clinical trial if one is available. For this reason, it may be beneficial to perform molecular testing in all patients with recurrent or progressive mucinous ovarian cancer.