Background

The NCI defines a rare cancer as one having an age-adjusted incidence of fewer than 15 cases per 100,000 per year.  Despite their rarity, these cancers collectively account for approximately 25% of all new cases of adult cancers each year and all pediatric cancers in the United States.

In general, rare cancers are understudied, especially outside of clinical treatment trials, impeding the progress of research and hindering informed decision-making for patients, physicians, and policymakers. For many rare cancers, research to identify causes or develop strategies for prevention and early detection is extremely difficult. There are often delays in diagnosis for patients with rare cancers due to the rarity of the disease and a lack of standard of care treatment options that can impact optimal care delivery and outcomes.  Conversely, for some rare cancers, the development of more effective therapies or early-detection strategies has led an increase in the number of short- and longer-term survivors; however, little is known about the survivorship needs of, and ways to address these needs for, these individuals.  Thus, there are numerous gaps in knowledge. These gaps have led to inequities and disadvantages in care for those individuals at risk or diagnosed with rare cancers compared with individuals at risk or diagnosed with more common cancers.

Research Objectives

This purpose of this NOSI is to stimulate observational and intervention research focused on rare cancers that is within the mission of DCCPS. DCCPS has several broad areas of interest: development and validation of methods, technologies, and tools in surveillance, statistics, epidemiology, health care delivery, and the behavioral sciences; identification of modifiable risk factors or host susceptibility factors associated with cancer etiology, cancer prevention, and treatment outcomes; and clinical and translational science to reduce risk, incidence, and death from cancer, as well as to enhance the quality of life for cancer survivors.

Research interests for this NOSI, reflecting the broad areas of interest of DCCPS, include but are not limited to:

• Population-level studies aimed at understanding the determinants of rare cancer occurrence and outcomes.
• Identification and understanding of personal susceptibility factors for rare cancers such as genetic/genomic, epigenetic, immunological, inflammation, and hormonal biological pathways, and social, cultural, and race/ethnic factors; characterizing factors to reduce rare cancer risk in humans, including exposures to physical and chemical agents; social and behavioral built environments; nutritional components; physical activity and energy balance; and infectious agents.
• Behavioral studies that focus on key psychological, social, behavioral, and biobehavioral targets across the cancer control continuum for rare cancers, from prevention to end-of-life care in general, high risk, underserved, and survivor populations.
• Health care delivery research that focuses on the study of cancer care, multilevel factors influencing care, and outcomes of care for rare cancer survivor populations. Cancer care refers to medical services offered across the cancer continuum, such as screening individuals not known to have cancer; timely and accurate diagnosis; treating cancer patients; following cancer survivors for recurrence, new cancers, and physical and psychological symptoms; and providing psychosocial support for patients and their caregivers.
• Research that aims to enhance the quality and/or length of survival for persons diagnosed with rare cancers or that will prevent, minimize, or manage adverse effects of cancer and its treatment.
• Surveillance research that focuses on developing analytical tools and methodologies in collecting, interpreting, and disseminating population-based cancer statistics in the US. Cancer surveillance provides a quantitative portrait of cancer and its determinants in a defined population.
• Implementation science that focuses on strategies to influence equitable adoption, implementation, sustainment and scale-up of evidence-based rare cancer control interventions, considers adaptation of interventions and settings to optimize uptake and impact of interventions, and (where indicated) de-implementation of ineffective or harmful interventions. Evidence-based cancer control interventions refer to the collection of research-tested practices and programs across the cancer continuum shown to benefit cancer-related outcomes.

Applicants are encouraged to leverage existing infrastructures and/or data sources, when appropriate.  Examples include the NCI’s Surveillance, Epidemiology, and End Results (SEER) Program and SEER Program Data & Software; established cancer epidemiology cohorts, such as those listed in the Cancer Epidemiology Descriptive Cohort Database (CEDCD); and genotype and phenotype data deposited in the database of Genotypes and Phenotypes (dbGaP).