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Merkel Cell Carcinoma

Stanley J. Miller, Murad Alam, James Andersen, Daniel Berg, Christopher K. Bichakjian, Glen Bowen, Richard T. Cheney, L. Frank Glass, Roy C. Grekin, Dennis E. Hallahan, Anne Kessinger, Nancy Y. Lee, Nanette Liegeois, Daniel D. Lydiatt, Jeff Michalski, William H. Morrison, Kishwer S. Nehal, Kelly C. Nelson, Paul Nghiem, Thomas Olencki, Allan R. Oseroff, Clifford S. Perlis, E. William Rosenberg, Ashok R. Shaha, Marshall M. Urist, and Linda C. Wang

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Basal Cell Skin Cancer, Version 1.2016, NCCN Clinical Practice Guidelines in Oncology

Christopher K. Bichakjian, Thomas Olencki, Sumaira Z. Aasi, Murad Alam, James S. Andersen, Daniel Berg, Glen M. Bowen, Richard T. Cheney, Gregory A. Daniels, L. Frank Glass, Roy C. Grekin, Kenneth Grossman, Susan A. Higgins, Alan L. Ho, Karl D. Lewis, Daniel D. Lydiatt, Kishwer S. Nehal, Paul Nghiem, Elise A. Olsen, Chrysalyne D. Schmults, Aleksandar Sekulic, Ashok R. Shaha, Wade L. Thorstad, Malika Tuli, Marshall M. Urist, Timothy S. Wang, Sandra L. Wong, John A. Zic, Karin G. Hoffmann, and Anita Engh

Basal cell carcinoma (BCC) of the skin is the most common cancer, with a higher incidence than all other malignancies combined. Although it is rare to metastasize, patients with multiple or frequently recurring BCC can suffer substantial comorbidity and be difficult to manage. Assessment of risk is a key element of management needed to inform treatment selection. The overall management of BCC primarily consists of surgical approaches, with radiation therapy as an alternate or adjuvant option. Many superficial therapies for BCC have been explored and continue to be developed, including topicals, cryosurgery, and photodynamic therapy. Two hedgehog pathway inhibitors were recently approved by the FDA for systemic treatment of advanced and metastatic BCC, and others are in development. The NCCN Guidelines for Basal Cell Skin Cancer, published in full herein, include recommendations for selecting among the various surgical approaches based on patient-, lesion-, and disease-specific factors, as well as guidance on when to use radiation therapy, superficial therapies, and hedgehog pathway inhibitors.

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Dermatofibrosarcoma Protuberans

Stanley J. Miller, Murad Alam, James S. Andersen, Daniel Berg, Christopher K. Bichakjian, Glen M. Bowen, Richard T. Cheney, L. Frank Glass, Roy C. Grekin, Alan L. Ho, Anne Kessinger, Nanette Liegeois, Daniel D. Lydiatt, Jeff Michalski, William H. Morrison, Kishwer S. Nehal, Kelly C. Nelson, Paul Nghiem, Thomas Olencki, Clifford S. Perlis, Ashok R. Shaha, Malika Tuli, Marshall M. Urist, Linda C. Wang, and John A. Zic

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Merkel Cell Carcinoma, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology

Christopher K. Bichakjian, Thomas Olencki, Sumaira Z. Aasi, Murad Alam, James S. Andersen, Rachel Blitzblau, Glen M. Bowen, Carlo M. Contreras, Gregory A. Daniels, Roy Decker, Jeffrey M. Farma, Kris Fisher, Brian Gastman, Karthik Ghosh, Roy C. Grekin, Kenneth Grossman, Alan L. Ho, Karl D. Lewis, Manisha Loss, Daniel D. Lydiatt, Jane Messina, Kishwer S. Nehal, Paul Nghiem, Igor Puzanov, Chrysalyne D. Schmults, Ashok R. Shaha, Valencia Thomas, Yaohui G. Xu, John A. Zic, Karin G. Hoffmann, and Anita M. Engh

This selection from the NCCN Guidelines for Merkel Cell Carcinoma (MCC) focuses on areas impacted by recently emerging data, including sections describing MCC risk factors, diagnosis, workup, follow-up, and management of advanced disease with radiation and systemic therapy. Included in these sections are discussion of the new recommendations for use of Merkel cell polyomavirus as a biomarker and new recommendations for use of checkpoint immunotherapies to treat metastatic or unresectable disease. The next update of the complete version of the NCCN Guidelines for MCC will include more detailed information about elements of pathology and addresses additional aspects of management of MCC, including surgical management of the primary tumor and draining nodal basin, radiation therapy as primary treatment, and management of recurrence.

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Basal Cell and Squamous Cell Skin Cancers

Stanley J. Miller, Murad Alam, James Andersen, Daniel Berg, Christopher K. Bichakjian, Glen Bowen, Richard T. Cheney, L. Frank Glass, Roy C. Grekin, Anne Kessinger, Nancy Y. Lee, Nanette Liegeois, Daniel D. Lydiatt, Jeff Michalski, William H. Morrison, Kishwer S. Nehal, Kelly C. Nelson, Paul Nghiem, Thomas Olencki, Clifford S. Perlis, E. William Rosenberg, Ashok R. Shaha, Marshall M. Urist, Linda C. Wang, and John A. Zic

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Merkel Cell Carcinoma, Version 1.2014

Christopher K. Bichakjian, Thomas Olencki, Murad Alam, James S. Andersen, Daniel Berg, Glen M. Bowen, Richard T. Cheney, Gregory A. Daniels, L. Frank Glass, Roy C. Grekin, Kenneth Grossman, Alan L. Ho, Karl D. Lewis, Daniel D. Lydiatt, William H. Morrison, Kishwer S. Nehal, Kelly C. Nelson, Paul Nghiem, Clifford S. Perlis, Ashok R. Shaha, Wade L. Thorstad, Malika Tuli, Marshall M. Urist, Timothy S. Wang, Andrew E. Werchniak, Sandra L. Wong, John A. Zic, Karin G. Hoffmann, Nicole R. McMillian, and Maria Ho

Merkel cell carcinoma is a rare, aggressive cutaneous tumor that combines the local recurrence rates of infiltrative nonmelanoma skin cancer with the regional and distant metastatic rates of thick melanoma. The NCCN Guidelines for Merkel Cell Carcinoma provide recommendations on the diagnosis and management of this aggressive disease based on clinical evidence and expert consensus. This version includes revisions regarding the use of PET/CT imaging and the addition of a new section on the principles of pathology to provide guidance on the analysis, interpretation, and reporting of pathology results.

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Dermatofibrosarcoma Protuberans, Version 1.2014

Christopher K. Bichakjian, Thomas Olencki, Murad Alam, James S. Andersen, Daniel Berg, Glen M. Bowen, Richard T. Cheney, Gregory A. Daniels, L. Frank Glass, Roy C. Grekin, Kenneth Grossman, Alan L. Ho, Karl D. Lewis, Daniel D. Lydiatt, William H. Morrison, Kishwer S. Nehal, Kelly C. Nelson, Paul Nghiem, Clifford S. Perlis, Ashok R. Shaha, Wade L. Thorstad, Malika Tuli, Marshall M. Urist, Timothy S. Wang, Andrew E. Werchniak, Sandra L. Wong, John A. Zic, Nicole McMillian, Karin Hoffman, and Maria Ho

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor characterized by a relatively high risk of local recurrence and low risk of metastasis. The NCCN Guidelines for DFSP provide multidisciplinary recommendations on the management of patients with this rare disease. These NCCN Guidelines Insights highlight the addition of the Principles of Pathology section, which provides recommendations on the pathologic assessment of DFSP. Because DFSP can mimic other lesions, immunohistochemical studies are often required to establish diagnosis. Cytogenetic testing for the characteristic translocation t(17;22)(q22;q13) can also be valuable in the differential diagnosis of DFSP with other histologically similar tumors.

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Basal Cell Skin Cancer, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology

Chrysalyne D. Schmults, Rachel Blitzblau, Sumaira Z. Aasi, Murad Alam, Arya Amini, Kristin Bibee, Jeremy Bordeaux, Pei-Ling Chen, Carlo M. Contreras, Dominick DiMaio, Jessica M. Donigan, Jeffrey M. Farma, Karthik Ghosh, Kelly Harms, Alan L. Ho, John Nicholas Lukens, Lawrence Mark, Theresa Medina, Kishwer S. Nehal, Paul Nghiem, Kelly Olino, Soo Park, Tejesh Patel, Igor Puzanov, Jason Rich, Aleksandar Sekulic, Ashok R. Shaha, Divya Srivastava, Valencia Thomas, Courtney Tomblinson, Puja Venkat, Yaohui Gloria Xu, Siegrid Yu, Mehran Yusuf, Beth McCullough, and Sara Espinosa

Basal cell carcinoma (BCC) is the most common form of skin cancer in the United States. Due to the high frequency, BCC occurrences are not typically recorded, and annual rates of incidence can only be estimated. Current estimated rates are 2 million Americans affected annually, and this continues to rise. Exposure to radiation, from either sunlight or previous medical therapy, is a key player in BCC development. BCC is not as aggressive as other skin cancers because it is less likely to metastasize. However, surgery and radiation are prevalent treatment options, therefore disfigurement and limitation of function are significant considerations. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline an updated risk stratification and treatment options available for BCC.

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NCCN Guidelines® Insights: Merkel Cell Carcinoma, Version 1.2024

Featured Updates to the NCCN Guidelines

Chrysalyne D. Schmults, Rachel Blitzblau, Sumaira Z. Aasi, Murad Alam, Arya Amini, Kristin Bibee, Diana Bolotin, Jeremy Bordeaux, Pei-Ling Chen, Carlo M. Contreras, Dominick DiMaio, Jessica M. Donigan, Jeffrey M. Farma, Karthik Ghosh, Kelly Harms, Alan L. Ho, John Nicholas Lukens, Susan Manber, Lawrence Mark, Theresa Medina, Kishwer S. Nehal, Paul Nghiem, Kelly Olino, Soo Park, Tejesh Patel, Igor Puzanov, Jason Rich, Aleksandar Sekulic, Ashok R. Shaha, Divya Srivastava, Valencia Thomas, Courtney Tomblinson, Puja Venkat, Yaohui Gloria Xu, Siegrid Yu, Mehran Yusuf, Beth McCullough, and Sara Espinosa

The NCCN Guidelines for Merkel Cell Carcinoma (MCC) provide recommendations for diagnostic workup, clinical stage, and treatment options for patients. The panel meets annually to discuss updates to the guidelines based on comments from expert review from panel members, institutional review, as well as submissions from within NCCN and external organizations. These NCCN Guidelines Insights focus on the introduction of a new page for locally advanced disease in the setting of clinical node negative status, entitled “Clinical N0 Disease, Locally Advanced MCC.” This new algorithm page addresses locally advanced disease, and the panel clarifies the meaning behind the term “nonsurgical” by further defining locally advanced disease. In addition, the guideline includes the management of in-transit disease and updates to the systemic therapy options.

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NCCN Guidelines® Insights: Squamous Cell Skin Cancer, Version 1.2022

Featured Updates to the NCCN Guidelines

Chrysalyne D. Schmults, Rachel Blitzblau, Sumaira Z. Aasi, Murad Alam, James S. Andersen, Brian C. Baumann, Jeremy Bordeaux, Pei-Ling Chen, Robert Chin, Carlo M. Contreras, Dominick DiMaio, Jessica M. Donigan, Jeffrey M. Farma, Karthik Ghosh, Roy C. Grekin, Kelly Harms, Alan L. Ho, Ashley Holder, John Nicholas Lukens, Theresa Medina, Kishwer S. Nehal, Paul Nghiem, Soo Park, Tejesh Patel, Igor Puzanov, Jeffrey Scott, Aleksandar Sekulic, Ashok R. Shaha, Divya Srivastava, William Stebbins, Valencia Thomas, Yaohui G. Xu, Beth McCullough, Mary A. Dwyer, and Mai Q. Nguyen

The NCCN Guidelines for Squamous Cell Skin Cancer provide recommendations for diagnostic workup, clinical stage, and treatment options for patients with cutaneous squamous cell carcinoma. The NCCN panel meets annually to discuss updates to the guidelines based on comments from panel members and the Institutional Review, as well as submissions from within NCCN and external organizations. These NCCN Guidelines Insights focus on the introduction of a new surgical recommendation terminology (peripheral and deep en face margin assessment), as well as recent updates on topical prophylaxis, immunotherapy for regional and metastatic disease, and radiation therapy.