approximately 70% of individuals with Cowden syndrome, 96 and the lifetime risk for thyroid cancer (follicular or papillary) has been estimated at 3% to 10%. 11 , 97 As in many other hereditary cancer syndromes, affected individuals are more likely to develop
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Mary B. Daly, Jennifer E. Axilbund, Saundra Buys, Beth Crawford, Carolyn D. Farrell, Susan Friedman, Judy E. Garber, Salil Goorha, Stephen B. Gruber, Heather Hampel, Virginia Kaklamani, Wendy Kohlmann, Allison Kurian, Jennifer Litton, P. Kelly Marcom, Robert Nussbaum, Kenneth Offit, Tuya Pal, Boris Pasche, Robert Pilarski, Gwen Reiser, Kristen Mahoney Shannon, Jeffrey R. Smith, Elizabeth Swisher, and Jeffrey N. Weitzel
Kamran Aghayev, Frank Vrionis, and Marc C. Chamberlain
invasive tumors with nerve root encasement and even extravertebral invasion have been reported. Histologically, myxopapillary ependymomas are characterized by myxoid changes in the stroma and papillary organization of tumor cells. 85 Microsurgery plays an
Dawn Provenzale, Samir Gupta, Dennis J. Ahnen, Travis Bray, Jamie A. Cannon, Gregory Cooper, Donald S. David, Dayna S. Early, Deborah Erwin, James M. Ford, Francis M. Giardiello, William Grady, Amy L. Halverson, Stanley R. Hamilton, Heather Hampel, Mohammad K. Ismail, Jason B. Klapman, David W. Larson, Audrey J. Lazenby, Patrick M. Lynch, Robert J. Mayer, Reid M. Ness, Scott E. Regenbogen, Niloy Jewel Samadder, Moshe Shike, Gideon Steinbach, David Weinberg, Mary Dwyer, and Susan Darlow
-morular variant of papillary thyroid cancer, hepatoblastoma, or multiple gastrointestinal hamartomatous polyps). Detailed strategies for screening for Lynch syndrome are provided below; see the NCCN guidelines for detail on follow-up patients meeting risk criteria
NCCN Guidelines Insights: Genetic/Familial High-Risk Assessment: Breast, Ovarian, and Pancreatic, Version 1.2020
Featured Updates to the NCCN Guidelines
Mary B. Daly, Robert Pilarski, Matthew B. Yurgelun, Michael P. Berry, Saundra S. Buys, Patricia Dickson, Susan M. Domchek, Ahmed Elkhanany, Susan Friedman, Judy E. Garber, Michael Goggins, Mollie L. Hutton, Seema Khan, Catherine Klein, Wendy Kohlmann, Allison W. Kurian, Christine Laronga, Jennifer K. Litton, Julie S. Mak, Carolyn S. Menendez, Sofia D. Merajver, Barbara S. Norquist, Kenneth Offit, Tuya Pal, Holly J. Pederson, Gwen Reiser, Kristen Mahoney Shannon, Kala Visvanathan, Jeffrey N. Weitzel, Myra J. Wick, Kari B. Wisinski, Mary A. Dwyer, and Susan D. Darlow
, although long-term studies are needed in this area. Lesions detected through routine screening may not always require resection (eg, sporadic branch duct intraductal papillary mucinous neoplasms). Therefore, larger long-term studies are needed to further
, endometrial, papillary urothelial, gastric, melanoma, and urinary tract cancers. More research is needed to understand the relationship between PALB2 and other cancer risks, particularly in males because they represented <1% of patients tested in this study
%) malignant fibrous histiocytoma, 2 (<1%) papillary cystadenocarcinoma, 2 (<1%) Merkel cell carcinoma, 2 (<1%), myoepithelial carcinoma, 2 (<1%) malignant mixed tumor, 1 (<1%)adenocarcinoma, and 15 (5%) unknown carcinoma. Tumor grade was also studied: 35 (13
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
BN Omura GA . Stage III ovarian tumors of low malignant potential treated with cisplatin combination therapy (a Gynecologic Oncology Group study) . Gynecol Oncol 1991 ; 41 : 230 – 233 . 152. Kennedy AW Hart WR . Ovarian papillary
Randall W. Burt, James S. Barthel, Kelli Bullard Dunn, Donald S. David, Ernesto Drelichman, James M. Ford, Francis M. Giardiello, Stephen B. Gruber, Amy L. Halverson, Stanley R. Hamilton, Mohammad K. Ismail, Kory Jasperson, Audrey J. Lazenby, Patrick M. Lynch, Edward W. Martin Jr., Robert J. Mayer, Reid M. Ness, Dawn Provenzale, M. Sambasiva Rao, Moshe Shike, Gideon Steinbach, Jonathan P. Terdiman, and David Weinberg
. High-grade dysplasia is defined as an adenoma that shows features of severe dysplasia (marked reduction of interglandular stromas with complex irregularity of glands, papillary infolding, and cytogenetic abnormalities) or carcinoma in situ (severe
Alan P. Venook, Maria E. Arcila, Al B. Benson III, Donald A. Berry, David Ross Camidge, Robert W. Carlson, Toni K. Choueiri, Valerie Guild, Gregory P. Kalemkerian, Razelle Kurzrock, Christine M. Lovly, Amy E. McKee, Robert J. Morgan, Anthony J. Olszanski, Mary W. Redman, Vered Stearns, Joan McClure, and Marian L. Birkeland
, constitute many different histologies, including type I and type II papillary and chromophobe tumors. Among the clear cell subset, 2 groups of molecularly targeted therapies have emerged for the treatment of metastatic disease; 5 agents target the VEGF
Robert A. Figlin, Elizabeth Brown, Andrew J. Armstrong, Wallace Akerley, Al B. Benson III, Harold J. Burstein, David S. Ettinger, Phillip G. Febbo, Matthew G. Fury, Gary R. Hudes, Merrill S. Kies, Eunice L. Kwak, Robert J. Morgan Jr., Joanne Mortimer, Karen Reckamp, Alan P. Venook, Frank Worden, and Yun Yen
clear cell (e.g., chromophobe, papillary histology) and those who had not undergone nephrectomy (one third of patients enrolled) were additional unique features. The primary end point of the trial was overall survival, with 2 comparisons: temsirolimus
