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Lauren E. Kernochan and Rochelle L. Garcia

Edited by Kerrin G. Robinson

that most but not all carcinosarcomas of the uterus are combination tumors . Cancer Res 1997 ; 57 : 5379 – 5385 . 3 Abeln EC Smit VT Wessels JW . Molecular genetic evidence for the conversion hypothesis of the origin of malignant mixed

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Robert J. Morgan Jr, Deborah K. Armstrong, Ronald D. Alvarez, Jamie N. Bakkum-Gamez, Kian Behbakht, Lee-may Chen, Larry Copeland, Marta Ann Crispens, Maria DeRosa, Oliver Dorigo, David M. Gershenson, Heidi J. Gray, Ardeshir Hakam, Laura J. Havrilesky, Carolyn Johnston, Shashikant Lele, Lainie Martin, Ursula A. Matulonis, David M. O'Malley, Richard T. Penson, Sanja Percac-Lima, Mario Pineda, Steven C. Plaxe, Matthew A. Powell, Elena Ratner, Steven W. Remmenga, Peter G. Rose, Paul Sabbatini, Joseph T. Santoso, Theresa L. Werner, Jennifer Burns, and Miranda Hughes

subtypes may occur. The less common ovarian histopathologies (LCOHs) include carcinosarcomas (malignant mixed Müllerian tumors [MMMTs] of the ovary), clear cell carcinomas, mucinous carcinomas, grade 1 (low-grade) serous carcinomas/endometrioid epithelial

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Deborah K. Armstrong, Ronald D. Alvarez, Jamie N. Bakkum-Gamez, Lisa Barroilhet, Kian Behbakht, Andrew Berchuck, Lee-may Chen, Mihaela Cristea, Maria DeRosa, Eric L. Eisenhauer, David M. Gershenson, Heidi J. Gray, Rachel Grisham, Ardeshir Hakam, Angela Jain, Amer Karam, Gottfried E. Konecny, Charles A. Leath III, Joyce Liu, Haider Mahdi, Lainie Martin, Daniela Matei, Michael McHale, Karen McLean, David S. Miller, David M. O’Malley, Sanja Percac-Lima, Elena Ratner, Steven W. Remmenga, Roberto Vargas, Theresa L. Werner, Emese Zsiros, Jennifer L. Burns, and Anita M. Engh

whether patients with LCOCs will benefit from IP/intravenous chemotherapy. In the NCCN Guidelines, the clear cell carcinoma and carcinosarcoma are the only LCOCs for which IP/intravenous chemotherapy is a recommended option, as these cancer types are

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Michelle C. Nguyen, Manisha H. Shah, David A. Liebner, Floor J. Backes, John Phay, and Lawrence A. Shirley

-oophorectomy and pelvic and periaortic lymph node dissection for stage III uterine carcinosarcoma in March 2016. She completed 6 cycles of carboplatin and paclitaxel therapy and 4,860 cGy of RT to the pelvic and aortic nodes. She later developed subcutaneous

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Robert J. Morgan Jr., Ronald D. Alvarez, Deborah K. Armstrong, Barry Boston, Robert A. Burger, Lee-may Chen, Larry Copeland, Marta Ann Crispens, David Gershenson, Heidi J. Gray, Perry W. Grigsby, Ardeshir Hakam, Laura J. Havrilesky, Carolyn Johnston, Shashikant Lele, Ursula A. Matulonis, David M. O'Malley, Richard T. Penson, Steven W. Remmenga, Paul Sabbatini, Russell J. Schilder, Julian C. Schink, Nelson Teng, and Theresa L. Werner

, including malignant germ cell neoplasms, carcinosarcomas (malignant mixed Müllerian tumors of the ovary [MMMT]), and sex cord-stromal tumors. The guidelines also discuss fallopian tube and primary peritoneal cancers, which are less-common neoplasms that are

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The Alvin J. Siteman Cancer Center

Epithelial ovarian cancer is the leading cause of death from gynecologic cancer and the fifth most common cause of cancer mortality in women in the United States. Fewer than 40% of women with ovarian cancer are cured, and 70% of patients present with advanced disease; because of the location of the ovaries, ovarian cancer has been difficult to diagnose at earlier stages. Epidemiologic studies have identified risk factors, including family history. The NCCN guidelines discuss epithelial ovarian cancer as well as less common ovarian histopathologies, including germ cell neoplasms, carcinosarcomas (malignant mixed Müllerian tumors of the ovary), and ovarian stromal tumors. For 2008, updates include the addition of platinum-based combination therapy as a possible treatment modality for recurrence and a listing of preferred agents for acceptable recurrence modalities. New information was also added to the section on clinical presentation.

For the most recent version of the guidelines, please visit NCCN.org

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Benjamin E. Greer, Wui-Jin Koh, Nadeem Abu-Rustum, Michael A. Bookman, Robert E. Bristow, Susana M. Campos, Kathleen R. Cho, Larry Copeland, Marta Ann Crispens, Patricia J. Eifel, Warner K. Huh, Wainwright Jaggernauth, Daniel S. Kapp, John J. Kavanagh, John R. Lurain III, Mark Morgan, Robert J. Morgan Jr, C. Bethan Powell, Steven W. Remmenga, R. Kevin Reynolds, Angeles Alvarez Secord, William Small Jr, and Nelson Teng

Kempson RL . Uterine papillary serous carcinoma revisited . Gynecol Oncol 1994 ; 54 : 261 – 263 . 90 McCluggage WG . Uterine carcinosarcomas (malignant mixed Mullerian tumors) are metaplastic carcinomas . Int J Gynecol Cancer 2002 ; 12

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Wui-Jin Koh, Benjamin E. Greer, Nadeem R. Abu-Rustum, Sachin M. Apte, Susana M. Campos, John Chan, Kathleen R. Cho, David Cohn, Marta Ann Crispens, Nefertiti DuPont, Patricia J. Eifel, Amanda Nickles Fader, Christine M. Fisher, David K. Gaffney, Suzanne George, Ernest Han, Warner K. Huh, John R. Lurain III, Lainie Martin, David Mutch, Steven W. Remmenga, R. Kevin Reynolds, William Small Jr, Nelson Teng, Todd Tillmanns, Fidel A. Valea, Nicole McMillian, and Miranda Hughes

epithelial tumor (ie, pure endometrioid cancer, uterine serous adenocarcinoma, clear cell adenocarcinoma, or carcinosarcoma, which is also known as malignant mixed Müllerian tumor [MMMT]); or 2) a stromal/mesenchymal tumor (ie, uterine leiomyosarcoma [uLMS

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Rajmohan Murali, Deborah F. Delair, Sarah M. Bean, Nadeem R. Abu-Rustum, and Robert A. Soslow

similar genomic classes, which were also associated with prognosis. 40 Uterine carcinosarcomas also frequently harbor mutations in TP53, PTEN, PIK3CA, PPP2R1A, FBXW7 , and KRAS , similar to endometrioid and serous carcinomas. 41 It is also apparent

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NCCN Guidelines® Insights: Uterine Neoplasms, Version 3.2021

Featured Updates to the NCCN Guidelines

Nadeem R. Abu-Rustum, Catheryn M. Yashar, Kristin Bradley, Susana M. Campos, Junzo Chino, Hye Sook Chon, Christina Chu, David Cohn, Marta Ann Crispens, Shari Damast, Elisabeth Diver, Christine M. Fisher, Peter Frederick, David K. Gaffney, Suzanne George, Robert Giuntoli II, Ernest Han, Brooke Howitt, Warner K. Huh, Jayanthi Lea, Andrea Mariani, David Mutch, Larissa Nekhlyudov, Mirna Podoll, Steven W. Remmenga, R. Kevin Reynolds, Ritu Salani, Rachel Sisodia, Pamela Soliman, Edward Tanner, Stefanie Ueda, Renata Urban, Stephanie L. Wethington, Emily Wyse, Kristine Zanotti, Nicole R. McMillian, and Angela D. Motter

differentiate between them and help provide a definitive diagnosis when used alongside histopathological evaluation. Of note, this article does not include certain tumors in Table 1 (ie, Müllerian adenosarcoma, carcinosarcoma) for which the panel does not