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Angela Jain, Paula D. Ryan and Michael V. Seiden

Approximately 22,000 cases of ovarian cancer occur each year in the United States, and likely fewer than 2000 cases of mucinous ovarian cancers. Although 90% of patients with mucinous ovarian cancer present with stage I disease and have curative surgeries, advanced-stage disease is known to have a poor response to standard platinum- and taxane-based chemotherapy. Despite limited enthusiasm, standard chemotherapy is still recommended for most patients with advanced-stage mucinous malignancies of the ovary. This report presents an unusual case of a woman with HER2-positive metastatic mucinous carcinoma of the ovary treated with chemotherapy regimens typically used for colorectal malignancies, followed by epidermal growth factor receptor–targeted therapies.

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NCCN Guidelines Insights: Ovarian Cancer, Version 1.2019

Featured Updates to the NCCN Guidelines

Deborah K. Armstrong, Ronald D. Alvarez, Jamie N. Bakkum-Gamez, Lisa Barroilhet, Kian Behbakht, Andrew Berchuck, Jonathan S. Berek, Lee-may Chen, Mihaela Cristea, Marie DeRosa, Adam C. ElNaggar, David M. Gershenson, Heidi J. Gray, Ardeshir Hakam, Angela Jain, Carolyn Johnston, Charles A. Leath III, Joyce Liu, Haider Mahdi, Daniela Matei, Michael McHale, Karen McLean, David M. O’Malley, Richard T. Penson, Sanja Percac-Lima, Elena Ratner, Steven W. Remmenga, Paul Sabbatini, Theresa L. Werner, Emese Zsiros, Jennifer L. Burns and Anita M. Engh

Epithelial ovarian cancer is the leading cause of death from gynecologic cancer in the United States, with less than half of patients living >5 years from diagnosis. A major challenge in treating ovarian cancer is that most patients have advanced disease at initial diagnosis. The best outcomes are observed in patients whose primary treatment includes complete resection of all visible disease plus combination platinum-based chemotherapy. Research efforts are focused on primary neoadjuvant treatments that may improve resectability, as well as systemic therapies providing improved long-term survival. These NCCN Guidelines Insights focus on recent updates to neoadjuvant chemotherapy recommendations, including the addition of hyperthermic intraperitoneal chemotherapy, and the role of PARP inhibitors and bevacizumab as maintenance therapy options in select patients who have completed primary chemotherapy.