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Elliott K. Yee, Natalie G. Coburn, Laura E. Davis, Alyson L. Mahar, Victoria Zuk, Vaibhav Gupta, Ying Liu, Craig C. Earle and Julie Hallet

Background: Little is known about how the geographic distribution of cancer services may influence disparities in outcomes for noncurable pancreatic adenocarcinoma. We therefore examined the geographic distribution of outcomes for this disease in relation to distance to cancer centers. Methods: We conducted a retrospective population-based analysis of adults in Ontario, Canada, diagnosed with noncurable pancreatic adenocarcinoma from 2004 through 2017 using linked administrative healthcare datasets. The exposure was distance from place of residence to the nearest cancer center providing medical oncology assessment and systemic therapy. Outcomes were medical oncology consultation, receipt of cancer-directed therapy, and overall survival. We examined the relationship between distance and outcomes using adjusted multivariable regression models. Results: Of 15,970 patients surviving a median of 3.3 months, 65.6% consulted medical oncology and 38.5% received systemic therapy. Regions with comparable outcomes were clustered throughout Ontario. Mapping revealed regional discordances between outcomes. Increasing distance (reference, ≤10 km) was independently associated with lower likelihood of medical oncology consultation (relative risks [95% CI] for 11–50, 51–100, and ≥101 km were 0.90 [0.83–0.98], 0.78 [0.62–0.99], and 0.77 [0.55–1.08], respectively) and worse survival (hazard ratios [95% CI] for 11–50, 51–100, and ≥101 km were 1.08 [1.04–1.12], 1.17 [1.10–1.25], and 1.10 [1.02–1.18], respectively), but not with likelihood of receiving therapy. Receipt of therapy seems less sensitive to distance, suggesting that distance limits entry into the cancer care system via oncology consultation. Regional outcome discordances suggest inefficiencies within and protective factors outside of the cancer care system. Conclusions: These findings provide a basis for clinicians to optimize their practices for patients with noncurable pancreatic adenocarcinoma, for future studies investigating geographic barriers to care, and for regional interventions to improve access.

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Shaji K. Kumar, Natalie S. Callander, Kehinde Adekola, Larry Anderson, Muhamed Baljevic, Erica Campagnaro, Jorge J. Castillo, Jason C. Chandler, Caitlin Costello, Yvonne Efebera, Matthew Faiman, Alfred Garfall, Kelly Godby, Jens Hillengass, Leona Holmberg, Myo Htut, Carol Ann Huff, Yubin Kang, Malin Hultcrantz, Sarah Larson, Michaela Liedtke, Thomas Martin, James Omel, Kenneth Shain, Douglas Sborov, Keith Stockerl-Goldstein, Donna Weber, Jennifer Keller and Rashmi Kumar

Multiple myeloma is a malignant neoplasm of plasma cells that accumulate in bone marrow, leading to bone destruction and marrow failure. This manuscript discusses the management of patients with solitary plasmacytoma, smoldering multiple myeloma, and newly diagnosed multiple myeloma.

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NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021

Featured Updates to the NCCN Guidelines

Margaret von Mehren, John M. Kane III, Marilyn M. Bui, Edwin Choy, Mary Connelly, Sarah Dry, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, Jade Homsi, Vicki Keedy, Ciara M. Kelly, Edward Kim, David Liebner, Martin McCarter, Sean V. McGarry, Christian Meyer, Alberto S. Pappo, Amanda M. Parkes, I. Benjamin Paz, Ivy A. Petersen, Matthew Poppe, Richard F. Riedel, Brian Rubin, Scott Schuetze, Jacob Shabason, Jason K. Sicklick, Matthew B. Spraker, Melissa Zimel, Mary Anne Bergman and Giby V. George

The NCCN Guidelines for Soft Tissue Sarcoma provide recommendations for the diagnosis, evaluation, treatment, and follow-up for patients with soft tissue sarcomas. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including the development of a separate and distinct guideline for gastrointestinal stromal tumors (GISTs); reconception of the management of desmoid tumors; inclusion of further recommendations for the diagnosis and management of extremity/body wall, head/neck sarcomas, and retroperitoneal sarcomas; modification and addition of systemic therapy regimens for sarcoma subtypes; and revision of the principles of radiation therapy for soft tissue sarcomas.

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Emily C. Harrold, Ahmad F. Idris, Niamh M. Keegan, Lynda Corrigan, Min Yuen Teo, Martin O’Donnell, Sean Tee Lim, Eimear Duff, Dearbhaile M. O’Donnell, M. John Kennedy, Sue Sukor, Cliona Grant, David G. Gallagher, Sonya Collier, Tara Kingston, Ann Marie O’Dwyer and Sinead Cuffe

Background: The NCCN Guidelines for Survivorship recommend dedicated sleep assessment. Reported insomnia prevalence in the general Irish population is 6% to 15%. Reported insomnia prevalence internationally among new/recently diagnosed patients with cancer varies from 30.9% to 54.3%. Insomnia prevalence has not been previously quantified in an Irish oncology cohort. Methods: A 40-item questionnaire was prospectively administered to ambulatory patients with cancer aged ≥18 years. Prespecified criteria to define insomnia syndrome combined those of the International Classification of Sleep Disorders, version 1, and the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV). The Hospital Anxiety and Depression Scale-Depression/Anxiety (HADS-D/A) was used to screen for potential confounding variables. Results: The response rate to the questionnaire was 87% (294/337). The predominant respondent age group was 55 to 64 years (26%; 77/294), 70.7% were female (208/294), and the most common cancer subtypes were breast (37.4%), colorectal (12.9%), and lung (12.2%). A total of 62% (183/294) of patients reported sleep disturbance after diagnosis, 63% (115/183) reported moderate/severe distress related to this disturbance, and 37% (61/183) reported a significant impact on physical function. Although 33% (98/294) met insomnia syndrome criteria, only 34% (33/98) of these patients had a preexisting history of sleep disturbance. Female sex, age <65 years, cancer subtype, alcohol consumption, and HADS-D/A ≥11 were associated with statistically significant higher odds ratios (OR) of insomnia syndrome. Multivariate analysis identified breast cancer (OR, 3.17; P=.01), age <65 years (OR, 1.8; P=.03), and alcohol consumption (OR, 2.3; P=.005) as independent predictors of insomnia syndrome. Conclusions: Insomnia syndrome prevalence in this cohort is comparable to that reported previously and supports dedicated sleep assessment. This study identifies potentially modifiable risk factors for insomnia and demonstrates additional utility of the HADS score in identifying patients at risk.

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Karam Khaddour, Michael R. Chicoine, Jiayi Huang, Sonika Dahiya and George Ansstas

Craniopharyngiomas are rare tumors that arise in the suprasellar region of the brain and are known for their aggressive nature despite their WHO grade I. This is due to the complex neuroanatomy of the sellar/suprasellar region and their proximity to the optic nerve apparatus, hypothalamic–pituitary tract, and other critical neuroanatomical structures. Definitive treatment is based on a multidisciplinary approach and often involves a combination of surgical, radiation, and medical therapy. However, there is high morbidity associated with surgery and RT due to the complex neuroanatomy of this region. Recently, BRAF V600E somatic mutation has been identified in most papillary craniopharyngiomas. This discovery has led to the novel use of RAF pathway inhibitors to treat these tumors. We report the successful use of dabrafenib (BRAF inhibitor) and trametinib (mitogen-activated protein kinase kinase inhibitor) in the neoadjuvant setting followed by definitive stereotactic radiosurgery. We propose an algorithm based on available literature on the integration of targeted therapy in the management of papillary craniopharyngiomas. Our observations, together with prior case reports, advocate the incorporation of targeted therapy for unresectable craniopharyngiomas and reinforce that treatment with dual-targeted therapy is safe and effective.

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Daniel L. Hall and Jeffrey M. Peppercorn

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Sarju Ganatra, Sourbha S. Dani, Robert Redd, Kimberly Rieger-Christ, Rushin Patel, Rohan Parikh, Aarti Asnani, Vigyan Bang, Katherine Shreyder, Simarjeet S. Brar, Amitoj Singh, Dhruv S. Kazi, Avirup Guha, Salim S. Hayek, Ana Barac, Krishna S. Gunturu, Corrine Zarwan, Anne C. Mosenthal, Shakeeb A. Yunus, Amudha Kumar, Jaymin M. Patel, Richard D. Patten, David M. Venesy, Sachin P. Shah, Frederic S. Resnic, Anju Nohria and Suzanne J. Baron

Background: Cancer and cardiovascular disease (CVD) are independently associated with adverse outcomes in patients with COVID-19. However, outcomes in patients with COVID-19 with both cancer and comorbid CVD are unknown. Methods: This retrospective study included 2,476 patients who tested positive for SARS-CoV-2 at 4 Massachusetts hospitals between March 11 and May 21, 2020. Patients were stratified by a history of either cancer (n=195) or CVD (n=414) and subsequently by the presence of both cancer and CVD (n=82). We compared outcomes between patients with and without cancer and patients with both cancer and CVD compared with patients with either condition alone. The primary endpoint was COVID-19–associated severe disease, defined as a composite of the need for mechanical ventilation, shock, or death. Secondary endpoints included death, shock, need for mechanical ventilation, need for supplemental oxygen, arrhythmia, venous thromboembolism, encephalopathy, abnormal troponin level, and length of stay. Results: Multivariable analysis identified cancer as an independent predictor of COVID-19–associated severe disease among all infected patients. Patients with cancer were more likely to develop COVID-19–associated severe disease than were those without cancer (hazard ratio [HR], 2.02; 95% CI, 1.53–2.68; P<.001). Furthermore, patients with both cancer and CVD had a higher likelihood of COVID-19–associated severe disease compared with those with either cancer (HR, 1.86; 95% CI, 1.11–3.10; P=.02) or CVD (HR, 1.79; 95% CI, 1.21–2.66; P=.004) alone. Patients died more frequently if they had both cancer and CVD compared with either cancer (35% vs 17%; P=.004) or CVD (35% vs 21%; P=.009) alone. Arrhythmias and encephalopathy were also more frequent in patients with both cancer and CVD compared with those with cancer alone. Conclusions: Patients with a history of both cancer and CVD are at significantly higher risk of experiencing COVID-19–associated adverse outcomes. Aggressive public health measures are needed to mitigate the risks of COVID-19 infection in this vulnerable patient population.

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Louis Burt Nabors, Jana Portnow, Manmeet Ahluwalia, Joachim Baehring, Henry Brem, Steven Brem, Nicholas Butowski, Jian L. Campian, Stephen W. Clark, Andrew J. Fabiano, Peter Forsyth, Jona Hattangadi-Gluth, Matthias Holdhoff, Craig Horbinski, Larry Junck, Thomas Kaley, Priya Kumthekar, Jay S. Loeffler, Maciej M. Mrugala, Seema Nagpal, Manjari Pandey, Ian Parney, Katherine Peters, Vinay K. Puduvalli, Ian Robins, Jason Rockhill, Chad Rusthoven, Nicole Shonka, Dennis C. Shrieve, Lode J. Swinnen, Stephanie Weiss, Patrick Yung Wen, Nicole E. Willmarth, Mary Anne Bergman and Susan D. Darlow

The NCCN Guidelines for Central Nervous System (CNS) Cancers focus on management of adult CNS cancers ranging from noninvasive and surgically curable pilocytic astrocytomas to metastatic brain disease. The involvement of an interdisciplinary team, including neurosurgeons, radiation therapists, oncologists, neurologists, and neuroradiologists, is a key factor in the appropriate management of CNS cancers. Integrated histopathologic and molecular characterization of brain tumors such as gliomas should be standard practice. This article describes NCCN Guidelines recommendations for WHO grade I, II, III, and IV gliomas. Treatment of brain metastases, the most common intracranial tumors in adults, is also described.