The NPM-ALK fusion protein is found in ALK+ anaplastic large cell lymphomas harboring the t(2;5) chromosomal translocation. Patients harboring ALK translocations typically have an excellent prognosis with conventional chemotherapy and a reported 5-year survival rate of 70%. Although most patients with ALK+ anaplastic large cell lymphoma have a good prognosis, some patients do not respond to standard therapies. In patients with refractory anaplastic large cell lymphoma who can achieve remission, allogeneic stem cell transplant is a potentially curative option. This article describes a patient with refractory ALK+ anaplastic large cell lymphoma who experienced a complete response to the ALK inhibitor crizotinib and then underwent an allogeneic stem cell transplant followed by crizotinib maintenance therapy.
James M. Cleary, Scott Rodig, Paul M. Barr, Atul B. Shinagare, Jeffrey W. Clark, Geoffrey I. Shapiro and Philippe Armand
Razelle Kurzrock, A. Dimitrios Colevas, Anthony Olszanski, Wallace Akerley, Carlos L. Arteaga, William E. Carson III, Jeffrey W. Clark, John F. DiPersio, David S. Ettinger, Robert J. Morgan Jr, Lee S. Schwartzberg, Alan P. Venook, Christopher D. Gocke, Jonathan Tait and F. Marc Stewart
Background: With advances such as next-generation sequencing (NGS) increasing understanding of the basis of cancer and its response to treatment, NCCN believes it is important to understand how molecular profiling/diagnostic testing is being performed and used at NCCN Member Institutions and their community affiliates. Methods: The NCCN Oncology Research Program's Investigator Steering Committee and the NCCN Best Practices Committee gathered baseline information on the use of cancer-related molecular testing at NCCN Member Institutions and community members of the NCCN Affiliate Research Consortium through 2 separate surveys distributed in December 2013 and September 2014, respectively. Results: A total of 24 NCCN Member Institutions and 8 affiliate sites provided quantitative and qualitative data. In the context of these surveys, “molecular profiling/diagnostics” was defined as a panel of at least 10 genes examined as a diagnostic DNA test in a Clinical Laboratory Improvement Amendments (CLIA)–certified laboratory. Conclusions: Results indicated that molecular profiling/diagnostics are used at 100% of survey respondents' institutions to make patient care decisions. However, challenges relating to reimbursement, lack of data regarding actionable targets and targeted therapies, and access to drugs on or off clinical trials were cited as barriers to integration of molecular profiling into patient care. Frameworks for using molecular diagnostic results based on levels of evidence, alongside continued research into the predictive value of biomarkers and targeted therapies, are recommended to advance understanding of the role of genomic biomarkers. Greater evidence and consensus regarding the clinical and cost-effectiveness of molecular profiling may lead to broader insurance coverage and increased integration into patient care.
Matthew H. Kulke, Al B. Benson III, Emily Bergsland, Jordan D. Berlin, Lawrence S. Blaszkowsky, Michael A. Choti, Orlo H. Clark, Gerard M. Doherty, James Eason, Lyska Emerson, Paul F. Engstrom, Whitney S. Goldner, Martin J. Heslin, Fouad Kandeel, Pamela L. Kunz, Boris W. Kuvshinoff II, Jeffrey F. Moley, Venu G. Pillarisetty, Leonard Saltz, David E. Schteingart, Manisha H. Shah, Stephen Shibata, Jonathan R. Strosberg, Jean-Nicolas Vauthey, Rebekah White, James C. Yao, Deborah A. Freedman-Cass and Mary A. Dwyer
Neuroendocrine tumors comprise a broad family of tumors, the most common of which are carcinoid and pancreatic neuroendocrine tumors. The NCCN Neuroendocrine Tumors Guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine tumors. Most of the recommendations pertain to well-differentiated, low- to intermediate-grade tumors. This updated version of the NCCN Guidelines includes a new section on pathology for diagnosis and reporting and revised recommendations for the surgical management of neuroendocrine tumors of the pancreas.
James E. Montie, Peter E. Clark, Mario A. Eisenberger, Rizk El-Galley, Richard E. Greenberg, Harry W. Herr, Gary R. Hudes, Deborah A. Kuban, Timothy M. Kuzel, Paul H. Lange, Subodh M. Lele, Jeffrey Michalski, Anthony Patterson, Kamal S. Pohar, Jerome P. Richie, Wade J. Sexton, William U. Shipley, Eric J. Small, Donald L. Trump, Phillip J. Walther and Timothy G. Wilson
Philippe E. Spiess, Neeraj Agarwal, Rick Bangs, Stephen A. Boorjian, Mark K. Buyyounouski, Peter E. Clark, Tracy M. Downs, Jason A. Efstathiou, Thomas W. Flaig, Terence Friedlander, Richard E. Greenberg, Khurshid A. Guru, Noah Hahn, Harry W. Herr, Christopher Hoimes, Brant A. Inman, Masahito Jimbo, A. Karim Kader, Subodh M. Lele, Joshua J. Meeks, Jeff Michalski, Jeffrey S. Montgomery, Lance C. Pagliaro, Sumanta K. Pal, Anthony Patterson, Elizabeth R. Plimack, Kamal S. Pohar, Michael P. Porter, Mark A. Preston, Wade J. Sexton, Arlene O. Siefker-Radtke, Guru Sonpavde, Jonathan Tward, Geoffrey Wile, Mary A. Dwyer and Lisa A. Gurski
This selection from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Bladder Cancer focuses on systemic therapy for muscle-invasive urothelial bladder cancer, as substantial revisions were made in the 2017 updates, such as new recommendations for nivolumab, pembrolizumab, atezolizumab, durvalumab, and avelumab. The complete version of the NCCN Guidelines for Bladder Cancer addresses additional aspects of the management of bladder cancer, including non–muscle-invasive urothelial bladder cancer and nonurothelial histologies, as well as staging, evaluation, and follow-up.
Peter E. Clark, Philippe E. Spiess, Neeraj Agarwal, Rick Bangs, Stephen A. Boorjian, Mark K. Buyyounouski, Jason A. Efstathiou, Thomas W. Flaig, Terence Friedlander, Richard E. Greenberg, Khurshid A. Guru, Noah Hahn, Harry W. Herr, Christopher Hoimes, Brant A. Inman, A. Karim Kader, Adam S. Kibel, Timothy M. Kuzel, Subodh M. Lele, Joshua J. Meeks, Jeff Michalski, Jeffrey S. Montgomery, Lance C. Pagliaro, Sumanta K. Pal, Anthony Patterson, Daniel Petrylak, Elizabeth R. Plimack, Kamal S. Pohar, Michael P. Porter, Wade J. Sexton, Arlene O. Siefker-Radtke, Guru Sonpavde, Jonathan Tward, Geoffrey Wile, Mary A. Dwyer and Courtney Smith
These NCCN Guidelines Insights discuss the major recent updates to the NCCN Guidelines for Bladder Cancer based on the review of the evidence in conjunction with the expert opinion of the panel. Recent updates include (1) refining the recommendation of intravesical bacillus Calmette-Guérin, (2) strengthening the recommendations for perioperative systemic chemotherapy, and (3) incorporating immunotherapy into second-line therapy for locally advanced or metastatic disease. These NCCN Guidelines Insights further discuss factors that affect integration of these recommendations into clinical practice.